tag:blogger.com,1999:blog-8959359899491553872024-03-13T23:26:42.037+00:00Teach NeurologyA blog to teach under and post graduate trainees clinical neurologyGavin Giovannonihttp://www.blogger.com/profile/10222176473127281006noreply@blogger.comBlogger87125tag:blogger.com,1999:blog-895935989949155387.post-92180722150426274922021-09-09T08:23:00.001+01:002021-09-09T08:23:17.039+01:00Moved to substack<p> This blog has been moved to a <a href="https://teachneurology.substack.com/">new sight</a>!</p>Gavin Giovannonihttp://www.blogger.com/profile/03634514099871112077noreply@blogger.com0tag:blogger.com,1999:blog-895935989949155387.post-90824682676403349332020-09-18T09:29:00.003+01:002020-09-20T19:40:02.331+01:00Cerebellar ataxia and Romberg's test<span style="color: #351c75;"><b>Question</b>: "A patient with a pure cerebellar defect will not have a positive Romberg’s, because they will sway irrespective of their eyes being closed or open. I then read that a positive Romberg’s is due to either defective proprioception or vestibular sense."</span><br /><br />The confusion relates to how you perform the Romberg's test. It is correct that patients with pure cerebellar ataxia could have a positive Romberg's test if you don't stabilise them first by asking them to widen their base. You do this by asking them to open their legs and plant their feet as wide apart as possible until they stop swaying with their eyes open. Only then do you ask them to close their eyes? If they have a superimposed proprioceptive defect the body will start to sway and they will fall over. <br /><br />Please note in some patients with severe cerebellar ataxia you may not be able to get them to stand still, without a body sway, on a widened base. In this case, you can't do or interpret the Romberg's test. <p><br /></p><table align="center" cellpadding="0" cellspacing="0" class="tr-caption-container" style="margin-left: auto; margin-right: auto;"><tbody><tr><td style="text-align: center;"><a href="https://1.bp.blogspot.com/-GyodZZO69h4/X2RpmKUzUoI/AAAAAAAAjRE/bSwbZ_JclpkjRWmKEItBUpgULz5LZklqQCLcBGAsYHQ/s500/Rombergs.webp" style="margin-left: auto; margin-right: auto;"><img border="0" data-original-height="202" data-original-width="500" height="255" src="https://1.bp.blogspot.com/-GyodZZO69h4/X2RpmKUzUoI/AAAAAAAAjRE/bSwbZ_JclpkjRWmKEItBUpgULz5LZklqQCLcBGAsYHQ/w632-h255/Rombergs.webp" width="632" /></a></td></tr><tr><td class="tr-caption" style="text-align: center;">Image from <a href="https://www.google.com/url?sa=i&url=https%3A%2F%2Fquizlet.com%2Fca%2F313998469%2Fsensory-receptors-flash-cards%2F&psig=AOvVaw28ozVoqkteUGVMk1glXcDl&ust=1600502436292000&source=images&cd=vfe&ved=0CAIQjRxqFwoTCMDOxqye8usCFQAAAAAdAAAAABAW">Quizlet</a><br /></td></tr></tbody></table><br /><p>What is wrong in this online description of Romberg's test is making sure the patient is stable with their eyes open. This is done by asking them to widen their base and to stand with their feet apart. </p><p>There are other additional components that you may see some neurologists add to the Romberg's test. </p><p>1. Some neurologists ask patients to stretch out their arms in front of them and to look for pronator drift at the same time they are doing the Romberg's test. This is not essential but helps shorten the overall neurological examination. Please note that pronator drift is an upper motor neurone sign.</p><p><br /></p><div class="separator" style="clear: both; text-align: center;"><a href="https://1.bp.blogspot.com/-ck_iMD7vCik/X2RvS6nDV2I/AAAAAAAAjRU/7ZAKt0pUdgocw9c5t7HIxFK76R2D1q0JwCLcBGAsYHQ/s1280/F8.large_.jpg" style="margin-left: 1em; margin-right: 1em;"><img border="0" data-original-height="521" data-original-width="1280" height="241" src="https://1.bp.blogspot.com/-ck_iMD7vCik/X2RvS6nDV2I/AAAAAAAAjRU/7ZAKt0pUdgocw9c5t7HIxFK76R2D1q0JwCLcBGAsYHQ/w593-h241/F8.large_.jpg" width="593" /></a></div><br /><p>2. Some neurologists ask patients to tilt their heads back after first watching their posture with their head in the neutral position. The looking up at the ceiling manoeuvre will stimulate the posterior semicircular canals and cervical proprioceptive receptors. If there is a problem with the peripheral vestibular system or cervical spine it may cause the patient to develop increased body sway. </p><p>3. If the patient is steady with their eyes closed you can gently push them from side-to-side test their postural righting reflexes. This has may bring out a positive Romberg's test (swaying). However, it also testing the postural righting reflex which is coordinated by the pedunculopontine nucleus of the brainstem. This nucleus often degenerates in Parkinsonian syndromes such as Parkinson's disease, multisystem atrophy and progressive supranuclear atrophy. It is important that if the patient is not ataxic and has poor postural righting reflexes you don't interpret the Romberg's test as positive, but refer to the sign as indicating poor postural righting reflexes. </p><p>Patients with poor postural righting reflexes often don't extend their arms when falling and hence commonly suffer from head injuries and proximal upper limb fractures such as the head of the humerus when falling. In comparison when the righting reflexes are intact they will extend their arms to break their falls and hence tend to have distal fractures, such as Colles writs fractures, and abrasions on the palmar surface of the hand. </p><div class="separator" style="clear: both; text-align: center;"><iframe allowfullscreen="" class="BLOG_video_class" height="266" src="https://www.youtube.com/embed/dzt3NRh7OnA" width="320" youtube-src-id="dzt3NRh7OnA"></iframe></div><div class="separator" style="clear: both; text-align: center;"><br /></div><b>Level 1</b>: other doctors and medical studentsGavin Giovannonihttp://www.blogger.com/profile/03634514099871112077noreply@blogger.com0tag:blogger.com,1999:blog-895935989949155387.post-84592044189913424662020-09-18T08:53:00.007+01:002020-09-20T19:41:23.235+01:00Primary motor cortex<div class="separator"><div style="margin-left: 1em; margin-right: 1em;"><b>Question</b>: Why do lesions of the primary motor cortex result in flaccid paralysis? I thought upper motor neurone (UMN) lesions resulted in spastic paralysis, is it an exception?<br /><br />The observation that lesions of the primary motor cortex cause flaccid paralysis is based on 'pure lesional' studies in animals (dogs and primates) and date back to Penfield in the 1950s. It is very rare to see pure motor cortex lesion in clinical medicine; motor lesions usually extend beyond the motor cortex to involve adjacent areas, connecting fibre tracts and deep grey matter structures such as the basal ganglia and thalamus. It is these other systems that contribute to increased tone.</div><div style="margin-left: 1em; margin-right: 1em;"><br /></div><div style="margin-left: 1em; margin-right: 1em;">Please note that the increased tone compatible with an upper motor neurone lesion typically evolves over days to weeks after a new lesion. In the acute stage, i.e. within the first few hours to a few days, after a UMN lesion tone may be reduced or flaccid. This is particularly prominent with acute spinal cord lesions and in this context is referred to as spinal shock. <br /><br /><table align="center" cellpadding="0" cellspacing="0" class="tr-caption-container" style="margin-left: auto; margin-right: auto;"><tbody><tr><td style="text-align: center;"><img border="0" data-original-height="229" data-original-width="250" height="487" src="https://1.bp.blogspot.com/-rzZThN1XA1E/X2RmjV49XvI/AAAAAAAAjQ4/h_KrxNxICZIqPis6JVf6QV5NOoPc-CaBQCLcBGAsYHQ/w532-h487/250px-Human_motor_cortex.jpg" style="margin-left: auto; margin-right: auto;" width="532" /></td></tr><tr><td class="tr-caption" style="text-align: center;">Image from <a href="https://en.wikipedia.org/wiki/Motor_cortex" target="_blank">Wikipedia</a><br /></td></tr></tbody></table><br />R. Chris Miall. <a href="https://link.springer.com/referenceworkentry/10.1007%2F978-1-4614-1997-6_128" target="_blank">Cortical Motor Control</a>. Neuroscience in the 21st Century pp 1187-1208.<br /><br />The localization of motor functions within the cerebral cortex has a long history, dating back to the demonstration in 1870 by Fritsch and Hitzig that weak electrical stimulation of the cortex of the dog could evoke movements of the contralateral limbs. At about the same time, Hughlings Jackson made careful clinical observations of the convolutions of epileptic patients, including his own wife. Epileptics often have a spasm of the muscles that may spread sequentially from, for example, the fingers up the arm to the shoulder. Hughlings Jackson realized that the March of the seizures along the limb (Jacksonian March) might reflect some physiological event sweeping across a topographical map of the body within the brain. These ideas were later confirmed by experiments, initially on dogs and monkeys, in which the cortex was stimulated with brief electric shocks. In 1906, Sherrington showed that movements could be evoked most easily from an area now known as the primary motor cortex, and this finding was extended by Penfield in the 1950s, who demonstrated during brain surgery on epileptic patients that the body was topographically mapped on the surface of the human motor cortex (Fig. 37.1). More recently, the unknown events that Hughlings Jackson predicted were shown to be waves of neuronal activation which spread across this topographical map.</div><div style="margin-left: 1em; margin-right: 1em;"><br /></div><div style="margin-left: 1em; margin-right: 1em;"><b>Level 1</b>: other doctors and medical students</div></div>Gavin Giovannonihttp://www.blogger.com/profile/03634514099871112077noreply@blogger.com0tag:blogger.com,1999:blog-895935989949155387.post-77658731513410921412016-12-21T16:23:00.001+00:002016-12-21T16:23:29.945+00:00Dermatomyositis<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span lang="EN-GB" style="font-family: Calibri; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">Case<o:p></o:p></span></u></b></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span lang="EN-GB" style="font-family: Calibri; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;"><br /></span></u></b></div>
<div class="MsoNormal">
<span lang="EN-GB" style="font-family: Calibri; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">A 72 year-old female presented in
June 2015 with rash on her hands, face and around her nostrils. She was
referred to dermatologist, skin biopsy was performed and showed lichen planus,
which was subsequently treated with topical steroids and emollients.<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="font-family: Calibri; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">5 months later rash recurred,
spreading to the arms and torso. She developed progressive myalgia affecting
the neck and proximal limb muscles in combination with progressive swallowing difficulty.
Mobility deteriorated such that she started to fall in the community and was
unable to perform activities of daily living independently.<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="font-family: Calibri; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">After dermatological review polymyalgia
rheumatic was suspected for which she was started on 20 mg prednisolone.
Despite this, two months later she was unable to stand or sit without
assistance. Further deterioration in swallow resulted in presentation to Whipps
Cross Hospital and subsequent transfer to Royal London Hospital.<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="font-family: Calibri; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">Interview revealed no significant
past medical history except for idiopathic thrombocytopaenia and longstanding
osteoarthritis. She was on no regular medication and had no history of alcohol
use or smoking.<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
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<div class="MsoNormal">
<span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">On examination she
was short of breath (FVC<0.9) and failed both bedside and formal swallow
assessments. There was a heliotrope rash and Gottron’s papules as well as
erythematous rash on left elbow, torso and behind both ears. There were dry
mucous membranes (eyes and mouth). Weakness was pronounced in the proximal
muscle groups with distal sparing. She was areflexic, sensation was intact.<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;"><br /></span></div>
<div class="separator" style="clear: both; text-align: center;">
<a href="https://2.bp.blogspot.com/-vopkEib_kbc/WFqrXCm44JI/AAAAAAAAaZc/7UKn_OIHvcU_7KyBLR0R5xktbQmwJbH8QCLcB/s1600/Untitled.png" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="198" src="https://2.bp.blogspot.com/-vopkEib_kbc/WFqrXCm44JI/AAAAAAAAaZc/7UKn_OIHvcU_7KyBLR0R5xktbQmwJbH8QCLcB/s320/Untitled.png" width="320" /></a></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">Investigations<o:p></o:p></span></u></b></div>
<div class="MsoNormal">
<span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">Blood results showed
normal ESR and CRP with increased CK of 900, ANA 1/160 and ENA negative. <o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">MRI head and whole
spine showed increased signal within the neck and paraspinal muscles.<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">Nerve conduction
studies showed incidental mild left carpal tunnel syndrome, whilst needle EMG
sampling revealed no spontaneous activity in any of the muscles sampled.
However, voluntary activated motor units were markedly polypahsic and
short in duration with evidence of early recruitment. <o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">Muscle MRI showed increased
signal in relation to the musculature of the pelvic girdle<span style="mso-spacerun: yes;"> </span>and within the thigh musculature
bilaterally, which would support the diagnosis of myositis.<o:p></o:p></span></div>
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<div class="MsoNormal">
<span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">Muscle biopsy showed
widespread upregulation of MHC Class I, fibre necrosis with prominent
macrophagic (and milder lymphocytic) infiltration as well as fibre
regeneration.<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><span lang="EN-GB" style="font-family: Calibri; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">Diagnosis</span></b><span lang="EN-GB" style="font-family: Calibri; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;"> <o:p></o:p></span></div>
<div class="MsoNormal">
<span lang="EN-GB" style="font-family: Calibri; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">Inflammatory myopathy with
dermatological involvement (Dermatomyositis)<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><span lang="EN-GB" style="font-family: Calibri; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">Next steps<o:p></o:p></span></b></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><span lang="EN-GB" style="font-family: Calibri; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">The priority was to </span></b><span lang="EN-GB" style="font-family: Calibri; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">excluding
underlying malignancy.<b style="mso-bidi-font-weight: normal;"><o:p></o:p></b></span></div>
<div class="MsoNormal">
<span lang="EN-GB" style="font-family: Calibri; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">CXR, CT CAP and Mammogram were
performed.<o:p></o:p></span></div>
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<div class="MsoNormal">
<span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">CT CAP showed early
bronchiectatic changes in the left lung base but no malignancy. Mammogram
showed a dense, nodular background pattern with scattered benign
calcifications.<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="separator" style="clear: both; text-align: center;">
<a href="https://4.bp.blogspot.com/-lKiRoEt8Wf4/WFqrnRcu8-I/AAAAAAAAaZo/2bIDR_XF3oYKFUlxMBvXhzC7ndPmuPYOACLcB/s1600/Untitled.png" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="320" src="https://4.bp.blogspot.com/-lKiRoEt8Wf4/WFqrnRcu8-I/AAAAAAAAaZo/2bIDR_XF3oYKFUlxMBvXhzC7ndPmuPYOACLcB/s320/Untitled.png" width="220" /></a></div>
<div class="MsoNormal">
<span lang="EN-GB" style="font-family: Calibri; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">PET revealed mildly increased
uptake in the muscles and basal pleural thickening.<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="font-family: Calibri; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">An extended ENA panel including </span><span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">HMGCR antibodies were negative. <b style="mso-bidi-font-weight: normal;">Anti-TIF1 gamma antibody was positive.<o:p></o:p></b></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">Treatment:<o:p></o:p></span></u></b></div>
<div class="MsoNormal">
<span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">The focus of acute
treatment was to control the inflammatory drive leading to muscle damage. As
such we administered 3 days of IV methylprednisolone followed by 60 mg
prednisolone. One week later she was given four days of IV immunoglobulins. She
also received IV cyclophosphamide. <o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">For maintenance
therapy, methotrexate was given with folic acid, PPI cover and bone protection.<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">Owing to the
swallowing difficulties a radiological guided gastrostomy tube was inserted for
nutritional support.<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">She was discharged to
an inpatient rehabilitation unit and finally to home.<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">Outcome:<o:p></o:p></span></u></b></div>
<div class="MsoNormal">
<span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">Since August 2016
there are no further dermatological manifestations and she remains rash
free.<span style="mso-spacerun: yes;"> </span>There has been ongoing
improvement in her muscle strength such that she is able to rise from a chair
without the use of her arms and is independently mobile. Swallow has improved
such that she is meeting her nutritional needs orally and no longer requires
supplementation via gastrostomy tube. <o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">Unfortunately just 3
months after last clinic review she developed severe and rapidly progressive
abdominal swelling and large bilateral PEs. She has now been diagnosed with
ovarian teratoma, not previously evident on malignancy screen<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">Learning Points:<o:p></o:p></span></u></b></div>
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<br /></div>
<div class="MsoListParagraphCxSpFirst" style="mso-list: l0 level1 lfo1; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-bidi-font-family: Calibri; mso-bidi-theme-font: major-latin; mso-fareast-font-family: Calibri; mso-fareast-theme-font: major-latin; mso-hansi-theme-font: major-latin;"><span style="mso-list: Ignore;">1)<span style="font: 7.0pt "Times New Roman";">
</span></span></span><!--[endif]--><span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">Dermatomyositis
is a multisystem disorder.<o:p></o:p></span></div>
<div class="MsoListParagraphCxSpMiddle" style="mso-list: l0 level1 lfo1; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-bidi-font-family: Calibri; mso-bidi-theme-font: major-latin; mso-fareast-font-family: Calibri; mso-fareast-theme-font: major-latin; mso-hansi-theme-font: major-latin;"><span style="mso-list: Ignore;">2)<span style="font: 7.0pt "Times New Roman";">
</span></span></span><!--[endif]--><span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">Early
recognition of myositis and treatment can lead to excellent outcomes<o:p></o:p></span></div>
<div class="MsoListParagraphCxSpLast" style="mso-list: l0 level1 lfo1; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-bidi-font-family: Calibri; mso-bidi-theme-font: major-latin; mso-fareast-font-family: Calibri; mso-fareast-theme-font: major-latin; mso-hansi-theme-font: major-latin;"><span style="mso-list: Ignore;">3)<span style="font: 7.0pt "Times New Roman";">
</span></span></span><!--[endif]--><span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">TIF1
Gamma antibody is strongly associated with malignancy. The dermatological and
muscle disease can pre-date malignancy by 2 years or more.<o:p></o:p></span></div>
<div class="MsoNormal" style="margin-left: 18.0pt;">
<br /></div>
<div class="MsoNormal">
<br /></div>
