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Morning ReportAugust 7, 2012
Good Morning
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Chorea
**Show video**
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SymptomsAcute /subacute Chronic
Localized DiffuseSingle MultipleStatic Progressive
Constant IntermittentSingle Episode Recurrent
Abrupt GradualSevere MildPainful NonpainfulBilious Nonbilious
Sharp/Stabbing Dull/Vague
Problem Characteristics
Ill-appearing/Toxic
Well-appearing/Non-toxic
Localized problem
Systemic problem
Acquired Congenital
New problem Recurrence of old problem
Semantic Qualifiers
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Illness Scripts
Predisposing Conditions Age, gender, preceding events
(trauma, viral illness, etc), medication use, past medical history (diagnoses, surgeries, etc)
Pathophysiological Insult What is physically happening in
the body, organisms involved, etc.
Clinical Manifestations Signs and symptoms Labs and imaging
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Causes of Chorea**
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Diffusion-Weighted MRI
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Normal Vasculature
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MRI-T2
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MRI
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Predisposing Conditions
Bimodal age distribution First decade: 5-7 years old Fifth decade
Females > Males (2:1)Asian (especially Japanese)Associations
Down’s Syndrome Neurofibromatosis Type 1 Sickle Cell Disease Prior intracranial irradiation Autoimmune/collagen vascular disorders
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Pathophysiology
Chronic progressive cerebrovascular disorder Progressive stenosis of the terminal portion of
bilateral internal carotid arteries Also stenosis of the main branches of the internal
carotids (esp. middle and anterior cerebral arteries)
This leads to abnormally dilated collateral vessels at the base of the brain
Exact cause unknown General vascular dysplasia Genetic involvement? Autoimmune, environmental, infectious?
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Clinical ManifestationsResults from poor cerebral
perfusion and infarction Cause of 6% of pediatric
strokes Fixed, unilateral neurologic
deficit Often hemiplegia
SeizuresInvoluntary movement
disordersHeadacheTransient Ischemic Attacks
Precipitated by crying, coughing, blowing
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ImagingCT MRI CT Angiogram (gold standard)“Puff of Smoke”…Moyamoya
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Other
Treatment Neurosurgical revascularization…has been shown to
decrease the risk of stroke Aspirin: prevents micro-thrombi formation at the sites of stenosis Anti-epileptic medications Calcium channel blockers Steroids (as in our patient with chorea)
Early diagnosis and surgical management are important
Long-term outcome is most closely related to patient’s neurologic status at diagnosis and treatment
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Thanks!!
Noon conference = YOGA (12:15)