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<b style="mso-bidi-font-weight: normal;"><u><span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">Discussion<o:p></o:p></span></u></b></div>
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<div class="separator" style="clear: both; text-align: center;">
<a href="https://1.bp.blogspot.com/-u9lRob1eauI/WFqrwUfJLRI/AAAAAAAAaZw/baUsQnQfomsuIIO-9AKjfOZufMnjzmUhQCLcB/s1600/Untitled.png" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="129" src="https://1.bp.blogspot.com/-u9lRob1eauI/WFqrwUfJLRI/AAAAAAAAaZw/baUsQnQfomsuIIO-9AKjfOZufMnjzmUhQCLcB/s320/Untitled.png" width="320" /></a></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">Malignancy<o:p></o:p></span></u></b></div>
<div class="MsoNormal">
<span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">Dermatomyositis and
Polymositis are strongly associated with malignant disease, particularly
ovarian, cervix, lung, pancreatic, stomach, colorectal and non-Hodgkin
lymphoma. In both, incidence of malignant disease is highest at time of
myositis diagnosis and for one year afterwards but may arise before, or even
> 5 years after diagnosis.<a href="https://www.blogger.com/blogger.g?blogID=895935989949155387#_ftn1" name="_ftnref" style="mso-footnote-id: ftn;" title=""><span class="MsoFootnoteReference"><span style="mso-special-character: footnote;"><!--[if !supportFootnotes]-->[1]<!--[endif]--></span></span></a><o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">Predictors for low
risk (ie good prognostic signs) include, interstitial lung disease, Anti-Mi2 or
Antisynthethase. Anti-</span><span lang="EN-GB" style="font-family: Calibri; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">TIF1-gamma
antibody (like in the case of the patient described), </span><span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">Nuclear matrix protein (NMX), older age</span><span lang="EN-GB" style="font-family: Calibri; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;"> and d</span><span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">ysphagia are associated with increased risk of malignancy and are thus
poor prognostic signs.</span><span lang="EN-GB" style="font-family: Calibri; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;"><o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">TIF1-gamma<o:p></o:p></span></u></b></div>
<div class="MsoNormal">
<span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">Recently, it has been
revealed that an anti–transcriptional intermediary factor 1 g antibody
(TIF1-g-Ab) is frequently detected in the sera of patients with cancer
associated myositis (CAM).<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">Because TIF1-g
regulates the tumor growth factor b pathway, it is reported to be related to
tumor growth in some malignancies, and may therefore be involved in the key biological
mechanisms linking cancers and myositis.<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">Treatment<o:p></o:p></span></u></b></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">Despite the lack of
placebo-controlled trials, glucocorticoids are considered the mainstay of
initial treatment for idiopathic inflammatory myopathy and myositis-associated
interstitial lung disease.<span style="mso-spacerun: yes;">
</span>First-line conventional immunosuppressive drugs include either
methotrexate or azathioprine, and when they fail, more aggressive therapy
includes mycophenolate mofetil, tacrolimus or cyclosporine, intravenous
immunoglobulin, rituximab, or cyclophosphamide.<a href="https://www.blogger.com/blogger.g?blogID=895935989949155387#_ftn2" name="_ftnref" style="mso-footnote-id: ftn;" title=""><span class="MsoFootnoteReference"><span style="mso-special-character: footnote;"><!--[if !supportFootnotes]-->[2]<!--[endif]--></span></span></a>
<o:p></o:p></span></div>
<div class="MsoNormal">
<span lang="EN-GB" style="font-family: Calibri; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">IVIg demonstrated efficacy in DM
in a double-blind, controlled trial in 15 patients with refractory DM. In
another prospective open-label trial with 35 patients with PM, treatment with
IVIg was associated with a significant clinical improvement in 70% of the
patients, with stable efficacy reported in half of the patients, 3 years after
stopping IVIg. <a href="https://www.blogger.com/blogger.g?blogID=895935989949155387#_ftn3" name="_ftnref" style="mso-footnote-id: ftn;" title=""><span class="MsoFootnoteReference"><span style="mso-special-character: footnote;"><!--[if !supportFootnotes]-->[3]<!--[endif]--></span></span></a><a href="https://www.blogger.com/null" name="_GoBack"></a><o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">IM is a multisystem
disorder and concern should be given to extramuscular aspects of the disease,
particularly the potential for respiratory and cardiac involvement. <o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;">Also of relevance is
the occurrence of other complicating issues: pain, fatigue, dysphagia and
depression.<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span style="font-family: Calibri; mso-ansi-language: EN-US; mso-ascii-theme-font: major-latin; mso-hansi-theme-font: major-latin;"><o:p>Written by Dominika Raciborska & Rachelle Shafei</o:p></span></div>
<div style="mso-element: footnote-list;">
<hr align="left" size="1" width="33%" />
<!--[endif]-->
<div id="ftn" style="mso-element: footnote;">
<div class="MsoFootnoteText">
<a href="https://www.blogger.com/blogger.g?blogID=895935989949155387#_ftnref" name="_ftn1" style="mso-footnote-id: ftn;" title=""><span class="MsoFootnoteReference"><span lang="EN-GB"><span style="mso-special-character: footnote;"><!--[if !supportFootnotes]-->[1]<!--[endif]--></span></span></span></a><span lang="EN-GB"> </span><span style="mso-ansi-language: EN-US;">Chinoy et al. The
diagnostic utility of myositis autoantibody testing for predicting the risk of
cancer-associated myositis. Ann Rheum Dis 2007; 66 (10):1345<o:p></o:p></span></div>
<div class="MsoFootnoteText">
<br /></div>
</div>
<div id="ftn" style="mso-element: footnote;">
<div class="MsoFootnoteText">
<a href="https://www.blogger.com/blogger.g?blogID=895935989949155387#_ftnref" name="_ftn2" style="mso-footnote-id: ftn;" title=""><span class="MsoFootnoteReference"><span lang="EN-GB"><span style="mso-special-character: footnote;"><!--[if !supportFootnotes]-->[2]<!--[endif]--></span></span></span></a><span lang="EN-GB"> </span><span style="mso-ansi-language: EN-US;">Siamak Moghadam-Kia,
Rohit Aggarwal, and Chester V Oddis. Treatment of inflammatory myopathy:
emerging therapies and therapeutic targets. Expert Rev Clin Immunol. 2015;
11(11): 1265–1275.<o:p></o:p></span></div>
<div class="MsoFootnoteText">
<br /></div>
</div>
<div id="ftn" style="mso-element: footnote;">
<div class="MsoFootnoteText">
<a href="https://www.blogger.com/blogger.g?blogID=895935989949155387#_ftnref" name="_ftn3" style="mso-footnote-id: ftn;" title=""><span class="MsoFootnoteReference"><span lang="EN-GB"><span style="mso-special-character: footnote;"><!--[if !supportFootnotes]-->[3]<!--[endif]--></span></span></span></a><span lang="EN-GB"> </span><span style="mso-ansi-language: EN-US;">Dalakas MC, Illa I,
Dambrosia JM, et al. A controlled trial of high-dose intravenous immune
globulin infusions as treatment for dermatomyositis. N Engl J Med.
1993;329(27):1993–2000<o:p></o:p></span></div>
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Anonymoushttp://www.blogger.com/profile/08062870670799646040noreply@blogger.com0tag:blogger.com,1999:blog-895935989949155387.post-89677341924245841962016-10-14T14:02:00.000+01:002016-10-14T14:02:53.855+01:00Anterior choroidal artery syndrome<div dir="ltr" style="text-align: left;" trbidi="on">
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">We saw a 68-year old lady on the ward. She had no
past medical history of note.</span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">She presented to hospital with a self-terminating
generalised tonic-clonic (GTCS) and in addition was found to have a dense right
hemiplegia and expressive dysphasia. The presentation was preceded by a 3-4
week history of increasing confusion and disorientation.<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><br /></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><b style="mso-bidi-font-weight: normal;">On examination </b>she
appeared alert but had significant expressive dysphasia<b style="mso-bidi-font-weight: normal;"><o:p></o:p></b></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">She was consistently following instructions: 2 and 3 stage
commands. There was a subtle ptosis on the left and unequal pupil size. No
papilloedema. (Horner’s)<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">There was a small recent Neurosurgical scar in the right
frontal region and a dense right hemiplegia. On further inspection there was wasting
of FDIO on the right as well as heberden’s nodes & mild ulnar deviation of
the digits. On palpation there was arthralgia in small muscles of the hands,
ankle and knee on the right (- ?OA ?RA)<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">Auscultation of the heart was normal (with normal
echocardiogram) and there were no other systemic features (no fever,
lymphadenopathy or rash)<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><br /></span></div>
<div class="MsoNormal">
<u><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">Differentials based on clinical findings:</span></u></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">Most likely differential is a vasculitic syndrome leading to
ischaemia in the territory of <span lang="EN-US" style="color: black; mso-ansi-language: EN-US; mso-bidi-font-family: Helvetica;">anterior choroidal artery</span>,
MCA or internal carotid artery. VZV vasculitis is in the differential as is primary CNS angiitis<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">Angiocentric lymphoma<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">Vasculitis secondary to systemic disease such as rheumatoid
arthritis<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">PML could cause this but is usually more indolent<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">Susacs unlikely as there is no history of headache or
hearing deficit<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">PML would typically follow a more indolent course <o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<u><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">Learning points highlighted in discussion of the case:<o:p></o:p></span></u></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><br /></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">1. <b style="mso-bidi-font-weight: normal;">Differentials for
confusion/delirium:<o:p></o:p></b></span></div>
<div class="MsoListParagraphCxSpFirst" style="mso-list: l0 level1 lfo2; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><span style="font-family: "palatino linotype"; mso-bidi-font-family: "Palatino Linotype"; mso-fareast-font-family: "Palatino Linotype";"><span style="mso-list: Ignore;">-<span style="font-stretch: normal; font-style: normal; font-variant: normal; font-weight: normal; line-height: normal;"> </span></span></span><!--[endif]-->Drugs<o:p></o:p></span></div>
<div class="MsoListParagraphCxSpMiddle" style="mso-list: l0 level1 lfo2; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><span style="font-family: "palatino linotype"; mso-bidi-font-family: "Palatino Linotype"; mso-fareast-font-family: "Palatino Linotype";"><span style="mso-list: Ignore;">-<span style="font-stretch: normal; font-style: normal; font-variant: normal; font-weight: normal; line-height: normal;"> </span></span></span><!--[endif]-->Dementia
(DLB)<o:p></o:p></span></div>
<div class="MsoListParagraphCxSpMiddle" style="mso-list: l0 level1 lfo2; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><span style="font-family: "palatino linotype"; mso-bidi-font-family: "Palatino Linotype"; mso-fareast-font-family: "Palatino Linotype";"><span style="mso-list: Ignore;">-<span style="font-stretch: normal; font-style: normal; font-variant: normal; font-weight: normal; line-height: normal;"> </span></span></span><!--[endif]-->Infection
(urine and chest most commonly)<o:p></o:p></span></div>
<div class="MsoListParagraphCxSpMiddle" style="mso-list: l0 level1 lfo2; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><span style="font-family: "palatino linotype"; mso-bidi-font-family: "Palatino Linotype"; mso-fareast-font-family: "Palatino Linotype";"><span style="mso-list: Ignore;">-<span style="font-stretch: normal; font-style: normal; font-variant: normal; font-weight: normal; line-height: normal;"> </span></span></span><!--[endif]-->Head
trauma (look for rhinorrhoea, evidence of previous neurosurgery, evidence <o:p></o:p></span></div>
<div class="MsoListParagraphCxSpMiddle" style="mso-list: l0 level1 lfo2; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><span style="font-family: "palatino linotype"; mso-bidi-font-family: "Palatino Linotype"; mso-fareast-font-family: "Palatino Linotype";"><span style="mso-list: Ignore;">-<span style="font-stretch: normal; font-style: normal; font-variant: normal; font-weight: normal; line-height: normal;"> </span></span></span><!--[endif]-->Hypoxia
(ABG)<o:p></o:p></span></div>
<div class="MsoListParagraphCxSpMiddle" style="mso-list: l0 level1 lfo2; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><span style="font-family: "palatino linotype"; mso-bidi-font-family: "Palatino Linotype"; mso-fareast-font-family: "Palatino Linotype";"><span style="mso-list: Ignore;">-<span style="font-stretch: normal; font-style: normal; font-variant: normal; font-weight: normal; line-height: normal;"> </span></span></span><!--[endif]-->Psychiatric
<o:p></o:p></span></div>
<div class="MsoListParagraphCxSpMiddle" style="mso-list: l0 level1 lfo2; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><span style="font-family: "palatino linotype"; mso-bidi-font-family: "Palatino Linotype"; mso-fareast-font-family: "Palatino Linotype";"><span style="mso-list: Ignore;">-<span style="font-stretch: normal; font-style: normal; font-variant: normal; font-weight: normal; line-height: normal;"> </span></span></span><!--[endif]-->Seizures
(non-convulsive, may be secondary to either frontal or temporal seizures.
Typically confusion would be fluctuant)<o:p></o:p></span></div>
<div class="MsoListParagraphCxSpMiddle" style="mso-list: l0 level1 lfo2; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><span style="font-family: "palatino linotype"; mso-bidi-font-family: "Palatino Linotype"; mso-fareast-font-family: "Palatino Linotype";"><span style="mso-list: Ignore;">-<span style="font-stretch: normal; font-style: normal; font-variant: normal; font-weight: normal; line-height: normal;"> </span></span></span><!--[endif]-->Space
occupying lesion (papilloedema)<o:p></o:p></span></div>
<div class="MsoListParagraphCxSpLast" style="mso-list: l0 level1 lfo2; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><span style="font-family: "palatino linotype"; mso-bidi-font-family: "Palatino Linotype"; mso-fareast-font-family: "Palatino Linotype";"><span style="mso-list: Ignore;">-<span style="font-stretch: normal; font-style: normal; font-variant: normal; font-weight: normal; line-height: normal;"> </span></span></span><!--[endif]-->Metabolic
(Renal failure, Liver failure, electrolyte abnormality)<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><br /></span></div>
<div class="MsoNormal">
<o:p><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><br /></span></o:p></div>
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<b style="mso-bidi-font-weight: normal;"><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">2. The pathway of the
internal carotid artery:<o:p></o:p></span></b></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">Enters the cranium through the Foramen lacerum, then travels
through the<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">Cavernous sinus. It then turns to travel under the anterior
clinoid process emerging just below and posterior to the optic canal. The
internal carotid artery finally emerges through the dura just beneath the optic
nerve. <o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">There are 5 terminal branches: MCA, ACA, ophthalmic,
posterior communicating and anterior choroidal artery. The ophthalmic artery
supplies the contents of the orbit and continues forward to supply the central
part of the forehead.<o:p></o:p></span></div>
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<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><br /></span></div>
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<a href="https://1.bp.blogspot.com/-fWu_j4LGi94/V_EVXdpqjJI/AAAAAAAAAJQ/3YrK7_njPeIo9k09z9DV2gDTuKh4pAf-QCLcB/s1600/anterior%2Bchoroidal%2Bartery.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><img border="0" height="268" src="https://1.bp.blogspot.com/-fWu_j4LGi94/V_EVXdpqjJI/AAAAAAAAAJQ/3YrK7_njPeIo9k09z9DV2gDTuKh4pAf-QCLcB/s320/anterior%2Bchoroidal%2Bartery.jpg" width="320" /></span></a></div>
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<o:p><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><br /></span></o:p></div>
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<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><span lang="EN-US" style="mso-ansi-language: EN-US; mso-no-proof: yes;"><!--[if gte vml 1]><v:shapetype id="_x0000_t75" coordsize="21600,21600"
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<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><br /></span></div>
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<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><br /></span></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">3. Syndromes related
to anterior choroidal artery infarction<o:p></o:p></span></b></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">Most commonly patients present with a lacunar syndrome (85%)
but there are case studies of confusion and aphasia, presumably where the superficial
territory is involved, leading to cortical deficits.<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><br /></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">Further reading: <span lang="EN-US" style="color: black; mso-ansi-language: EN-US; mso-bidi-font-family: Helvetica;">Palomeras E, Fossas P, Cano AT,
Sanz P, Floriach M. Anterior choroidal artery infarction: a clinical, etiologic
and prognostic study. Acta Neurol Scand 2008: 118: 42–47</span><o:p></o:p></span></div>
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<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><br /></span></div>
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<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><br /></span></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">4. Horner syndrome<o:p></o:p></span></b></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">Classically the patient will have ptosis, miosis and
anhidrosis. There may be mild enophthalmos secondary to lid sagging. There is
also increased amplitude of accommodation. Acute features of sympathetic
disruption include ipsilateral conjunctival injection and nasal stuffiness. <o:p></o:p></span></div>
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<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><br /></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">The precise clinical manifestation depends upon the site of
the lesion along the three-neuron sympathetic (adrenergic) pathway, that
originates in the hypothalamus:<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><br /></span></div>
<div class="MsoListParagraphCxSpFirst" style="mso-list: l1 level1 lfo1; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><span style="font-family: "palatino linotype"; mso-bidi-font-family: "Palatino Linotype"; mso-fareast-font-family: "Palatino Linotype";"><span style="mso-list: Ignore;">-<span style="font-stretch: normal; font-style: normal; font-variant: normal; font-weight: normal; line-height: normal;"> </span></span></span><!--[endif]--><u>1<sup>st</sup>
order neuron</u>: descends caudally from hypothalamus to the first synapse located
in the spinal cord (levels C8-T2). Causes include stroke, demyelination,
pituitary or base of skull tumours, basal meningitis, neck trauma,
syringomyelia, Arnold chiari malformation and spinal cord tumours.<o:p></o:p></span><br />
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><br /></span></div>
<div class="MsoListParagraphCxSpMiddle" style="mso-list: l1 level1 lfo1; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><span style="font-family: "palatino linotype"; mso-bidi-font-family: "Palatino Linotype"; mso-fareast-font-family: "Palatino Linotype";"><span style="mso-list: Ignore;">-<span style="font-stretch: normal; font-style: normal; font-variant: normal; font-weight: normal; line-height: normal;"> </span></span></span><!--[endif]--><u>2<sup>nd</sup>
order neuron</u>: travels from sympathetic trunk, through the brachial plexus, over
the lung apex and ascends to the superior ganglion, located near the angle of
the mandible and bifurcation of the common carotid artery. Causes include
apical lung tumours (eg. Pancoast’s), lymphadenopathy (lymphoma, leukaemia,
TB), lower brachial plexus trauma, common carotid or subclavian aneurysm,
neuroblastoma or mandibular dental abscess.<o:p></o:p></span><br />
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><br /></span></div>
<div class="MsoListParagraphCxSpLast" style="mso-list: l1 level1 lfo1; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><span style="font-family: "palatino linotype"; mso-bidi-font-family: "Palatino Linotype"; mso-fareast-font-family: "Palatino Linotype";"><span style="mso-list: Ignore;">-<span style="font-stretch: normal; font-style: normal; font-variant: normal; font-weight: normal; line-height: normal;"> </span></span></span><!--[endif]--><u>3<sup>rd</sup>
order neuron</u>: ascends within the adventitia of the internal carotid artery,
through the cavernous sinous in close relation to CN VI. The oculosympathetic
pathway then joins V1. In the orbit the fibres innervate the iris dilator
muscle as well as Muller’s muscle (responsible for a small proportion of upper
lid elevation and lower lid retraction). This innervation accounts for the
minor ptosis (<2mm). Anhidrosis is not a feature of 3<sup>rd</sup> order
lesions as the sympathetic fibre responsible for sweating and vasodilation
branch off at the superior cervical ganglion. Causes include cluster headache
or migraine, herpes zoster infection, internal carotid artery dissection,
carotid-cavernous fistula and temporal arteritis.<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><br /></span></div>
<div class="MsoNormal">
<u><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">Neurological signs can help to localise the lesion:<o:p></o:p></span></u></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">diplopia, vertigo, ataxia, lateralised weakness <span style="font-family: "wingdings"; mso-ascii-font-family: Cambria; mso-ascii-theme-font: minor-latin; mso-char-type: symbol; mso-hansi-font-family: Cambria; mso-hansi-theme-font: minor-latin; mso-symbol-font-family: Wingdings;"><span style="mso-char-type: symbol; mso-symbol-font-family: Wingdings;">à</span></span><b style="mso-bidi-font-weight: normal;"> Brainstem</b><o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">bilat/ipsilat weakness, long tract signs, sensory level,
bladder or bowel involvement <span style="font-family: "wingdings"; mso-ascii-font-family: Cambria; mso-ascii-theme-font: minor-latin; mso-char-type: symbol; mso-hansi-font-family: Cambria; mso-hansi-theme-font: minor-latin; mso-symbol-font-family: Wingdings;"><span style="mso-char-type: symbol; mso-symbol-font-family: Wingdings;">à</span></span>
<b style="mso-bidi-font-weight: normal;">Myelopathy</b><o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">Arm pain/weakness <span style="font-family: "wingdings"; mso-ascii-font-family: Cambria; mso-ascii-theme-font: minor-latin; mso-char-type: symbol; mso-hansi-font-family: Cambria; mso-hansi-theme-font: minor-latin; mso-symbol-font-family: Wingdings;"><span style="mso-char-type: symbol; mso-symbol-font-family: Wingdings;">à</span></span>
<b style="mso-bidi-font-weight: normal;">brachial plexus or lung apex</b><o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">Ipsilateral ocular paresis & CN VI palsy, no brainstem
signs <span style="font-family: "wingdings"; mso-ascii-font-family: Cambria; mso-ascii-theme-font: minor-latin; mso-char-type: symbol; mso-hansi-font-family: Cambria; mso-hansi-theme-font: minor-latin; mso-symbol-font-family: Wingdings;"><span style="mso-char-type: symbol; mso-symbol-font-family: Wingdings;">à</span></span> <b style="mso-bidi-font-weight: normal;">cavernous sinus</b><o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">Isolated honers with neck pain <b style="mso-bidi-font-weight: normal;"><span style="font-family: "wingdings"; mso-ascii-font-family: Cambria; mso-ascii-theme-font: minor-latin; mso-char-type: symbol; mso-hansi-font-family: Cambria; mso-hansi-theme-font: minor-latin; mso-symbol-font-family: Wingdings;"><span style="mso-char-type: symbol; mso-symbol-font-family: Wingdings;">à</span></span>internal
carotid artery dissection</b></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><br /></span></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">5. Neurological
manifestations of rheumatoid arthritis<o:p></o:p></span></b></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><br /></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">Manifestations may be either central or peripheral and
related to the disease itself or disease modifying treatment. <o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><br /></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">CNS manifestations: cervical myelopathy (secondary to
atlantoaxial subluxation), vasculitis, rheumatoid nodules within the CNS,
meningitis and rarely progressive multifocal leucoencephalopathy (risk
increased after rituximab therapy) and a hyperviscosity syndrome. Stroke also
occurs with increased frequency<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><br /></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">PNS manifestations: compression neuropathies (most common,
secondary to joint deformities, inflamed synovium, ligaments or compressive
tendon sheaths), distal sensory neuropathy, sensori-motor neuropathy or
autonomic neuropathy (thought to be secondary to vasculitic process)<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><br /></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">Neuromuscular manifestations: myopathy, disuse atrophy,
denervation atrophy, myositis.<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><br /></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">RA may rarely be complicated by secondary amyloidosis.<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><br /></span></div>
<div class="MsoNormal">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">Drugs effects:<o:p></o:p></span></div>
<div class="bulletindent1" style="background: white; line-height: 15.1pt; margin-bottom: 1.5pt; margin-left: 24.0pt; margin-right: 0cm; margin-top: 1.5pt;">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><span class="glyph"><span style="color: black; font-size: 12.0pt;">●</span></span><span style="color: black; font-size: 12.0pt;">Nonsteroidal antiinflammatory drugs (NSAIDs) </span><span style="color: black; font-size: 12.0pt;"><span style="mso-char-type: symbol; mso-symbol-font-family: Wingdings;">à</span></span><span style="color: black; font-size: 12.0pt;"> headaches, drowsiness, and aseptic meningitis<o:p></o:p></span></span></div>
<div class="bulletindent1" style="background: white; line-height: 15.1pt; margin-bottom: 1.5pt; margin-left: 24.0pt; margin-right: 0cm; margin-top: 1.5pt;">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><span class="glyph"><span style="color: black; font-size: 12.0pt;">●</span></span><span style="color: black; font-size: 12.0pt;">Glucocorticoids </span><span style="color: black; font-size: 12.0pt;"><span style="mso-char-type: symbol; mso-symbol-font-family: Wingdings;">à</span></span><span style="color: black; font-size: 12.0pt;">
myopathy, depression, psychosis & intracranial hypertension<o:p></o:p></span></span></div>
<div class="bulletindent1" style="background: white; line-height: 15.1pt; margin-bottom: 1.5pt; margin-left: 24.0pt; margin-right: 0cm; margin-top: 1.5pt;">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><span class="glyph"><span style="color: black; font-size: 12.0pt;">●</span></span><span style="color: black; font-size: 12.0pt;">Gold </span><span style="color: black; font-size: 12.0pt;"><span style="mso-char-type: symbol; mso-symbol-font-family: Wingdings;">à</span></span><span style="color: black; font-size: 12.0pt;"> peripheral neuropathy,
cranial nerve palsies and Guillain-Barré syndr<o:p></o:p></span></span></div>
<div class="bulletindent1" style="background: white; line-height: 15.1pt; margin-bottom: 1.5pt; margin-left: 24.0pt; margin-right: 0cm; margin-top: 1.5pt;">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><span class="glyph"><span style="color: black; font-size: 12.0pt;">●</span></span><span class="glyph"><span style="color: black; font-size: 12.0pt;">Methotrexate</span></span><span class="glyph"><span style="font-size: 12.0pt;">, </span></span><span style="font-size: 12.0pt;">Sulfasalazine<span class="apple-converted-space"> </span>and<span class="apple-converted-space"> </span>leflunomide<span class="apple-converted-space"> </span></span><span style="font-size: 12.0pt;"><span style="mso-char-type: symbol; mso-symbol-font-family: Wingdings;">à</span></span><span style="font-size: 12.0pt;"> headaches. <o:p></o:p></span></span></div>
<div class="bulletindent1" style="background: white; line-height: 15.1pt; margin-bottom: 1.5pt; margin-left: 24.0pt; margin-right: 0cm; margin-top: 1.5pt;">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><span class="glyph"><span style="font-size: 12.0pt;">●</span></span><span style="font-size: 12.0pt;">Leflunomide<span class="apple-converted-space"> </span></span><span style="font-size: 12.0pt;"><span style="mso-char-type: symbol; mso-symbol-font-family: Wingdings;">à</span></span><span style="font-size: 12.0pt;">peripheral<span style="color: black;"> neuropathy<o:p></o:p></span></span></span></div>
<div class="bulletindent1" style="background: white; line-height: 15.1pt; margin-bottom: 1.5pt; margin-left: 24.0pt; margin-right: 0cm; margin-top: 1.5pt;">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><span class="glyph"><span style="color: black; font-size: 12.0pt;">●</span></span><span style="color: black; font-size: 12.0pt;"> anti-TNF therapies </span><span style="color: black; font-size: 12.0pt;"><span style="mso-char-type: symbol; mso-symbol-font-family: Wingdings;">à</span></span><span style="color: black; font-size: 12.0pt;"> increase
the risk of demyelinating disease</span></span></div>
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Anonymoushttp://www.blogger.com/profile/12855758388031782186noreply@blogger.com0tag:blogger.com,1999:blog-895935989949155387.post-20221653210014028522016-10-12T20:40:00.000+01:002016-11-29T08:20:10.343+00:00Chorea acanthocytosis<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span style="font-family: "times new roman"; mso-ansi-language: EN-US;">Chorea-acanthocytosis<o:p></o:p></span></u></b></div>
<div class="MsoNormal">
<br />
2 cases of patients with speech difficulties, vocal ticks, then progressing to choreiform movements and finally to dystonias were investigated.</div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span style="font-family: "times new roman"; mso-ansi-language: EN-US;">Investigations<o:p></o:p></span></u></b></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">Investigations revealed CK of 600-1200.<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">Neurophysiology showed mild sensory and motor axonal neuropathy, whereas
MRI revealed atrophy of caudate and lentiform nucleus.<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="separator" style="clear: both; text-align: center;">
<a href="https://2.bp.blogspot.com/-Vc2MdNNRI38/V_qoiAdhMuI/AAAAAAAAZXU/8UUI9zi3wnAtNe0hMy1_HG3nS9qO2jx5wCLcB/s1600/Untitled.png" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="154" src="https://2.bp.blogspot.com/-Vc2MdNNRI38/V_qoiAdhMuI/AAAAAAAAZXU/8UUI9zi3wnAtNe0hMy1_HG3nS9qO2jx5wCLcB/s320/Untitled.png" width="320" /></a></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span style="font-family: "times new roman"; mso-ansi-language: EN-US;">DaTSCAN showing decreased
uptake in the basal ganglia bilaterally<o:p></o:p></span></u></b></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">In both cases peripheral blood films revealed acanthocytes. 25% in the
first case and 10-70% in the second case.<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">Diagnostic test showed compound heterozygous mutations in exon 4
(c.0237del, pE80KfsX11) and 72 (c.9429_9432del, p.R3143SfsX5) of <i>VPS13A</i><span style="mso-bidi-font-style: italic;"> in case 1 and </span>compound heterozygous
mutations in exon 14 (c.1208_1211del, p.Q403RfsX6) and 56 (c.7867C>T,
p.R2623X) of <i>VPS13A</i><span style="mso-bidi-font-style: italic;"> confirming
the suspected diagnosis of chorea-acanthocytosis.</span><o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span style="font-family: "times new roman"; mso-ansi-language: EN-US;">Discussion<o:p></o:p></span></u></b></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">Chorea acanthocytosis is a rare autosomal recessive disorder affecting ~1000
worldwide and is caused by mutations in <i>VPS13A</i> gene.<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">Clincial features include chorea, oromandibular dystonia (which may be
mutilating) or generalized dystonia, phonic tics, feeding/ tongue protrusion
dystonia, head drops,<span style="mso-spacerun: yes;"> </span>‘rubber man’
gait, seizures, neuropathy and behavioural disturbance (change in personality,
OCD, disinhibition). The latter may be a presenting feature.<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">Diagnosis of chorea-acanthocytosis is primarily clinical with
characteristic MRI findings supplied by evidence of muscle disease. MRI and CT
might show dilatation of anterior horn of lateral ventricles and atrophy of the
caudate nuclei.<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">Peripheral blood film may show acanthocytes in 5-50% of the red cell
population. It has to be noted, however, that in some cases acanthocytosis may
appear later or may be absent altogether.<span style="mso-spacerun: yes;">
</span>Majority of patients will also have increased creatinine kinase (CK), as
exemplified by the two cases described above.<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">Central nuclei and atrophic fibres will be key findings on muscle
biopsy.<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<u><span style="font-family: "times new roman"; mso-ansi-language: EN-US;">There are several causes of neuro-acanthocytosis (oromandibular dystonia
as prominent feature ** yes<span style="mso-spacerun: yes;"> </span>*perhaps)<o:p></o:p></span></u></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">•Chorea-acanthocytosis**<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">•McLeod’s syndrome*<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">•Huntington’s disease-like type 2<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">•Pantothenate kinase associated neurodegeneration (PKAN)*<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">•Hypoprebetalipoproteinaemia, acanthocytosis, retinitis pigmentosa and
pallidal degeneration (HARP) syndrome**<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span lang="EN-GB" style="font-family: "times new roman";">McLeod syndrome<o:p></o:p></span></u></b></div>
<div class="MsoNormal">
<span lang="EN-GB" style="font-family: "times new roman";">McLeod
neuroacanthocytosis syndrome is an X-linked recessive (mutations in XK gene) multisystem
disorder with haematological, hepatological, neuromuscular and central nervous
system involvement in middle-aged males.<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">Cardiomyopathy and conduction abnormalities as well as dystonia and
chorea are a common finding. <o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">Seizures and oromandibular dystonia are, however, less common than in chorea-acanthocytosis.
<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span style="font-family: "times new roman"; mso-ansi-language: EN-US;">Oromandibular
dystonia<o:p></o:p></span></u></b></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">Oromandibular dystonia is characterized by prolonged spasms caused by
contraction of the muscles of the mouth and mandible. It involves the muscles
of facial expression, mastication, tongue and eyelids.<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">It can be drug-induced, caused by structural lesions or encephalitis. It
may also be genetic (e.g. McLeod syndrome, Ataxia-telangiectasia, Wilson’s and
HD).<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span lang="EN-GB" style="font-family: "times new roman";">Summary<o:p></o:p></span></u></b></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="font-family: "times new roman";">The cases
described are examples of chorea- acanthocytosis ( of 21 and 18 year disease
duration respectively).<o:p></o:p></span></div>
<div class="MsoNormal">
<span lang="EN-GB" style="font-family: "times new roman";">Notably,
both patients developed parkinsonism after a decade of disease duration and
both had abnormal DaTSCANs showing nigrostratial denervation.<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">Clinically, progressive parkinsonism appears to evolve in later stages
of chorea- acanthocytosis and gradually replaces the hyperkinetic abnormal
movements, in a manner similar to that observed in Huntington’s disease and
other neurodegenerative causes of chorea. The hypothesis of the nigrostriatal
pathway being gradually involved in the neurodegenerative process is further
supported by the findings of severe loss of dopamine D2-receptor-bearing striatal
neurons and loss of dopaminergic projections from the SN to the posterior
putamen in a PET study (unconfirmed case).<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span style="font-family: "times new roman"; mso-ansi-language: EN-US;">This phenotypal
shift has clinical implications:<o:p></o:p></span></u></b></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">•Withdrawal of neuroleptics and tetrabenazine<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">•L-dopa use may be limited, amantadine reportedly helps gait<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">•DBS may be useful in some cases<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="separator" style="clear: both; text-align: center;">
<a href="https://2.bp.blogspot.com/-qz9Q8MGZ_Gs/V_qovoGA33I/AAAAAAAAZXY/mzvK__4DTbsfT97YBIrJxG0LVgPhQyCggCLcB/s1600/Untitled.png" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="239" src="https://2.bp.blogspot.com/-qz9Q8MGZ_Gs/V_qovoGA33I/AAAAAAAAZXY/mzvK__4DTbsfT97YBIrJxG0LVgPhQyCggCLcB/s320/Untitled.png" width="320" /></a></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span lang="EN-GB" style="font-family: "times new roman";">Useful mnemonic: DEPICTING Chorea<o:p></o:p></span></u></b></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">D – Drug induced<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">E – Endocrine<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">P – Paraneoplastic/polycythaemia vera<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">I – Infectious/immune mediated<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">C – Chorea gravidarum<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">T – Toxic<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">I – Ischaemic<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">N – Neonatal hypoxia<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "times new roman"; mso-ansi-language: EN-US;">G – Genetic<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span lang="EN-GB" style="font-family: "times new roman";">Further Reading<o:p></o:p></span></u></b></div>
<div class="MsoNormal">
<span style="background: white; color: black; font-family: "times new roman";">Bohlega S.
Chorea-acanthocytosis: clinical and genetic findings in three families from the
Arabian peninsula..</span><span style="color: black; font-family: "times new roman"; mso-ansi-language: EN-US;"> </span><i style="mso-bidi-font-style: normal;"><span style="background: white; color: black; font-family: "times new roman";">Mov Disord.</span></i><i style="mso-bidi-font-style: normal;"><span style="color: black; font-family: "times new roman"; mso-ansi-language: EN-US;"> </span></i><span style="background: white; color: black; font-family: "times new roman";">2003; 18(4): 403-407.<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span style="background: white; color: black; font-family: "times new roman";">Baeza V et al.
Chorea-Acanthocytosis..</span><span style="color: black; font-family: "times new roman"; mso-ansi-language: EN-US;"> </span><i style="mso-bidi-font-style: normal;"><span style="background: white; color: black; font-family: "times new roman";">Gene Reviews</span></i><i style="mso-bidi-font-style: normal;"><span style="color: black; font-family: "times new roman"; mso-ansi-language: EN-US;"> </span></i><span style="background: white; color: black; font-family: "times new roman";">2002. <o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="affinlinebook" style="background: white;">
<span style="color: black; font-family: "times new roman"; font-size: 12.0pt;">In:<span class="apple-converted-space"> </span></span><a href="https://www.ncbi.nlm.nih.gov/pubmed?term=Pagon%20RA%5BEditor%5D"><span style="color: #660066; font-family: "times new roman"; font-size: 12.0pt;">Pagon RA</span></a><span style="color: black; font-family: "times new roman"; font-size: 12.0pt;">,<span class="apple-converted-space"> </span></span><a href="https://www.ncbi.nlm.nih.gov/pubmed?term=Adam%20MP%5BEditor%5D"><span style="color: #660066; font-family: "times new roman"; font-size: 12.0pt;">Adam MP</span></a><span style="color: black; font-family: "times new roman"; font-size: 12.0pt;">,<span class="apple-converted-space"> </span></span><a href="https://www.ncbi.nlm.nih.gov/pubmed?term=Ardinger%20HH%5BEditor%5D"><span style="color: #660066; font-family: "times new roman"; font-size: 12.0pt;">Ardinger HH</span></a><span style="color: black; font-family: "times new roman"; font-size: 12.0pt;">,<span class="apple-converted-space"> </span></span><a href="https://www.ncbi.nlm.nih.gov/pubmed?term=Wallace%20SE%5BEditor%5D"><span style="color: #660066; font-family: "times new roman"; font-size: 12.0pt;">Wallace SE</span></a><span style="color: black; font-family: "times new roman"; font-size: 12.0pt;">,<span class="apple-converted-space"> </span></span><a href="https://www.ncbi.nlm.nih.gov/pubmed?term=Amemiya%20A%5BEditor%5D"><span style="color: #660066; font-family: "times new roman"; font-size: 12.0pt;">Amemiya A</span></a><span style="color: black; font-family: "times new roman"; font-size: 12.0pt;">,<span class="apple-converted-space"> </span></span><a href="https://www.ncbi.nlm.nih.gov/pubmed?term=Bean%20LJH%5BEditor%5D"><span style="color: #660066; font-family: "times new roman"; font-size: 12.0pt;">Bean LJH</span></a><span style="color: black; font-family: "times new roman"; font-size: 12.0pt;">,<span class="apple-converted-space"> </span></span><a href="https://www.ncbi.nlm.nih.gov/pubmed?term=Bird%20TD%5BEditor%5D"><span style="color: #660066; font-family: "times new roman"; font-size: 12.0pt;">Bird TD</span></a><span style="color: black; font-family: "times new roman"; font-size: 12.0pt;">,<span class="apple-converted-space"> </span></span><a href="https://www.ncbi.nlm.nih.gov/pubmed?term=Fong%20CT%5BEditor%5D"><span style="color: #660066; font-family: "times new roman"; font-size: 12.0pt;">Fong CT</span></a><span style="color: black; font-family: "times new roman"; font-size: 12.0pt;">,<span class="apple-converted-space"> </span></span><a href="https://www.ncbi.nlm.nih.gov/pubmed?term=Mefford%20HC%5BEditor%5D"><span style="color: #660066; font-family: "times new roman"; font-size: 12.0pt;">Mefford HC</span></a><span style="color: black; font-family: "times new roman"; font-size: 12.0pt;">,<span class="apple-converted-space"> </span></span><a href="https://www.ncbi.nlm.nih.gov/pubmed?term=Smith%20RJH%5BEditor%5D"><span style="color: #660066; font-family: "times new roman"; font-size: 12.0pt;">Smith RJH</span></a><span style="color: black; font-family: "times new roman"; font-size: 12.0pt;">,<span class="apple-converted-space"> </span></span><a href="https://www.ncbi.nlm.nih.gov/pubmed?term=Stephens%20K%5BEditor%5D"><span style="color: #660066; font-family: "times new roman"; font-size: 12.0pt;">Stephens K</span></a><span style="color: black; font-family: "times new roman"; font-size: 12.0pt;">, editors.<span class="apple-converted-space"> </span></span><span style="color: black; font-family: "times new roman"; font-size: 12.0pt;">GeneReviews</span><sup><span style="color: black; font-family: "times new roman"; font-size: 12.0pt;">®</span></sup><span class="apple-converted-space"><span style="color: black; font-family: "times new roman"; font-size: 12.0pt;"> </span></span><span style="color: black; font-family: "times new roman"; font-size: 12.0pt;">[Internet]. Seattle (WA):
University of Washington, Seattle; 1993-2016. 2002 Jun 14 [updated 2014 Jan
30].<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="background: white; color: black; font-family: "times new roman";">Schneider R, Hoffman HT. Oromandibular
dystonia: a clinical report..</span><span style="color: black; font-family: "times new roman"; mso-ansi-language: EN-US;"> </span><i style="mso-bidi-font-style: normal;"><span style="background: white; color: black; font-family: "times new roman";">J Prosthet Dent.</span></i><i style="mso-bidi-font-style: normal;"><span style="color: black; font-family: "times new roman"; mso-ansi-language: EN-US;"> </span></i><span style="background: white; color: black; font-family: "times new roman";">2011; 106(6): 355-358.</span><span style="font-family: "times new roman"; mso-ansi-language: EN-US; mso-bidi-font-size: 10.0pt;"><o:p></o:p></span></div>
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Anonymoushttp://www.blogger.com/profile/08062870670799646040noreply@blogger.com0tag:blogger.com,1999:blog-895935989949155387.post-83252515865757518042016-10-05T18:32:00.000+01:002016-11-29T08:22:45.646+00:00ADEM: sinister repercussions of Mycoplasma infection<div style="direction: ltr; margin-bottom: 0pt; margin-left: .4in; margin-top: 3.0pt; mso-line-break-override: none; punctuation-wrap: hanging; text-align: left; text-indent: -.28in; unicode-bidi: embed; word-break: normal;">
<div class="MsoNormal" style="text-indent: 0px;">
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">A case of a patient initially treated for pneumonia and then presenting with generalised progressive weakness was described.</span><br />
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span lang="EN-GB" style="font-family: "arial"; mso-bidi-font-family: Arial;">Examination<o:p></o:p></span></u></b></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="font-family: "arial"; mso-bidi-font-family: Arial;">On systemic examination she appeared unwell with tachypnoea. There were
bilateral crackles to the midzones, a large reducible hernia and pitting oedema
up to her shins. <o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="font-family: "arial"; mso-bidi-font-family: Arial;">Cranial nerve examination revealed saccadic intrusions of pursuit and
right rapid afferent pupilllary defect. Fundoscopy was not possible. Cranial
nerves were otherwise intact. <o:p></o:p></span></div>
<div class="MsoNormal">
<span lang="EN-GB" style="font-family: "arial"; mso-bidi-font-family: Arial;">Tone was normal on the right but was increased on the left side. There
was reduced power distally in the upper limbs. Lower limb examination revealed
reduced power (most marked proximally in hips and knees with both flexion and extension
scoring 2/5 with distal power more preserved: plantarflexion scored 4/5
bilaterally). Reflexes were brisk throughout except for ankle reflexes which
were 1+ bilaterally), plantar response was extensor.<o:p></o:p></span></div>
<div class="MsoNormal">
<span lang="EN-GB" style="font-family: "arial"; mso-bidi-font-family: Arial;">Sensation to pinprick revealed a sensory level at T8. Vibration sense
was intact in the upper limbs but reduced to the knee bilaterally in lower
limbs. Proprioception was reduced to ankles bilaterally.<o:p></o:p></span></div>
<div class="MsoNormal">
<span lang="EN-GB" style="font-family: "arial"; mso-bidi-font-family: Arial;">Cerebellar examination revealed dysarthria, dysmetria (with left side
being more affected than right) and dysdiadokinesis on the left. <o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span lang="EN-GB" style="font-family: "arial"; mso-bidi-font-family: Arial;">Investigations<o:p></o:p></span></u></b></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">MRI
Head</span></b><span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;"> was compared to the one performed few days later and
showed that previous abnormal T2/flair signal in midbrain, superior cerebellar
peduncles and at the right middle cerebellar peduncles and pons was less
conspicuous in keeping with an evolving inflammatory process.<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;"><span style="mso-spacerun: yes;"> </span></span></div>
<div class="separator" style="clear: both; text-align: center;">
<a href="https://2.bp.blogspot.com/-euf27XWGPwg/V-rdIubO7LI/AAAAAAAAZMM/m7tgB-tD90g0rBmk52xY1Ju53cptdO3GwCLcB/s1600/Untitled.png" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="320" src="https://2.bp.blogspot.com/-euf27XWGPwg/V-rdIubO7LI/AAAAAAAAZMM/m7tgB-tD90g0rBmk52xY1Ju53cptdO3GwCLcB/s320/Untitled.png" width="232" /></a></div>
<br />
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">MRI
Whole Spine</span></b><span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;"> revealed an extensive signal abnormality throughout
the lower cervical and thoracic spinal cord with a confluent segment extending
from T7-T10 and more patchy involvement in the cervicothoracic cord superiorly.<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">Microbiology revealed positive CMV and VZV IgG but
otherwise no abnormalities. Protein electrophoresis showed polyclonal bands.
Autoimmune screen revealed mildly positive pANCA. Haematological investigation showed
neutrophilia (WCC 35) and blood film revealed toxic shift (metamyelocytes).<o:p></o:p></span></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">CSF</span></b><span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;"><span style="mso-spacerun: yes;"> </span>showed 1 WCC, 123 RBC, 0.26 Protein
(normal)<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">Visual
Evoked Potentials</span></b><span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;"> showed a well formed response from the left eye
with no reproducible response from the right eye.<span style="mso-spacerun: yes;"> </span>Somatosensory evoked potentials were normal in the upper
limb. Lower limb abnormalities (showing borderline delayed cortical response-
P40) could be in keeping with central demyelination.<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span lang="EN-GB" style="font-family: "arial"; mso-bidi-font-family: Arial;">Differentials<o:p></o:p></span></u></b></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="font-family: "arial"; mso-bidi-font-family: Arial;">Taking all of the above into consideration a longitudinally extensive
transverse myelitis with midbrain/ superior cerebellar peduncle involvement was
the pathological process. Neuromyelitis optica (NMO) and Acute Disseminated
encephalomyelitis (ADEM) were the top differential diagnoses.<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">Management<o:p></o:p></span></u></b></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">The patient was commenced on 3 day course IVMP
followed by oral Prednisolone and a week later transferred to RLH for further
investigation and management. <o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">At RLH patient was managed with 4 days of PLEX with
weaning of oral steroids. Noticeable neurological improvement was noticed- the
patient, who was bedbound on admission and had slurred speech, eventually
managed to mobilize with a frame and her speech began to normalise. Full blood
count normalized throughout the admission and there were no further temperature
spikes.<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">Diagnosis<o:p></o:p></span></u></b></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">Further results were negative for Aquaporin 4, making
the diagnosis of NMO less likely. Mycoplasma serology was resulted as positive (1/2560)
leading to the diagnosis of ADEM secondary to Mycoplasma Pneumonia. The patient
was treated with a 14-day course of Clarithromycin.<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">Discussion<o:p></o:p></span></u></b></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">Acute Disseminated Encephalomyelitis (ADEM) is a
widespread acute autoimmune demyelinating disease, which affects brain and
spinal cord. Typically, neuroimaging shows multifocal white matter lesions and as
such the clinical presentation includes both motor and sensory impairment as
well as autonomic dysfunction (in line with presentation of this patient who
had reduced power in lower limbs, brisk reflexes and reduced sensation in lower
limbs as well as dysarthria and incoordination).<span style="mso-bidi-font-style: italic;"><o:p></o:p></span></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">Although incompletely understood, ADEM appears to be
triggered by an environmental stimulus in genetically susceptible individuals. Amongst
the causes of ADEM the most common is parainfectious but it may be idiopathic
or rarely, following vaccination. <o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">Parainfectious ADEM is preceded by a viral or
bacterial infectious process. Common bacterial causes include Streptococcus,
Mycoplasma pneumoniae and Haemophilia Influenzae. Other associated pathogens
include rubella, Epstein-barr virus, herpes simplex virus, human-herpes virus
6, influenza and human immunodeficiency virus.<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">M. pneumoniae infections can be complicated by
neurological disorders, resulting in myelitis, cerebrovascular disorders,
servere encephalitis and meningitis. In study conducted by Guleria et al. neurological
symptoms were found in 7% of all patients hospitalized for M. pneumoniae.<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">Antineuronal antibodies have been demonstrated in M.
pneumoniae infections with or without CNS disease<span style="mso-spacerun: yes;"> </span><span style="mso-bidi-font-style: italic;">(Nishimura et al
1996).<o:p></o:p></span></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">M.pneumoniae may lead to ADEM either by neuroinvasion
or immune complex-mediated vasculopathy.<a href="https://www.blogger.com/null" name="_GoBack"></a><o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">ADEM
and the role of neuroinvasion (Stamm et al 2008)<o:p></o:p></span></u></b></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">Stamm et al described a case of 45-year-old previously
healthy man who presented with fever, cough and non-purulent sputum.<span style="mso-spacerun: yes;"> </span>Diagnosis of bilateral basal pneumonia
was made and the patient was treated with Clarithromycin. Within 4 days,
however, rapidly ascending polyradiculoneuropathy<span style="mso-spacerun: yes;"> </span>developed, resulting in facial palsy, opththalmoplegia and
tetraparesis. Viral PCR and bacterial and viral serology were negative.<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">CT head showed brain oedema and inflammatory/
demyelinating lesions in the subcortical white matter, whilst EMG revealed
severe peripheral neuropathy.<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">No antiganglioside (GM) 1 or anti-GM2 antibodies were
discovered. <o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">Differential diagnoses included polyradiculoneuropathy
(atypical Guillain-Barré syndrome) and acute encephalitis as complications of
bilateral pneumonia caused by M. pneumoniae. The patient was commenced on
Clarithromycin with Amoxicillin and Ceftriaxone<span style="mso-spacerun: yes;"> </span>then given IVIG (0.4 g/ kg bodyweight/day for 5 days).<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">He died of intractable cerebral edema 10 days after
the onset of neurologic symptoms. <span style="mso-spacerun: yes;"> </span>At
Autopsy M. pneumoniae RNA detected in brain tissue by nucleic acid
hybridization. <o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">The case suggests a role of invasion of the CNS by the
organism itself. Interestingly, neuroinvasion is more prevalent in patients
with early onset neurologic complications. The effects of the neuroinvasion,
however, remain unclear. The organism may either cause direct damage or trigger
a more violent immunologic reaction.<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">Parainfectious
ADEM (Gupta et al 2007)<o:p></o:p></span></u></b></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">A 41-year-old man presented with a 2-week history of
lethargy, chills, nausea, vomiting and a productive cough. CT Chest showed
right lower lobe pneumonia and the patient was commenced on IV Amoxicillin and
Doxycycline. <o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">One week later he developed lower limb weakness, which
progressed to complete paraplegia with urinary retention. Six days later patchy
visual loss in both eyes follows and fundoscopy revealed swollen optic disc
bilaterally. <o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">Serology suggested recent Mycoplasma pneumoniae
infection with a M. pneumoniae agglutination antibody titre of 1 in 1280. <o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">MRI showed increased T2 signal and swelling of the cord
extending from T3 to T8, as well as several white matter lesions in the
periventricular white matter of the cerebral hemispheres, whereas CSF revealed a
mononuclear pleocytosis of 24 mononuclear cells per microlitre.<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">A diagnosis of acute disseminated encephalomyelitis
(ADEM) secondary to M. pneumoniae was made and patient was commenced on IV
methylprednisolone was commenced at 1 g daily. <span style="mso-spacerun: yes;"> </span>This resulted in no improvement over the course of the next
three days, so treatment was changed to high-dose oral prednisolone and plasma
exchange. <o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">A total of 10 exchanges were carried out over 3 weeks.
This resulted in improved vision and the patient regaining normal lower limb
power and sphincter control over the next two months.<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">This dramatic response to plasma exchange supports a
hypothesis that the ADEM was secondary to an immune complex-mediated
vasculopathy<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span lang="EN-GB" style="font-family: "arial"; mso-bidi-font-family: Arial;">Key points<o:p></o:p></span></u></b></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span style="mso-bidi-font-weight: normal;"><span style="font-family: "arial"; font-weight: bold; text-decoration: underline;">Neurologic manifestations</span></span><span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">
occur approximately ten days after the onset of the initial respiratory tract
infection<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;"><b style="mso-bidi-font-weight: normal;"><u>CSF:</u></b> CSF Gram stain and
bacterial cultures are usually negative. The CSF leukocyte count is elevated
predominantly mononuclear pleocytosis and most cases of M.
pneumoniae-associated ADEM have a normal CSF/serum glucose ratio.<o:p></o:p></span></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">Serology or PCR: </span></u></b><span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">IgM
antibodies can be detected shortly after the acute infection, may persist for
up to 6 months and are followed by IgG titer elevation.</span></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">A
positive cold haemagglutinins titer (non-specific) <o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "arial";"><br /></span><b style="font-family: arial;"><u>MRI Head/ Spinal Cord:</u></b><span style="font-family: "arial";"> </span><span style="font-family: "arial";">characteristic findings: patchy asymmetric
or diffuse signal changes of gray and white matter and multifocal, asymmetric
foci of high signal intensity on flair and T2 weighted images.</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-GB" style="font-family: "arial"; mso-bidi-font-family: Arial;"><b style="mso-bidi-font-weight: normal;"><u>Management:</u></b> </span><span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">Antibiotic therapy has been
temporally associated with clinical improvement in some cases of M.
pneumoniae-associated ADEM/ATM</span><span lang="EN-GB" style="font-family: "arial"; mso-bidi-font-family: Arial;"><o:p></o:p></span></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">Corticosteroids </span></b><span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">are
useful in the initial management of ADEM and transverse myelitis with their
main contribution being the shortening of the duration of neurologic findings
(only if no infective source identified). <o:p></o:p></span></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">Intravenous immune globulin</span></b><span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">
is usually used in case of no response to steroids.<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">Last therapeutic measure: <b style="mso-bidi-font-weight: normal;">Plasma
Exchange</b><o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">In
this case:<o:p></o:p></span></u></b></div>
<div class="MsoNormal">
<span lang="EN-GB" style="font-family: "arial"; mso-bidi-font-family: Arial;">Patient presented with worsened neurology (cerebellar and thoracic spine
involvement) after a severe chest infection. Blood tests were unremarkable
except for Mycoplasma serology. This shows the importance of translating a wide
differential into investigations.<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<b style="mso-bidi-font-weight: normal;"><u><span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">References
and recommended reading<o:p></o:p></span></u></b></div>
<div class="MsoNormal">
<span lang="EN-GB" style="font-family: "arial"; mso-bidi-font-family: Arial;">1.</span><span style="background: white; color: black; font-family: "arial";">Garg
R K. Acute disseminated encephalomyelitis.</span><span style="color: black; font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;"> </span><i style="mso-bidi-font-style: normal;"><span style="background: white; color: black; font-family: "arial";">Postgrad Med J</span></i><i style="mso-bidi-font-style: normal;"><span style="color: black; font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;"> </span></i><span style="background: white; color: black; font-family: "arial";">2013; 79(): 11-17.</span><span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial; mso-bidi-font-size: 10.0pt;"><o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">2. Ning MM, Smirnakis S, Furie KL, Sheen VL. Adult
acute disseminated encephalomyelitis associated with poststreptococcal
infection. J Clin Neurosci. 2005;12:298–300.<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial; mso-bidi-font-size: 10.0pt;">3. Sotgiu S, Pugliatti M,
Rosati G, Deiana A, Sechi P. Neurological disorders associated with Mycoplasma
pneumoniae infection. Eur J Neurol 10: 165-168, 2003.<o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial; mso-bidi-font-size: 10.0pt;">4. Guleria R, Nisar N,
Chawla TC, Biswas NR. Mycoplasma pneumoniae and central nervous system
complications: a review. J Lab Clin Med 146: 55-63, 2005.<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">5. Höllinger P, Sturzenegger M, Mathis J,
Schroth G, Hess CW. Acute disseminated encephalomyelitis in adults: a
reappraisal of clinical, CSF, EEG, and MRI findings. J Neurol. 2002;249:320–9.<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">6. Beleza P, Ribeiro M, Pereira J, Ferreira C, Jordão
MJ, Almeida F. Probable acute disseminated encephalomyelitis due to<i>Haemophilus
influenzae</i> meningitis. Dev Med Child Neurol. 2008;50:388–91<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">7. Stamm B, Moschopulos M, Hungerbuehler
H, Guarner J, Genrich GL, Zaki SR. Neuroinvasion
by Mycoplasma pneumoniae in acute disseminated
encephalomyelitis. Emerg Infect Dis. 2008;14(4):641-3<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">8. Gupta A, Kimber T, Crompton JL, Karagiannis A.
Acute disseminated encephalomyelitis secondary to Mycoplasma pneumoniae. Intern
Med J. 2009 Jan;39(1):68-9<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">9. Tsiodras S, Kelesidis T, Kelesidis I, Voumbourakis
K, Giamarellou H. Mycoplasma pneumoniae-associated myelitis: a comprehensive
review. Eur J Neurol. 2006 Feb;13(2):112-24.<o:p></o:p></span></div>
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<span style="font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: Arial;">10. Nishimura M, Saida T, Kuroki S, Kawabata T,
Obayashi H, Saida K, Uchiyama T. Post-infectious encephalitis with anti-galactocerebroside
antibody subsequent to Mycoplasma pneumoniae infection. J Neurol Sci. 1996
1;140(1-2):91-5.<o:p></o:p></span></div>
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Anonymoushttp://www.blogger.com/profile/08062870670799646040noreply@blogger.com0tag:blogger.com,1999:blog-895935989949155387.post-64350791528139553982016-09-30T17:21:00.004+01:002016-11-29T08:25:50.724+00:00A Sudden,Thunder-like Headache in a Young Patient<div dir="ltr" style="text-align: left;" trbidi="on">
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; font-size: 11pt;">A young female presented with a sudden onset of stabbing, occipital headache, which climaxed </span><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; font-size: 11pt;">within a few seconds. She described it as the worst headache ever experienced. Maximal pain intensity continued for 2 minutes and then subsided but continued for a further few hours. Headache was associated with mild photophobia and neck stiffness but nevertheless she was able to attend and function at work.</span><br />
<br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="font-size: 11pt;">A flu-like prodrome was described over the preceding day, characterized by </span><span lang="EN-GB" style="font-size: 11pt;">non productive cough, myalgia and fatigue.</span></span><br />
<div style="text-align: center;">
<span lang="EN-GB" style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; font-size: 11pt;"><br /></span></div>
<div style="text-align: center;">
<iframe allowfullscreen="" frameborder="0" height="485" marginheight="0" marginwidth="0" scrolling="no" src="//www.slideshare.net/slideshow/embed_code/key/NecRiLJ8iCi0Oc" style="border-width: 1px; border: 1px solid #ccc; margin-bottom: 5px; max-width: 100%;" width="595"> </iframe><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;">
</span></div>
<div style="text-align: center;">
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span></div>
<div style="text-align: left;">
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span lang="EN-GB" style="font-size: 11pt;">The patient </span><span style="font-size: 11pt;">was not on any regular prescribed, over the counter or herbal
medications but did admit to occasional cocaine and MDMA use (last intake 3
months ago). She drank alcohol within safe limits. A cousin had suffered
from an intracerebral haemorrhage but no details were known.<o:p></o:p></span></span></div>
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span>
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; font-size: 11pt;">The patient presented to her GP surgery and was promptly referred
to hospital.<o:p></o:p></span><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span>
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; font-size: 11pt;">On hospital review she was headache free. Examination was normal.
Kernig's sign was negative, although there was some pain on neck flexion.<o:p></o:p></span><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span>
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; font-size: 11pt;">The differential diagnoses for thunderclap headache remain
wide at this stage and include:<o:p></o:p></span><br />
<div style="margin-left: 36.0pt; mso-list: l1 level1 lfo1; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="font-size: 11pt;">1.<span style="font-size: 7pt; font-stretch: normal;"> </span></span><!--[endif]--><span style="font-size: 11pt;">subarachnoid haemorrhage<o:p></o:p></span></span></div>
<div style="margin-left: 36.0pt; mso-list: l1 level1 lfo1; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="font-size: 11pt;">2.<span style="font-size: 7pt; font-stretch: normal;"> </span></span><!--[endif]--><span style="font-size: 11pt;">cerebral venous sinus thrombosis<o:p></o:p></span></span></div>
<div style="margin-left: 36.0pt; mso-list: l1 level1 lfo1; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="font-size: 11pt;">3.<span style="font-size: 7pt; font-stretch: normal;"> </span></span><!--[endif]--><span style="font-size: 11pt;">pituitary apoplexy<o:p></o:p></span></span></div>
<div style="margin-left: 36.0pt; mso-list: l1 level1 lfo1; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="font-size: 11pt;">4.<span style="font-size: 7pt; font-stretch: normal;"> </span></span><!--[endif]--><span style="font-size: 11pt;">cervicocephalic arterial dissection<o:p></o:p></span></span></div>
<div style="margin-left: 36.0pt; mso-list: l1 level1 lfo1; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="font-size: 11pt;">5.<span style="font-size: 7pt; font-stretch: normal;"> </span></span><!--[endif]--><span style="font-size: 11pt;">acute hypertensive crisis<o:p></o:p></span></span></div>
<div style="margin-left: 36.0pt; mso-list: l1 level1 lfo1; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="font-size: 11pt;">6.<span style="font-size: 7pt; font-stretch: normal;"> </span></span><!--[endif]--><span style="font-size: 11pt;">spontaneous intracranial hypertension<o:p></o:p></span></span></div>
<div style="margin-left: 36.0pt; mso-list: l1 level1 lfo1; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="font-size: 11pt;">7.<span style="font-size: 7pt; font-stretch: normal;"> </span></span><!--[endif]--><span style="font-size: 11pt;">idiopathic thunderclap headache.<o:p></o:p></span></span></div>
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span>
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; font-size: 11pt;">Investigation revealed normal inflammatory markers and
unremarkable CT head. <o:p></o:p></span><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; font-size: 11pt;">LP showed WCC <5, RCC < 5, no organisms/growth,
CSF protein 0.28g/L, glucose 3.4mmol/L (no serum) bilirubin +<i> pointing to a diagnosis of SAH</i><o:p></o:p></span><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span>
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; font-size: 11pt;">After emergency Neurosurgical transfer CT Angiogram was performed.
MRI Head and MRA Intracranial did not identify underlying abnormality. Interval
outpatient MRI and MRA were planned as an outpatient but after delay in discharge
were done 5 days later after complete resolution of the symptoms. Interval scan confirmed the presence of
a para-ophthalmic segment aneurysm.<o:p></o:p></span><br />
<br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><u><span style="font-size: 11pt;">Some interesting points were discussed at the Neuroscience
Meeting:</span></u><span style="font-size: 11pt;"><o:p></o:p></span></span><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span>
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><b><span style="font-size: 11pt;">1. Subarachnoid Haemorrhage
and Intracranial Aneurysm</span></b><span style="font-size: 11pt;"><o:p></o:p></span></span><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span>
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; font-size: 11pt;">Spontaneous subarachnoid haemorrhage (SAH) in the case presented
was caused by a rupture of an intracranial aneurysm, which accounts for the majority
(80-90%) of such events.(1) Mortality for untreated aneurysmal subarachnoid haemorrhage
is high (50)% within the first month mainly due to risk of re-rupture(2),
therefore early identification and definitive treatment of the aneurysm is
generally advocated.<o:p></o:p></span><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span>
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; font-size: 11pt;">Cases of non-aneurysmal spontaneous SAH may be due to:<o:p></o:p></span><br />
<div style="margin-left: 36.0pt; mso-list: l0 level1 lfo2; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="font-size: 11pt;">·<span style="font-size: 7pt; font-stretch: normal;"> </span></span><!--[endif]--><span style="font-size: 11pt;">perimesencephalic hemorrhage<o:p></o:p></span></span></div>
<div style="margin-left: 36.0pt; mso-list: l0 level1 lfo2; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="font-size: 11pt;">·<span style="font-size: 7pt; font-stretch: normal;"> </span></span><!--[endif]--><span style="font-size: 11pt;">cerebral artery dissection<o:p></o:p></span></span></div>
<div style="margin-left: 36.0pt; mso-list: l0 level1 lfo2; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="font-size: 11pt;">·<span style="font-size: 7pt; font-stretch: normal;"> </span></span><!--[endif]--><span style="font-size: 11pt;">vascuilitis<o:p></o:p></span></span></div>
<div style="margin-left: 36.0pt; mso-list: l0 level1 lfo2; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="font-size: 11pt;">·<span style="font-size: 7pt; font-stretch: normal;"> </span></span><!--[endif]--><span style="font-size: 11pt;">brain or spinal artery malformations<o:p></o:p></span></span></div>
<div style="margin-left: 36.0pt; mso-list: l0 level1 lfo2; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="font-size: 11pt;">·<span style="font-size: 7pt; font-stretch: normal;"> </span></span><!--[endif]--><span style="font-size: 11pt;">vasculitis<o:p></o:p></span></span></div>
<div style="margin-left: 36.0pt; mso-list: l0 level1 lfo2; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="font-size: 11pt;">·<span style="font-size: 7pt; font-stretch: normal;"> </span></span><!--[endif]--><span style="font-size: 11pt;">stroke<o:p></o:p></span></span></div>
<div style="margin-left: 36.0pt; mso-list: l0 level1 lfo2; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="font-size: 11pt;">·<span style="font-size: 7pt; font-stretch: normal;"> </span></span><!--[endif]--><span style="font-size: 11pt;">venous sinus thrombosis<o:p></o:p></span></span></div>
<div style="margin-left: 36.0pt; mso-list: l0 level1 lfo2; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="font-size: 11pt;">·<span style="font-size: 7pt; font-stretch: normal;"> </span></span><!--[endif]--><span style="font-size: 11pt;">sickle cell disease<o:p></o:p></span></span></div>
<div style="margin-left: 36.0pt; mso-list: l0 level1 lfo2; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="font-size: 11pt;">·<span style="font-size: 7pt; font-stretch: normal;"> </span></span><!--[endif]--><span style="font-size: 11pt;">pituitary apoplexy<o:p></o:p></span></span></div>
<div style="margin-left: 36.0pt; mso-list: l0 level1 lfo2; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="font-size: 11pt;">·<span style="font-size: 7pt; font-stretch: normal;"> </span></span><!--[endif]--><span style="font-size: 11pt;">substance abuse(which could have been perhaps suggested as a
culprit in this case considering patient's history of MDMA and cocaine use)<o:p></o:p></span></span></div>
<div style="margin-left: 36.0pt; mso-list: l0 level1 lfo2; text-indent: -18.0pt;">
<!--[if !supportLists]--><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="font-size: 11pt;">·<span style="font-size: 7pt; font-stretch: normal;"> </span></span><!--[endif]--><span style="font-size: 11pt;">reversible cerebral vasoconstriction syndrome <o:p></o:p></span></span></div>
<br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><b><span style="font-size: 11pt;">2. CT and MR
Angiography as non-invasive technique primary detection tools for detection of
aneurysm in SAH</span></b><span style="font-size: 11pt;"><o:p></o:p></span></span><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span>
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; font-size: 11pt;">Sensitivity of single slice CTA in detection of intracranial
aneurysms has been reported between 77% and 100%, specificity between 79% and
100% (3,4,5) with some (6) claiming that the introduction of spiral CTA achieves
“equivalent diagnostic accuracy to that of conventional DSA in the detection of
(some) aneurysms”. Some studies, however, have found only 90% sensitivity
even with spiral CTA in detection of aneurysms < 3mm.(7) The alternative
non-invasive tool for detection of aneurysm in SAH is Magnetic Resonance Angiography.
MRA is comparable to CTA for medium and large aneurysms, however in aneurysms
smaller than 5 mm, sensitivity decreases to 56% and therefore CTA has been
shown to be superior. In addition, discordance between DSA (digital subtraction
angiography) and MRA was notable in anterior communicating artery (ACOM)
aneurysms.(8)<o:p></o:p></span><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span>
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span>
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><b><span style="font-size: 11pt;">3. How often does
adjunct catheter angiography change management?</span></b><span style="font-size: 11pt;"><o:p></o:p></span></span><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span>
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; font-size: 11pt;">In a large study by Tomycz et al, 361 patients underwent DSA (Jan 08-09).
Three months prior to DSA, 163 of those had undergone CTA or
MRA. These patients had either suffered from acute SAH (65/163) or were
seen electively in clinic with diagnosis of ‘presumed intracranial aneurysm’. Based
on new information from DSA management plan changed in 17/90 (18.9%) of
patients who had undergone CTA and 22/73 (30.1%) who had undergone MRA<span class="apple-converted-space"> </span><b>(which
in combination equates to 39/163 (23.9%)).<span class="apple-converted-space"> </span></b>(9)
<o:p></o:p></span><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span>
<b><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; font-size: 11pt;">The most common
reason for changing the management plan was a discrepancy in aneurysm size
(beyond 5mm) between DSA and non-invasive imaging techniques.<o:p></o:p></span></b><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span>
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; font-size: 11pt;">It is interesting, therefore, to see whether this will change
clinical practice and indeed, whether it should. Does 24% justify subjecting
76% of other patients, in whom management won't be changed after an adjunct
catheter angiography, to such an invasive diagnostic technique, which carries
(albeit a small- 2.63%(11) risk of neurologic complications?</span><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span>
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><b><span style="font-size: 11pt;">4. Yield of LP in CT
negative patients (10)</span></b><span style="font-size: 11pt;"><o:p></o:p></span></span><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span>
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; font-size: 11pt;">A recent study by Harris et al concluded low yield of LP in
patients where CT has failed to diagnose SAH: Out of 2,248 patients
identified in retrospective data collection 1,898 CSF samples were suitable
for analysis. Out of those 1507 (79.4%) were negative, 299 (15.6%)
inconclusive and 92 (4.1%) had CSF positive for bilirubin, of which 8
(0.4%) were diagnosed with aneurysm on further imaging. It's worth noting
that 13.2% of samples were un-interpretable due to the presence of oxyhaemoglobin
which obscures the bilirubin peak on spectrophotometry.<o:p></o:p></span><br />
<br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; font-size: 11pt;">A new clinical decision tool has been proposed (12):<o:p></o:p></span><br />
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<a href="https://3.bp.blogspot.com/-LM7xsxgIvVA/V98HrBdnTdI/AAAAAAAAZDQ/6TM8BGJFcuU-ZPLQGgRA4VMUcZ3VHD3LwCLcB/s1600/Screen%2Bshot%2B2016-09-18%2Bat%2B9.36.41%2BPM.png" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><img border="0" height="345" src="https://3.bp.blogspot.com/-LM7xsxgIvVA/V98HrBdnTdI/AAAAAAAAZDQ/6TM8BGJFcuU-ZPLQGgRA4VMUcZ3VHD3LwCLcB/s640/Screen%2Bshot%2B2016-09-18%2Bat%2B9.36.41%2BPM.png" width="640" /></span></a></div>
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="font-family: "times" , "times new roman" , serif; mso-ansi-language: EN-US;"><br /></span>
<span style="font-family: "times" , "times new roman" , serif; mso-ansi-language: EN-US;"><span style="font-size: 14.6667px;">Of note, age >40 at presentation makes SAH more likely in patients with a typical history.</span></span> </span><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; mso-ansi-language: EN-US;"><br /></span>
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; mso-ansi-language: EN-US;"><b>References</b>: </span><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="font-family: "times" , "times new roman" , serif; mso-ansi-language: EN-US;"><br /></span>
<span style="font-family: "times" , "times new roman" , serif;"><span style="font-family: "times new roman"; mso-ansi-language: EN-US;">1. </span><span style="line-height: 90%; text-indent: 0in;">Kirkpatrick </span><span style="line-height: 90%; text-indent: 0in;">PJ. Subarachnoid </span><span style="line-height: 90%; text-indent: 0in;">haemorrhage</span><span style="line-height: 90%; text-indent: 0in;"> and intracranial aneurysms: what neurologists need to
know. </span><span style="font-style: italic; line-height: 90%; text-indent: 0in;">J </span><span style="font-style: italic; line-height: 90%; text-indent: 0in;">Neurol</span><span style="font-style: italic; line-height: 90%; text-indent: 0in;"> </span><span style="font-style: italic; line-height: 90%; text-indent: 0in;">Neurosurg</span><span style="font-style: italic; line-height: 90%; text-indent: 0in;">
Psychiatry</span><span style="line-height: 90%; text-indent: 0in;"> 2002;73(</span><span style="line-height: 90%; text-indent: 0in;">suppl</span><span style="line-height: 90%; text-indent: 0in;"> 1):</span><span style="line-height: 90%; text-indent: 0in;">i28–i33.</span></span></span><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="line-height: 90%; text-indent: 0in;">2.</span>Locksley
HB. Natural history
of subarachnoid hemorrhage, intracranial aneurysms and arteriovenous
malformations: based on 6368 cases in the cooperative study. <span style="font-style: italic;">J </span><span style="font-style: italic;">Neurosurg</span> 1966;25(2):219–239. </span><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;">3.<span style="line-height: 90%; text-indent: 0in;">Chapell</span><span style="line-height: 90%; text-indent: 0in;"> ET, Moure FC, Good MC. Comparison of computed
tomography angiography with digital subtraction angiography in the diagnosis of
cerebral aneurysms: A meta-analysis. Neurosurgery. </span><span style="line-height: 90%; text-indent: 0in;">2003;52:624–31</span></span><br />
<!--EndFragment--><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;">4.Cloft HJ, Joseph GJ, Dion
DE. Meta-analysis of risks of cerebral angiography in patients with
subarachnoid hemorrhage, intracranial aneurysm and arteriovenous malformation:
A meta-analysis. Stroke. 1999;30:317–20.</span><br />
<!--StartFragment--><!--EndFragment--><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;">5.Henriëtte E. Westerlaan, J.M.C. van Dijk, Marijke C.
Jansen-van der Weide, Jan Cees de Groot,
Rob J. M. Groen, Jan Jakob A. Mooij, and Matthijs Oudkerk. Intracranial
Aneurysms in Patients with Subarachnoid Hemorrhage: CT Angiography as a Primary
Examination Tool for Diagnosis—Systematic Review and Meta-Analysis. Radiology
2011 258:1, 134-145.</span><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;">6.Hope
JK, Wilson JL,
Thomson FJ. Three-dimensional CT angiography in the detection and
characterization of intracranial berry aneurysms. AJNR Am J Neuroradiol. 1996;17:439–45. </span><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;">7.<span style="line-height: 90%; text-indent: 0in;">Jacobson DM, Trobe JD. The emerging role of MRA in the
management of patients with third cranial nerve palsy. Am J </span><span style="line-height: 90%; text-indent: 0in;">Ophthalmol</span><span style="line-height: 90%; text-indent: 0in;">. 1999;128:94–6.</span><span style="line-height: 90%; text-indent: 0in;"></span></span><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="line-height: 90%; text-indent: 0in;">8.</span>Schwab
K, Gailloud P, Wyse G, Tamargo R. Limitations of
magnetic resonance imaging and magnetic resonance angiography in the diagnosis
of intracranial aneurysms. Neurosurgery. 2008;63:29–35.</span><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;">9.<span style="line-height: 90%; text-indent: 0in;">Tomycz</span><span style="line-height: 90%; text-indent: 0in;"> L, Bansal N, Hawley C, Goddard T, </span><span style="line-height: 90%; text-indent: 0in;">Ayad</span><span style="line-height: 90%; text-indent: 0in;"> M, </span><span style="line-height: 90%; text-indent: 0in;">Mericle</span><span style="line-height: 90%; text-indent: 0in;"> R. “Real world”
analysis comparison of non-invasive imaging to conventional catheter
angiography in the diagnosis of cerebral aneurysms. </span><span style="line-height: 90%; text-indent: 0in;">Surg</span><span style="line-height: 90%; text-indent: 0in;"> </span><span style="line-height: 90%; text-indent: 0in;">Neurol</span><span style="line-height: 90%; text-indent: 0in;"> </span><span style="line-height: 90%; text-indent: 0in;">Int</span><span style="line-height: 90%; text-indent: 0in;"> 2011;2:134.</span></span><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="line-height: 90%; text-indent: 0in;">10.</span><span style="background-color: white; line-height: 17.92px;">Timothy J. Kaufmann, MD, John Huston, III, MD, Jay N. Mandrekar, PhD, Cathy D. Schleck, BS, Kent R. Thielen, MD, and David F. Kallmes, MD . Complications of Diagnostic Cerebral Angiography: Evaluation of 19 826 Consecutive Patients. </span><i style="background-color: white; line-height: 17.92px;">RSNA Radiology </i><span style="background-color: white; line-height: 17.92px;">2006; 243(3)</span></span><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="line-height: 90%; text-indent: 0in;"></span><span style="line-height: 90%; text-indent: 0in;">11.Sayer D, Bloom B, Fernando K, Jones S, Benton S, Dev S,
Harris T. An observational study of 2,248 patients presenting with headache, </span><span style="line-height: 90%; text-indent: 0in;">suggestice</span><span style="line-height: 90%; text-indent: 0in;"> of SAH, who </span><span style="line-height: 90%; text-indent: 0in;">receieved</span><span style="line-height: 90%; text-indent: 0in;"> lumbar punctures.</span><span style="line-height: 90%; text-indent: 0in;"></span></span><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="line-height: 90%; text-indent: 0in;">12. </span>Kimura,
A, Kobayashi K, Yamaguchi H, Takahashi T, Harada M, Honda H, Mori Y, Tanaka A.</span><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;">Nw clinical decision
rule to exclude subarachnoid haemorhage for acute headache; a multicentre observational study.
BMJ 2016;6: </span><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; font-style: italic;">epud</span><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; font-style: italic;"> ahead of print</span><br />
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Anonymoushttp://www.blogger.com/profile/08062870670799646040noreply@blogger.com0tag:blogger.com,1999:blog-895935989949155387.post-76206395437281469362016-09-21T23:00:00.000+01:002016-11-29T08:26:38.871+00:00'When the Penny Drops': A Curious Case<div dir="ltr" style="text-align: left;" trbidi="on">
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<span style="color: black; font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; mso-ansi-language: EN-US; mso-bidi-font-family: "Times New Roman"; mso-bidi-font-size: 13.5pt;">A
young man in his twenties presented with
a six-year history of progressive lower limb symptoms including positive
sensory change, poor balance and deteriorating lower limb weakness. </span></div>
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<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span></div>
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<b style="mso-bidi-font-weight: normal;"><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; mso-ansi-language: EN-US; mso-bidi-font-size: 10.0pt;"><u>Examination</u><o:p></o:p></span></b></div>
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<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span></div>
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<span style="color: black; font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; mso-ansi-language: EN-US; mso-bidi-font-family: "Times New Roman"; mso-bidi-font-size: 13.5pt;">Examination
revealed cachexia and wasting: there was bilateral facial wasting particularly
of temporalis as well as wasting of lower limbs proximally and distally. There
was bilateral foot drop, flat feet and Romberg’s was positive.<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span></div>
<div class="MsoNormal">
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; mso-ansi-language: EN-US; mso-bidi-font-family: "Times New Roman"; mso-bidi-font-size: 10.0pt;">Cranial nerve
exam revealed visual acuity of 6/9 on the right with perception to light
on left. Corneal scarring was noted with normal retinas. Pupillary response was
normal on the right and sluggish on the left. There was also a bilateral 50%
ptosis and tarsoraphy.<o:p></o:p></span></div>
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<span style="font-family: "times" , "times new roman" , serif;">Cranial nerves exam revealed visual acuity of 6/9 on the right with perception to light on left. Corneal scarring was noted with normal retinas. Pupillary response was normal on the right and sluggish on the left. There was also a bilateral 50% ptosis and taroraphy.</span></span><br />
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<a href="https://1.bp.blogspot.com/-AkUvDWQ1auY/V9cqkgqbCCI/AAAAAAAAY7s/Ev9G2BZIJpo0a-qB-lT0vNkD0enqC5xQACLcB/s1600/Untitled.png" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><img border="0" height="123" src="https://1.bp.blogspot.com/-AkUvDWQ1auY/V9cqkgqbCCI/AAAAAAAAY7s/Ev9G2BZIJpo0a-qB-lT0vNkD0enqC5xQACLcB/s320/Untitled.png" width="320" /></span></a></div>
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bilateral decrease in facial sensation and severe bilateral facial weakness.
Abnormal audiogram and reduced gag reflex were found. Uvula was, however, central
and palate/ tongue normal.<o:p></o:p></span></div>
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<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; mso-ansi-language: EN-US; mso-bidi-font-family: "Times New Roman"; mso-bidi-font-size: 10.0pt;">Upper limb
examination was normal. Lower limb examination revealed global wasting and
areflexia.<o:p></o:p></span></div>
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<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span></div>
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<a href="https://4.bp.blogspot.com/-kXPFN4FtdS4/V9nYy-D9_JI/AAAAAAAAY-I/HXEh4WI4HUkNb71kdLAGvsPI1zQ7DFDfACLcB/s1600/Untitled.png" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><img border="0" height="96" src="https://4.bp.blogspot.com/-kXPFN4FtdS4/V9nYy-D9_JI/AAAAAAAAY-I/HXEh4WI4HUkNb71kdLAGvsPI1zQ7DFDfACLcB/s320/Untitled.png" width="320" /></span></a></div>
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<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;">Sensory examination revealed absent joint position sense to hips and pin prick exam revealed patchy deficits up to the buttocks.</span><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span>
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<b><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><u>Investigations</u><o:p></o:p></span></b></div>
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<div class="MsoNormal">
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;">Routine
investigations as well as vasculitis and infection screen were normal
with CK of 300. Gangliosides and anti-neuronals as well as genetics
for HMN, ALS, FAP, FSHD, MD and SMA were negative.<o:p></o:p></span></div>
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<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;">Muscle
biopsy was performed and it showed non-specific fibre atrophy.<o:p></o:p></span></div>
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<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="font-family: "arial";">Examination
of ventricular CSF showed </span><span style="font-family: "arial";"> </span><span style="font-family: "arial";">protein of 4.15 g/L RCC 10,000, WCC 12 (lymphocytes)
and normal glucose. Cytology showed RBCs++ and OCB-ve.<o:p></o:p></span></span></div>
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<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span></div>
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<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="font-family: "arial";">NCS
and EMG showed no impairment in sensory nerves. There was, however, severe
axonal damage noted in motor studies in lower limbs. Facial nerve conduction
studies revealed axonal damage, whereas EMG showed neuropathic process with
large MUP recruitment (proximal and distal). As on clinical examination, upper
limbs were normal.</span><span style="font-family: "times new roman";"><o:p></o:p></span></span><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span></div>
<div class="separator" style="clear: both; text-align: center;">
<br /></div>
<div class="MsoNormal">
<span style="color: black; font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; mso-ansi-language: EN-US; mso-bidi-font-family: "Times New Roman"; mso-bidi-font-size: 13.5pt;">Imaging
revealed infiltrative process with leptomeninges and lumbosacral radiculopathy.
It was inconsistent with differential diagnosis of neurosarcoid.<o:p></o:p></span></div>
<div class="MsoNormal">
<span style="color: black; font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; mso-ansi-language: EN-US; mso-bidi-font-family: "Times New Roman"; mso-bidi-font-size: 13.5pt;">Infection
origin was also excluded due to inconsistent timeline of the disease as was
amyloid on the basis of negative genetic testing and blood screen.
Taking this into consideration, the diagnoses of choroid plexus
papilloma and lymphoma were suggested.<o:p></o:p></span></div>
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<a href="https://1.bp.blogspot.com/-toK-S1PkM0k/V9cxNIZNyBI/AAAAAAAAY8Q/Gcp-nhDgtPAYRX227ODj4-cacYq2sJP_wCLcB/s1600/Screen%2Bshot%2B2016-09-12%2Bat%2B11.47.49%2BPM.png" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><img border="0" height="406" src="https://1.bp.blogspot.com/-toK-S1PkM0k/V9cxNIZNyBI/AAAAAAAAY8Q/Gcp-nhDgtPAYRX227ODj4-cacYq2sJP_wCLcB/s320/Screen%2Bshot%2B2016-09-12%2Bat%2B11.47.49%2BPM.png" width="640" /></span></a></div>
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<b><u><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;">Choroid Plexus Papillomas and discussion of the case</span></u></b></div>
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<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span></div>
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<span style="color: black; font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; mso-ansi-language: EN-US; mso-bidi-font-family: "Times New Roman"; mso-bidi-font-size: 13.5pt;">Choroid
plexus neoplasms are rare in all age groups, representing 5 % of all paediatric
brain tumours and less than 1 % of adult intracranial tumours.<o:p></o:p></span></div>
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<span style="color: black; font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; mso-ansi-language: EN-US; mso-bidi-font-family: "Times New Roman"; mso-bidi-font-size: 13.5pt;">Most
of them are sporadic, yet they constitute component of some syndromes such as
Aicardi syndrome, Down syndrome (1)or von Hippel- Lindau disease. Germline
mutations of TP53 also seem to predispose to them.(2) As in this case, most of
choroid plexus neoplasms arise within the ventricles. (most commonly within the
fourth with the lateral ventricles being second in frequency).<o:p></o:p></span></div>
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<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span></div>
<div class="MsoNormal">
<span style="color: black; font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; mso-ansi-language: EN-US; mso-bidi-font-family: "Times New Roman"; mso-bidi-font-size: 13.5pt;">Hydrocephalus,
the commonest presentation of choroid plexus papillomas, can be produced by a
number of different mechanisms: local expansion of the ventricles, spontaneous
haemorrhage or by the overproduction of CSF by the tumour itself. Patients
present with headache, vomiting and altered mental status or ataxia, as in this
case.<o:p></o:p></span></div>
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<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span></div>
<div class="MsoNormal">
<span style="color: black; font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; mso-ansi-language: EN-US; mso-bidi-font-family: "Times New Roman"; mso-bidi-font-size: 13.5pt;">Lower
limb symptoms are explained by the seeding of CSF and drop metastases
which can occur throughout the neuoraxis with particular predominance in
cauda equina region. Interestingly, unlike in other neoplasms, seeding also
occurs in benign choroid plexus papillomas (as in this patient), however more
commonly the spread is achieved by leptomeningeal dissemination. (3)<o:p></o:p></span></div>
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<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span></div>
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<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="color: black; font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: "Times New Roman"; mso-bidi-font-size: 13.5pt;">Choroid
plexus papillomas generally have a better prognosis than choroid plexus
carcinomas. The latter, more aggresive, have a greater tendency for
leptomeningeal</span><span style="color: black; font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: "Times New Roman";"> </span><span style="color: black; font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: "Times New Roman"; mso-bidi-font-size: 13.5pt;">dissemination. Favourable outcome can be achieved when
gross total resection is combined with adjuvant chemo and radiotherapy.(4) In
this case suggested treatment options included removing the primary, resecting
metastatic lesion (surgically or stereotactically) as well as adjuvant
chemotherapy (Lomustine) and radiotherapy (50 Gy). Limited evidence does show
success of chemotherapy with alkylating agents in extensive spinal seeding.<o:p></o:p></span></span></div>
<div class="MsoNormal" style="background: white;">
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span></div>
<div class="MsoNormal" style="background: white;">
<span style="color: black; font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif; mso-ansi-language: EN-US; mso-bidi-font-family: "Times New Roman"; mso-bidi-font-size: 13.5pt;">The case emphasized the importance of nerve conduction
studies in differentiating between postganglionic (plexopathies, neuropathies)
and preganglionic disorders (cauda equina lesions, radiculopathies, posterior
column diseases). In case of preganglionic lesion, as in this case, the sensory
nerve action potential is normal due to the axonal transport form the cell body
to the peripheral axon remaining intact. Furthermore, due to the fact that the
cell body of motor nerves is located in the anterior horn of the spinal cord
the motor nerve conduction will show abnormality in both types of lesions: preganglionic
and postganglionic. <o:p></o:p></span></div>
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<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span></div>
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<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="color: black; font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: "Times New Roman"; mso-bidi-font-size: 13.5pt;">Lastly, the case could be used to support Hickam's
dictum, which is in stark contrast to Occram's razor advocating the restriction
of differential diagnoses to minimum.</span><span style="color: black; font-family: "times new roman"; mso-ansi-language: EN-US; mso-bidi-font-family: "Times New Roman"; mso-bidi-font-size: 13.5pt;"> </span><span style="color: black; font-family: "arial"; mso-ansi-language: EN-US; mso-bidi-font-family: "Times New Roman"; mso-bidi-font-size: 13.5pt;">Hickman's dictum, on the other hand, encompasses a
wider view and allows for the fact that patients can have multiple diseases at the
same time. Moreover, it takes into account the fact that they might present
with a very rare disease, which would be excluded by firm believers in Occram's
razor due to it being statistically insignificant. Which one should we adopt?
Is it possible to find a comfortable middle ground?</span><span style="color: black; font-family: "times new roman"; mso-ansi-language: EN-US; mso-bidi-font-family: "Times New Roman"; mso-bidi-font-size: 13.5pt;"><o:p></o:p></span></span></div>
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<u><b><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;">Learning points:</span></b></u></div>
<div class="separator" style="clear: both; text-align: left;">
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;">1) Occam's razor vs Hickam's dictum</span></div>
<div class="separator" style="clear: both; text-align: left;">
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;">2) History key: acquired vs inherited</span></div>
<div class="separator" style="clear: both; text-align: left;">
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;">3) Neurogenic, preganglionic lesions in NCS/ EMG-</span></div>
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<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span></div>
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<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><b><u>Further reading:</u></b></span></div>
<div class="separator" style="clear: both; text-align: left;">
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;">1) <span style="background-color: white;">Hori A, Walter GF, Haas J, Becker H. Down syndrome complicated by brain tumors: case report and review of the literature. </span><em style="background-color: white;">Brain Dev</em><span style="background-color: white;">. 1992 Nov. 14(6):396-400.</span></span></div>
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<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="background-color: white;">2)</span><span style="background-color: white;">Pianetti Filho G, Fonseca LF, da Silva MC. Choroid plexus papilloma and Aicardi syndrome: case report. </span><em style="background-color: white;">Arq Neuropsiquiatr</em><span style="background-color: white;">. 2002 Dec. 60(4):1008-10. </span></span></div>
<div class="separator" style="clear: both; text-align: left;">
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="background-color: white;">3) </span><span style="background-color: white;">Menon G, Nair SN, Baldawa SS, Rao RB, Krishnakumar KP, Gopalakrishnan CV. Choroid plexus tumors: an institutional series of 25 patients. </span><em style="background-color: white;">Neurol India</em><span style="background-color: white;">. 2010 May-Jun. 58(3):429-35. </span></span></div>
<div class="separator" style="clear: both; text-align: left;">
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><span style="background-color: white;">4)</span><span style="background-color: white;">Wrede B, Hasselblatt M, Peters O, Thall PF, Kutluk T, Moghrabi A, et al. Atypical choroid plexus papilloma: clinical experience in the CPT-SIOP-2000 study. </span><em style="background-color: white;">J Neurooncol</em><span style="background-color: white;">. 2009 Dec. 95(3):383-92. </span></span></div>
<div class="separator" style="clear: both; text-align: left;">
<span style="background-color: white;"><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span></span></div>
<div class="separator" style="clear: both; text-align: left;">
<span style="background-color: white;"><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;">Enomoto H, Mizuno M, Katsumata T, Doi T : Intracranial metastasis of a choroid plexus papilloma originating in the cerebellopontine angle region : a case report. Surg Neurol 36 : 54-58, 1991.</span></span></div>
<div class="separator" style="clear: both; text-align: left;">
<span style="background-color: white;"><span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;">Kaptanoglu E, Tun K, Celikmez RC, Ozen O, Taskin Y : Spinal drop metastasis of choroid plexus papilloma. J Clin Neurosci 14 : 381-383, 2007.</span></span></div>
</div>
Anonymoushttp://www.blogger.com/profile/08062870670799646040noreply@blogger.com0tag:blogger.com,1999:blog-895935989949155387.post-72864265411091937562016-08-31T08:52:00.000+01:002016-08-31T17:40:05.475+01:00TB & parainfectious myelitis<div dir="ltr" style="text-align: left;" trbidi="on">
<span style="color: #073763; font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;">Why are absent sensory nerve action potentials such an important finding in cases of myelitis? <b>#TeachNeuro</b></span><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span>
<span style="color: #073763; font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;">I saw a patient last week on the ward with necrotic myelitis with lower motor neurone signs in the lower limbs and mixed upper and lower motor neurone signs in the upper limb. He had a sensory level at C8/T1 and a motor level at C7/C8. The presumptive diagnosis has been TB myelitis based on low CSF glucose level compared to the peripheral blood and raised total protein. CSF cultures have been negative and a peripheral screen has not shown systemic TB, including a whole body FDG-PET study. When I trained in South Africa I saw several cases of so called TB-related parainfectious myelitis. These patients all had active TB and developed their myelitis shortly before, or after, starting their anti-tuberculous drugs (see article by <a href="http://www.ncbi.nlm.nih.gov/pubmed/2345617">Silber et al. 1990</a>). The hypothesis underlying these cases is that the myelitis is a monophasic autoimmune disease. Mycobacteria are potent adjuvants and are well known to trigger, or boost, autoimmune responses in animals. In fact, complete Freund's adjuvant (CFA or FCA) is composed of inactivated and dried mycobacteria (usually M. tuberculosis) in an oil emulsion. CFA is often referred to as the immunologist's dirty little secret and is what is used to trigger EAE (experimental allergic encephalomyelitis).</span><br />
<span style="color: #073763; font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span>
<span style="color: #073763; font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;">The one red flag in relation to this patient is that his nerve conduction studies show absent SNAPs (sensory nerve action potentials) in the lower limbs and a whole body work-up has not shown peripheral TB. Why is finding absent SNAPs such an important clue in this case?</span><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span>
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><b>Essential Reads</b>: </span><br />
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span>
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;">Silber et al. <a href="http://www.ncbi.nlm.nih.gov/pubmed/2345617">Neuromyelitis optica (Devic's syndrome) and pulmonary tuberculosis</a>. Neurology. 1990 Jun;40(6):934-8.<br /><br />Neuromyelitis optica and acute necrotic myelopathy occur in association with pulmonary tuberculosis. We studied 8 patients with either neuromyelitis optica (6), acute myelopathy (1), or acute optic neuropathy (1) in close temporal association with pulmonary tuberculosis, but with no evidence for CNS tuberculosis. Neurologic symptoms preceded the use of antituberculosis medication in 5 patients. Different patients showed similar clinical features, suggesting a consistent disease pattern. Autopsy examination (1 patient) revealed extensive spinal cord and optic nerve demyelination. We identified only 5 additional patients seen over the same period with idiopathic neuromyelitis optica, thus suggesting that the close temporal relationship to pulmonary tuberculosis is not coincidental. The syndrome is most likely due to an immune reaction to tuberculosisrather than the use of antituberculosis medication.</span><br />
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<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;"><br /></span></div>
<span style="font-family: "helvetica neue" , "arial" , "helvetica" , sans-serif;">Hughes & Mair. <a href="http://brain.oxfordjournals.org/content/brain/100/2/223.full.pdf">Acute necrotic myelopathy with pulmonary tuberculosis</a>. Brain. 1977 Jun;100(2):223-38.</span></div>
Gavin Giovannonihttp://www.blogger.com/profile/03634514099871112077noreply@blogger.com0tag:blogger.com,1999:blog-895935989949155387.post-70883985417556748852016-08-30T06:15:00.004+01:002016-08-30T06:15:56.405+01:00Clinical Skills: auscultation of the heart<div dir="ltr" style="text-align: left;" trbidi="on">
<div dir="ltr" style="text-align: left;" trbidi="on">
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">Do you know how to interpret auscultation of the heart? <b>#TeachNeuro</b></span><br />
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"><br /></span>
<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">I have very fond memories of learning auscultation of the heart from one of the most astute cardiologists of his generation, Professor John Barlow, at the University of the Witwatersrand. Professor Barlow's clinical skills were legendary and there was nothing more entertaining than attending his cardiology rounds at Baragwanath hospital. I recall him on more than one occasion accurately predicting the pressure gradient across a tight aortic stenosis and on several occasions he even outperformed the earlier generation echo machines. I recently repurchased Professor Barlow's album on the auscultation of the heart. I sincerely hope you enjoy listening to it as much as I did as a medical student. The aim of this post is not hark back to a previous era of medicine, but to learn from the giants of our field. Although cardiac auscultation is a dying skill knowing how to auscultate and interpret the findings reinforces important aspects in relation to the neurophysiology and physiology of the heart. Enjoy.</span><br />
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<iframe allowfullscreen="" frameborder="0" height="800" marginheight="0" marginwidth="0" scrolling="no" src="//www.slideshare.net/slideshow/embed_code/key/q0MwNJ5Xnc2vyW" style="border-width: 1px; border: 1px solid #ccc; margin-bottom: 5px; max-width: 100%;" width="680"> </iframe><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;"> </span></div>
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<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif; font-size: x-small;"><strong> <a href="https://www.slideshare.net/gavingiovannoni/auscultation-of-the-heart-by-barlow-and-pocock" target="_blank" title="Auscultation of the heart by Barlow and Pocock">Auscultation of the heart by Barlow and Pocock</a> </strong> from <strong><a href="https://www.slideshare.net/gavingiovannoni" target="_blank">Gavin Giovannoni</a></strong> </span></div>
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Gavin Giovannonihttp://www.blogger.com/profile/03634514099871112077noreply@blogger.com1tag:blogger.com,1999:blog-895935989949155387.post-66125382317625858962016-08-30T04:24:00.002+01:002016-08-30T04:24:29.452+01:00Functional Neuroanatomy <div dir="ltr" style="text-align: left;" trbidi="on">
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<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">Functional <b>#neuroanatom</b>y is a core skill for all neurology trainees. <b>#TeachNeuro</b></span></div>
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<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">I remain flabbergasted by the response I recently received from a trainee neurologist when I asked him how he would localise the anatomical site of a IIIrd nerve palsy. His response:<b> <i>“I would order an MRI scan to localise the lesion”</i></b>. What is happening to our basic neurological skills? It has become clear to me that basic neuroanatomy knowledge amongst most neurology trainees is rudimentary, and is simply not detailed enough, to assist in the anatomical localisation of lesions. I am therefore proposing that all trainees relearn <b>functional neuroanatomy</b> in a way that is designed to augment their skills in undertaking and interpreting the neurological examination. The following is a list of questions all neurology trainees should be able to answer: </span><div>
<ol style="text-align: left;">
<li><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">What does a IIIrd palsy look like?</span></li>
<li><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">What are the clinical features of a lesion involving the nucleus of the IIIrd nerve?</span></li>
<li><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">How do you localise a brainstem, or fasicular, IIIrd nerve palsy?</span></li>
<li><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">How do you localise a third palsy to the subarachnoid space?</span></li>
<li><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">How do you assess a the function of the trochlear nerve (IVth) in the presence of a IIIrd nerve palsy?</span></li>
<li><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">What features helps localise the IIIrd palsy to the cavernous sinus?</span></li>
<li><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">What is the significance of a pupil sparing IIIrd palsy? </span></li>
<li><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">What is the significance of fixed mid-sized pupil in association with a IIIrd palsy?</span></li>
<li><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">What are the features of a palsy of the superior division of the IIIrd nerve?</span></li>
<li><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">What are the features of a palsy of the inferior division of the IIIrd nerve?</span></li>
<li><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">How do you localise a IIIrd nerve palsy to the superior orbital fissure? </span></li>
<li><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">Is diplopia and invariable clinical feature of a IIIrd nerve palsy?</span></li>
<li><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">Can you describe the anatomy of the the IIIrd nerve?</span></li>
<li><span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">Can you describe the paths of the parasympathetic and sympathetic innervation of the eye?</span></li>
</ol>
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<span style="font-family: Helvetica Neue, Arial, Helvetica, sans-serif;">When I get the time I will produce a simple one-page infographic to answer all these questions. Does anyone want to help? I feel so strongly about this that I will be writing a commentary for Practical Neurology on this issue. </span></div>
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Gavin Giovannonihttp://www.blogger.com/profile/03634514099871112077noreply@blogger.com1tag:blogger.com,1999:blog-895935989949155387.post-11996346921502945232014-01-16T17:52:00.000+00:002014-01-16T17:52:08.535+00:00BB2: Vestibular System<div dir="ltr" style="text-align: left;" trbidi="on">
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Level 1<br />
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Gavin Giovannonihttp://www.blogger.com/profile/03634514099871112077noreply@blogger.com0tag:blogger.com,1999:blog-895935989949155387.post-9336097980964784312014-01-13T08:39:00.002+00:002014-01-13T08:39:31.651+00:00Brain & Behavior 2: Lecture and lecture notes on MS<div dir="ltr" style="text-align: left;" trbidi="on">
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<span style="font-size: large;"><b>Level 1</b>: The following is my lecture and lecture notes from the lecture I gave on 10 January 2014 to the 2nd year medical students. You can download these from SlideShare by clicking on the slide share icon.</span><br />
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Gavin Giovannonihttp://www.blogger.com/profile/03634514099871112077noreply@blogger.com0tag:blogger.com,1999:blog-895935989949155387.post-46756677626547580092013-08-08T20:48:00.003+01:002013-08-08T20:48:55.744+01:00How does the brain see?<div dir="ltr" style="text-align: left;" trbidi="on">
<b>Level 1</b><br />
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<i>"You may find this animation of interest."</i><br />
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Gavin Giovannonihttp://www.blogger.com/profile/03634514099871112077noreply@blogger.com1tag:blogger.com,1999:blog-895935989949155387.post-27428928937323301152013-06-09T10:49:00.000+01:002013-06-09T10:49:30.249+01:00Neuroprotection in stroke; a slow burner<!--[if gte mso 9]><xml>
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<u><span lang="EN-US" style="color: #343434; font-family: Calibri; font-size: 15.0pt; mso-bidi-font-family: Calibri;">Neuroprotection in stroke;
a slow burner.<o:p></o:p></span></u></div>
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<br /></div>
<div class="MsoNormal">
<span lang="EN-US" style="color: #343434; font-family: Calibri; font-size: 15.0pt; mso-bidi-font-family: Calibri;">One of the real surprises of
medical school is coming to terms with the protracted nature of medical
research. Developing a robust drug which
passes appropriate quality and safety standards is increasingly likely to tip-toe over the decade mark onwards. <o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-US" style="color: #343434; font-family: Calibri; font-size: 15.0pt; mso-bidi-font-family: Calibri;">Within Neurology, arguably the most
troublesome of clinic trials has revolved around finding a group of
neuroprotective agents which tick all the boxes. Neuroprotection works on the
principal that neuronal tissue may be salvaged from an ischaemic event by
endowing it with a greater resilience to the effects of hypoxia. <o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-US" style="color: #343434; font-family: Calibri; font-size: 15.0pt; mso-bidi-font-family: Calibri;">While neuroprotective agents offer
a tantalizing holy grail of a lower risk, lower resource alternative to
thrombolytics, the development process has been dogged by setbacks..... To the tune
of over 1000 unsuccessful potential treatments.
Most of which have been nipped in the bud by the jump from
cellular and rodent models to human trials by an unwanted side
effects profile or ineffectiveness. <o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-US" style="color: #343434; font-family: Calibri; font-size: 15.0pt; mso-bidi-font-family: Calibri;">NA-1, is a neuroprotective agent that
has previously been shown to be efficacious in a primate model of stroke. It works by disrupting the neurotoxic
pathways of NMDA glutamate receptors and imbuing cells with a greater tolerance
to hypoxia. A recent Lancet Neurology paper followed a phase II trial of
individuals undergoing endovascular intracranial aneurysm repair. Participants were randomized to receive
either intraoperative NA-1 or a saline control. Up to 90% of individuals
undergoing this procedure show evidence of small embolic iatrogenic stroke on
diffusion weighted MRI. So the study
group was a very appropriate target to put it mildly. <o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-US" style="color: #343434; font-family: Calibri; font-size: 15.0pt; mso-bidi-font-family: Calibri;">Individuals receiving NA-1 were
significantly less likely to have experienced new brain infarctions (OR 0.53 CL
0.38-74) on DW MRI then controls. More
so, rather conspicuously, the authors reported no serious adverse effects of
the study drug, a promising finding that if genuine warrants a larger trial.
However the drug's administration soley to individuals who were anaesthetised may
limit its study in the near future to invasive treatments carrying a risk of
brain injury rather than community admitted patients with ischaemic
stroke. <o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-US" style="color: #343434; font-family: Calibri; font-size: 15.0pt; mso-bidi-font-family: Calibri;">For the moment the clock keeps
ticking…</span><span lang="EN-US"><o:p></o:p></span></div>
<!--EndFragment-->Unknownnoreply@blogger.com0tag:blogger.com,1999:blog-895935989949155387.post-37656048771755932162013-05-24T13:58:00.002+01:002013-06-05T11:49:50.163+01:00Hitting the sweet spot<div class="ecxMsoNormal">
<br /></div>
<div class="ecxMsoNormal">
<span lang="EN-US">In his landmark work <i>The Structure of Scientific Revolutions</i> Thomas Kuhn argued that far from being a well ordered and timely process, scientific advancement occurs in an erratic, opportunistic and even irrational manner often with no overt overarching dialogue. The scientific community is just that, a community. Ideas continuously flourish, grow and are put to the mill to make room for better fitting (or perhaps just better sounding) paradigms. So it may be worth asking, in the age of the meme driven global village, what hope is there for mere individuals to make ripples in a sea of convolution and hyperspecialisation?</span></div>
<div>
</div>
<div class="ecxMsoNormal">
<span lang="EN-US">Well, perhaps more than we think. Contributing to scientific progress as a whole, far from becoming more unreachable for individuals or indeed small groups of interested individuals, may actually be becoming more attainable. This is thanks to the widening array of easily accessible and evolving technologies which have, at present, outstripped our capacity to come up with innovative uses for them.</span></div>
<div>
</div>
<div class="ecxMsoNormal">
<span lang="EN-US">Echoing this idea, the BMJ, recently published a meta-analysis of several trials looking at the use of ultrasound as a guidance technique for lumbar puncture. With the advent of portable ultrasound probes, novel uses are in abundance. Screening for parkinsons disease, the management of shoulder dislocation and joint reduction to name a few. One of the advantages of metaanalyses is that they clump together groups of similar studies to look at outcomes which may not be obvious in small studies or have the statistical power to be significant. Through grouping the studies the authors were able to show that ultrasound guided lumbar puncture has a significantly lower failure rate and risk of trauma than unguided procedures. A relatively simple finding which may forever change the way we perform this essential diagnostic investigation.</span></div>
<div>
</div>
<div class="ecxMsoNormal">
<span lang="EN-US">It seems that the chance of having a idea that is ripe for the times has never been better...</span></div>
Unknownnoreply@blogger.com1tag:blogger.com,1999:blog-895935989949155387.post-90989583547807106752013-05-19T21:34:00.000+01:002013-05-19T21:34:00.268+01:00Niblet by Andrew Cummings<div dir="ltr" style="text-align: left;" trbidi="on">
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<b><span lang="EN-US" style="font-family: "Calibri","sans-serif"; mso-ansi-language: EN-US;">Getting into minutiae in a big way….</span></b><span style="font-family: "Calibri","sans-serif";"><o:p></o:p></span></div>
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<span lang="EN-US" style="font-family: "Calibri","sans-serif"; mso-ansi-language: EN-US;">“<i>You can know the name of a bird in all the
languages of the world, but when you're finished, you'll know absolutely <a href="http://en.wikiquote.org/wiki/Nothing" target="_blank"><span style="color: windowtext; text-decoration: none; text-underline: none;">nothing</span></a>
whatever about the bird...”</i></span><span style="font-family: "Calibri","sans-serif";"><o:p></o:p></span></div>
<div class="MsoNormal">
<i><span lang="EN-US" style="font-family: "Calibri","sans-serif"; mso-ansi-language: EN-US;"> </span></i><span lang="EN-US" style="font-family: "Calibri","sans-serif"; mso-ansi-language: EN-US;">Richard Feynman</span><span style="font-family: "Calibri","sans-serif";"><o:p></o:p></span></div>
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<span lang="EN-US" style="font-family: "Calibri","sans-serif"; mso-ansi-language: EN-US;">In our investigation centric age you are likely
someday to find yourself nodding blandly at an MRI head report with the words
“age related changes with SVD only” hanging loosely in the opening
sentence. For all our sense of familiarity with small vessel disease (SVD) its
apparent unobtrusiveness disguises a world of complexity. </span><span style="font-family: "Calibri","sans-serif";"><o:p></o:p></span></div>
<div class="MsoNormal">
<span lang="EN-US" style="font-family: "Calibri","sans-serif"; mso-ansi-language: EN-US;"><br /></span></div>
<div class="separator" style="clear: both; text-align: center;">
<a href="http://www.strokegenomics.org/img/WMD_Hyperintensity.jpg" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" src="http://www.strokegenomics.org/img/WMD_Hyperintensity.jpg" /></a></div>
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<span lang="EN-US" style="font-family: "Calibri","sans-serif"; mso-ansi-language: EN-US;"><br /></span></div>
<div class="MsoNormal">
<span style="font-family: Calibri, sans-serif;">The putative role for SVD in neurological, psychiatric
and cognitive disease burden is huge. It is responsible for around a
fifth of all strokes, doubles the overall risk and contributes to around half
of all dementias. In this months Lancet Neurology, Joanna Wardlaw produced a
well thought out review which goes into detail about the who’s who of this
disease.</span></div>
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<span lang="EN-US" style="font-family: "Calibri","sans-serif"; mso-ansi-language: EN-US;">But lets go back a step. What actually is
SVD? On pathological sections, typical T1-, T2-weighted imaging and FLAIR
scans, SVD appears as a combination of small subcortical hemorrhages and
infarctions, their resultant lacunes (CSF filled cavities) and brain
atrophy. With the advent of more advanced forms of imaging such as
diffusion tensor imaging and magnetization ratio, the more indolent effects of
SVD; altered myelination, focal thinning of cortical grey matter and even
disruption to axonal transport have been observed. This is all well and
good, but the key question is what is the actual cause of these phenomenon? </span><span style="font-family: "Calibri","sans-serif";"><o:p></o:p></span></div>
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<span lang="EN-US" style="font-family: "Calibri","sans-serif"; mso-ansi-language: EN-US;">At a histopathological level SVD appears as a diffuse,
intrinsic disease of small arterioles with a characteristic appearance of
damage from inflammatory cell infiltration into the vessel wall and
perivasculature. Hypertension, vasospasm and microatheroma have all been
put forward as possible mechanisms for this. </span><span style="font-family: "Calibri","sans-serif";"><o:p></o:p></span></div>
<div class="MsoNormal">
<span lang="EN-US" style="font-family: "Calibri","sans-serif"; mso-ansi-language: EN-US;"><br /></span></div>
<div class="MsoNormal">
<span lang="EN-US" style="font-family: "Calibri","sans-serif"; mso-ansi-language: EN-US;">B</span><span style="font-family: Calibri, sans-serif;">ut there’s a difficulty…</span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-US" style="font-family: "Calibri","sans-serif"; mso-ansi-language: EN-US;">The bare bones of it is that while SVD and cortical
strokes are globally associated with vascular risk factors, in studies of
normotensive patients with SVD verified at autopsy a <i>majority</i> showed
limited vascular risk factors. While hypertension is known associated
with the development of white matter hyperintensities (another radiological
sign of SVD) the mechanism by which these lead to vascular damage is convoluted
at best. There is a suggestion, that part of the observed association we
see between SVD and hypertension may actually be due in part to co-localisation
of genetic loci for susceptibility to both these conditions. </span><span style="font-family: "Calibri","sans-serif";"><o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-US" style="font-family: "Calibri","sans-serif"; mso-ansi-language: EN-US;">So if hypertension isn’t the only answer, what else
might be? Wardlaw and colleagues noticed that in many
pathological specimens of lacunar infarcts, a central perforating arteriole
with a thickened wall runs through the centre of the infarct <i><u>rather </u></i>than
proximal to it. This suggests that rather than occlusion and resultant
hypoxia occurring downstream of an atheromatous lesions (as we are familiar
with), something else is happening directly around the affected vessel that
leads to the eventual infarction. That something, the authors suggest, is
endothelial dysfunction. </span><span style="font-family: "Calibri","sans-serif";"><o:p></o:p></span></div>
<div class="MsoNormal">
<br /></div>
<div class="MsoNormal">
<span lang="EN-US" style="font-family: "Calibri","sans-serif"; mso-ansi-language: EN-US;">The cells of the cerebrovascular endothelium are most
closely “packed” at the level of capillaries with widening gap junctions in
arterioles and venules. With advancing age, inflammation and oxidative
stress this barrier becomes more permeable. Interestingly, in animal
models of SVD, short exposure to salt (a known risk factor) is enough to cause
a worsened degree of the latter two of these alongside small vessel
pathology. The authors suggest that in areas of the brain which have
impaired endothelial function, a leakage of plasma contents and migration of
inflammatory cells ensues. This leads to distortion of the arteriolar
lumen eventually leading to thrombosis. Perivascular tissue damage also takes
place, resulting in the white matter demyelination and dilatation of
perivascular spaces observed as hyperintensities on MRI. </span></div>
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<span lang="EN-US" style="font-family: "Calibri","sans-serif"; mso-ansi-language: EN-US;"><br /></span></div>
<div class="MsoNormal">
<span lang="EN-US" style="font-family: "Calibri","sans-serif"; mso-ansi-language: EN-US;">By Andrew Cummings (Academic F2, Barts Health)</span></div>
</div>
Gavin Giovannonihttp://www.blogger.com/profile/03634514099871112077noreply@blogger.com1tag:blogger.com,1999:blog-895935989949155387.post-46578270912353659002013-04-03T08:02:00.001+01:002013-04-03T08:05:29.775+01:00Case study: apnoea post cardiac arrest<div dir="ltr" style="text-align: left;" trbidi="on">
<span style="font-family: inherit;">You are called to see a patient who has had a cardiac arrest and was successfully resuscitated and is the intensive care unit in a "coma". The patient is not on a ventilator and is breathing spontaneously. You notice that the patient has periods of apnoea. </span><span style="font-family: inherit;">The following is a trace of the patient's breathing pattern:</span><br />
<span style="font-family: inherit;"><br /></span>
<br />
<div class="separator" style="clear: both; text-align: center;">
<a href="http://3.bp.blogspot.com/-2SufBYckiZ8/UVvOkMB4_jI/AAAAAAAABCM/CJ4Hx0X0cCE/s1600/Breathing-ataxic.png" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><span style="font-family: inherit;"><img border="0" height="344" src="http://3.bp.blogspot.com/-2SufBYckiZ8/UVvOkMB4_jI/AAAAAAAABCM/CJ4Hx0X0cCE/s640/Breathing-ataxic.png" width="640" /></span></a></div>
<span style="font-family: inherit;"><br /></span>
<span style="font-family: inherit;"><b>Questions</b>:</span><br />
<span style="font-family: inherit;"><br /></span>
<span style="font-family: inherit;"><b>Level 1</b>:</span><br />
<span style="font-family: inherit;"><br /></span>
<span style="font-family: inherit;">How do you define apnoea?</span><br />
<span style="font-family: inherit;"><br /></span>
<span style="font-family: inherit;"><b>Level 2</b>:</span><br />
<span style="font-family: inherit;"><br /></span>
<span style="font-family: inherit;">What is this pattern or breathing called?</span><br />
<span style="font-family: inherit;"><br /></span>
<span style="font-family: inherit;">What is the prognostic significance of this breathing pattern?</span><br />
<span style="font-family: inherit;"><br /></span>
<span style="background-color: white; line-height: 18.18181800842285px;"><span style="font-family: inherit;">How does it differ from Cheyne-Stokes respiration?</span></span><br />
<span style="background-color: white; line-height: 18.18181800842285px;"><span style="font-family: inherit;"><br /></span></span>
<span style="background-color: white; line-height: 18.18181800842285px;"><span style="font-family: inherit;"><b><a href="https://sites.google.com/a/giovannoni.net/teach-neurology/4-case-studies/4-06-brainstem/apnoea/answ">Answers</a></b></span></span></div>
Gavin Giovannonihttp://www.blogger.com/profile/03634514099871112077noreply@blogger.com0tag:blogger.com,1999:blog-895935989949155387.post-83168937331458273392013-01-19T14:24:00.000+00:002013-01-19T14:24:03.789+00:00The Neurological Exam<div dir="ltr" style="text-align: left;" trbidi="on">
<span style="font-size: large;"><b>Level 1</b>: the neurological examination</span><br />
<span style="font-size: large;"><br /></span>
<span style="font-size: large;">I found a very good site that is well indexed with embedded videos of the neurological examination. You may want to use this as a refresher. </span><br />
<span style="font-size: large;"><br /></span>
<span style="font-size: large;"><a href="http://www.neuroexam.com/neuroexam/">Neuroexam</a></span><br />
<span style="font-size: large;"><br /></span>
<span style="font-size: large;">Knowing how to perform the neurological examination and knowing the relevant functional neuroanatomy that underpins the examination is vital if you want to graduate from medical school a neurophile. The alternative is neurophobia and a life of professional hell every time you have to see a patient with a neurological complaint. </span></div>
Gavin Giovannonihttp://www.blogger.com/profile/03634514099871112077noreply@blogger.com0tag:blogger.com,1999:blog-895935989949155387.post-19953616919556566732013-01-13T14:48:00.001+00:002013-01-14T23:32:11.191+00:00Blink reflex or optical reflex<div dir="ltr" style="text-align: left;" trbidi="on">
<span style="font-size: large;"><b>Level 1</b>: optical reflex &<b> Level 3</b>: blindsight</span><div>
<span style="font-size: large;"><br /></span></div>
<div>
<span style="font-size: large;"><b>Optical reflex</b>: In addition to the corneal or sensory blink reflex, blinking can be elicited by visual or auditory input; i.e. bright lights, central and peripheral stimuli and loud noises. The evolutionary purpose of this reflex is again to protect the eyes from foreign bodies and bright lights. Blinking in response to a threatening visual stimulus is known as the optical reflex. </span></div>
<div>
<span style="font-size: large;"><br /></span></div>
<div>
<span style="font-size: large;"><b>Neuroanatomy and neurophysiology</b>: The optical reflex is subcortical and is controlled via a pathway that bypasses the lateral geniculate body, optic radiations and visual cortex and goes directly to the superior colliculi. This pathway then relays to several brain stem and spinal nuclei via the tectobulbar and tectospinal pathways that initiate the motor response; i.e. blinking, flexing your arms upwards to protect your eyes, flexion and lateral rotation of the head away from the stimulus and finally flexion of the trunk and lower limbs to duck under the stimulus. Most of us will have experienced this reflex, for example when an insect flies suddenly into our field of vision, or when we duck or dive to prevent being hit in the face by a tennis or football, or the reflex ducking of the head to prevent hitting an overhanging branch when walking. This neuronal pathway is responsible for reflex movements in response to visual stimuli and is the pathway that allows us to play ball sports that require very rapid movements, for example tennis, cricket, baseball etc. A cricketer or tennis player hits the ball before they become consciously aware of the ball. </span><br />
<div>
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<a href="http://4.bp.blogspot.com/-tKUNRpub2xg/UPK_AOmvM7I/AAAAAAAAAsI/HZHaSs7o3WU/s1600/Optic+Reflex.png" imageanchor="1" style="margin-left: 1em; margin-right: 1em;"><img border="0" height="640" src="http://4.bp.blogspot.com/-tKUNRpub2xg/UPK_AOmvM7I/AAAAAAAAAsI/HZHaSs7o3WU/s640/Optic+Reflex.png" width="620" /></a></div>
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<span style="font-size: large;"><b>Clinical Utility</b>: The optical reflexes can be used to test visual function in patients who are semi-concious or unconscious and indicate that the retina and brain stem are functioning. This reflex is particularly useful in young children. It is also used in patient who present with blindness; if present it indicates that the lesion is very posterior, i.e. cortical, or the patient has functional or hysterical blindness. In these situations the pupillary reflexes are also present , but not the <a href="http://en.wikipedia.org/wiki/Optokinetic_reflex">optokinetic response or reflex</a> that is a more complex visual reflex that relies cortical functioning to activate it. </span></div>
<div>
<span style="font-size: large;"><br /></span></div>
<div>
<span style="font-size: large;">The optical reflex is usually tested creating a threatening lateral visual stimulus, typically your finger or hand that is brought into the lateral visual field very rapidly. There is one caveat to the so called hand method is that it may cause a gust of air over the cornea that can stimulate the corneal reflex. To prevent this from happening it can be done from behind a glass screen. This is rarely necessary in a clinical situation. </span></div>
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<div class="separator" style="clear: both; text-align: center;">
<object class="BLOGGER-youtube-video" classid="clsid:D27CDB6E-AE6D-11cf-96B8-444553540000" codebase="http://download.macromedia.com/pub/shockwave/cabs/flash/swflash.cab#version=6,0,40,0" data-thumbnail-src="http://2.gvt0.com/vi/JxD_v71XmBY/0.jpg" height="266" width="320"><param name="movie" value="http://www.youtube.com/v/JxD_v71XmBY&fs=1&source=uds" /><param name="bgcolor" value="#FFFFFF" /><param name="allowFullScreen" value="true" /><embed width="320" height="266" src="http://www.youtube.com/v/JxD_v71XmBY&fs=1&source=uds" type="application/x-shockwave-flash" allowfullscreen="true"></embed></object></div>
<span style="font-size: x-small;"></span><br />
<div style="text-align: center;">
<span style="font-size: x-small;">This science experiment shows the optical blink reflex very well!</span></div>
<span style="font-size: x-small;">
</span><br />
<div>
<span style="font-size: large;"><br /></span></div>
<div>
<span style="font-size: large;">The optical reflex is responsible for the clinical phenomenon of blindsight, i.e. patients responding to visual stimuli without being of aware of it. The majority of these patients are conciously awarr of being blind, but rarely they may deny being blind. The latter is referred to as the</span><span style="font-size: large;"> <a href="http://en.wikipedia.org/wiki/Anton%27s_syndrome">Anton-Babinski syndrome</a>. </span></div>
<div>
<br /></div>
<span style="font-size: large;">The following is a short clip from the two-part documentary "Phantoms in the Brain" in which neurologist V S Ramachandran describes how the study of patients with certain types of brain damage can give us clues about the nature of consicousness and perception. For those of you who are interested in this can see both documentaries for free online. </span><br />
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<iframe allowfullscreen='allowfullscreen' webkitallowfullscreen='webkitallowfullscreen' mozallowfullscreen='mozallowfullscreen' width='320' height='266' src='https://www.youtube.com/embed/RuNDkcbq8PY?feature=player_embedded' frameborder='0'></iframe></div>
<span style="font-size: x-small;"></span><br />
<div style="text-align: center;">
<span style="font-size: x-small;">"Phantoms in the Brain" V S Ramachandran </span></div>
<span style="font-size: x-small;">
</span><span style="font-size: large;"><br />For those of you who are interested you may find this case study of interest: </span></div>
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<span style="font-size: large;"><br /></span><a href="http://brain.oxfordjournals.org/content/126/2/267.long"><span style="font-size: large;">Hamm et al. </span><span style="font-size: large;">Affective blindsight: intact fear conditioning to a visual cue in a cortically blind patient. </span><span style="font-size: large;">Brain. 2003 Feb;126(Pt 2):267-75.</span></a><span style="font-size: large;"><br /><br />Blindsight refers to remarkable residual visual abilities of patients with damage to the primary visual cortex (V1). Recent studies revealed that such residual abilities do not apply only to relatively simple object discriminations, but that these patients can also differentially categorize and respond to emotionally salient stimuli. The current study reports on a case of intact fear conditioning to a visual cue in a male patient with complete bilateral cortical blindness. The patient was admitted to the stroke unit of the neurological department because of complete loss of vision. Both CT and structural MRI scans confirmed lesions in both territories of the posterior cerebral artery. No visual evoked potentials could be detected confirming complete cortical blindness. During fear conditioning, a visual cue predicted the occurrence of an aversive electric shock. Acoustic startle probes were presented during and between the conditioned stimuli. Relative to the control condition, startle reflexes were substantially potentiated when elicited in the presence of the conditioned stimuli. No such potentiation was observed prior to conditioning. These data suggest that fear learning to visual cues does not require a cortical representation of the conditioned stimulus in the primary sensory cortex and that subcortical pathways are sufficient to activate the fear module in humans.</span></div>
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<span style="font-size: large;"><b>Further reading</b>: </span><a href="http://en.wikipedia.org/wiki/Blindsight" style="font-size: x-large;">blindsight</a></div>
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Gavin Giovannonihttp://www.blogger.com/profile/03634514099871112077noreply@blogger.com3tag:blogger.com,1999:blog-895935989949155387.post-19161751552777399682013-01-11T11:07:00.000+00:002013-01-11T11:19:24.005+00:00Multiple sclerosis lecture - Brain and Behaviour 2<div dir="ltr" style="text-align: left;" trbidi="on">
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<span style="font-size: large;"><b>Level 1</b>: Year-2 Brain & Behaviour Lecture on 17th Jan 2013</span><br />
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<span style="font-size: large;"><b>Saltatory axonal conduction!</b></span></div>
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Gavin Giovannonihttp://www.blogger.com/profile/03634514099871112077noreply@blogger.com0tag:blogger.com,1999:blog-895935989949155387.post-1115316557784367832013-01-11T09:15:00.003+00:002013-01-11T09:17:29.620+00:00The corneal or blink reflex<div dir="ltr" style="text-align: left;" trbidi="on">
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<b><span style="font-size: large;">Level 1</span></b></div>
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<span style="font-size: large;"><b>Description</b>: The corneal is one of the blink reflexes, is an involuntary blinking of the eyelids elicited by stimulation of the cornea. Stimulation should elicit both a direct and indirect or consensual response (opposite eye). The reflex consumes a rapid rate of 0.1 second. The evolutionary purpose of this reflex is to protect the eyes from foreign bodies. </span><br />
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<span style="font-size: large;"><b>Neuroanatomy</b>: As will all reflexes it has an afferent (sensory) and efferent (motor) arm. The reflex is mediated by the nasociliary branch of the ophthalmic branch (Vi) of the trigeminal or 5th cranial nerve that senses the stimulus on the cornea, lid, or conjunctiva. The temporal and zygomatic branches of the facial or 7th cranial nerve initiates the motor response. The reflex is driven via interneurones in the medulla. </span><br />
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<span style="font-size: large;"><b>Interpretation</b>: An absent corneal reflex can be due to sensory loss in Vi (e.g. neuropathy or ganglionpathy), weakness or paralysis of the facial muscles (myopathy) or facial nerve (facial palsy, for example <a href="http://en.wikipedia.org/wiki/Bell%27s_palsy">Bell's palsy</a>) or brain stem disease. For a myopathy to cause a loss of the blink reflex the weakness has to be very severe, for example a <a href="http://en.wikipedia.org/wiki/Chronic_progressive_external_ophthalmoplegia">chronic progressive external ophthalmoplegia</a> (CPEO)</span></div>
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<span style="font-size: large;">Contact lenses may diminish or abolish the testing of this reflex; therefore an absent corneal reflex is not necessarily abnormal. The examination of the corneal reflex is useful in unconscious patients and if present indicates that the lower brain stem is functioning. It is used as part of the assessment for determining if someone is brain dead; if the corneal reflex is present the person can't be diagnosed with brain death.</span></div>
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<span style="font-size: large;"><b>Clinical demonstration</b>: The following YouTube video shows you how to do a corneal reflex:</span></div>
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<span style="font-size: large;"><b>Neurophysiology</b>: The blink reflex can be tested electrophysiologically by stimulating the supra-orbital nerve and measuring the blink in both eyes. The ipsilateral blink occurs quicker (R1 component) compared to the contralateral blink that occurs a few milliseconds later with the R2 component. In the figure below you will notice that the R2 component affects both eyes, i.e. the ipsilateral eye has a double input. The figure below demonstrates the hypothesized wiring diagram of the blink reflex. </span></div>
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Gavin Giovannonihttp://www.blogger.com/profile/03634514099871112077noreply@blogger.com3tag:blogger.com,1999:blog-895935989949155387.post-19293290141538961932013-01-11T08:42:00.002+00:002013-01-11T08:42:27.619+00:00James Parkinson's London<div dir="ltr" style="text-align: left;" trbidi="on">
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<span style="font-size: large;">It should be compulsory for all medical students at Barts and The London to watch this video:</span><br />
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<span style="font-size: large;"><i>"Professor Gerald Stern, Emeritus Professor of Neurology UCL, who narrates this short documentary is an alumnus of The London Medical School. He also grew up in Whitechapel; his grandfather ran a general store on the Whitechapel Road next to the Whitechapel Bell Foundry on the site that is now the East London Mosque! Professor Stern is one of my mentors and a great neurologist." </i></span></div>
Gavin Giovannonihttp://www.blogger.com/profile/03634514099871112077noreply@blogger.com0tag:blogger.com,1999:blog-895935989949155387.post-7015684760850991712013-01-04T08:12:00.001+00:002013-01-04T08:12:24.710+00:004th-yr lecture notes on multiple sclerosis<div dir="ltr" style="text-align: left;" trbidi="on">
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<span style="font-size: large;"><b>Level 1:</b> the following are my lecture notes and presentation from my lecture on the 17th December 2012. These can be downloaded. </span><br />
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Gavin Giovannonihttp://www.blogger.com/profile/03634514099871112077noreply@blogger.com1