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Good Morning 

Good Morning . Morning Report August 7, 2012. Chorea. **Show video**. Semantic Qualifiers. Illness Scripts. Predisposing Conditions Age, gender, preceding events (trauma, viral illness, etc), medication use, past medical history (diagnoses, surgeries, etc) Pathophysiological Insult

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Good Morning 

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  1. Good Morning  Morning Report August 7, 2012

  2. Chorea **Show video**

  3. Semantic Qualifiers

  4. 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

  5. Causes of Chorea**

  6. Diffusion-Weighted MRI

  7. Normal Vasculature

  8. MRI-T2

  9. MRI

  10. 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

  11. 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?

  12. Clinical Manifestations • Results from poor cerebral perfusion and infarction • Cause of 6% of pediatric strokes • Fixed, unilateral neurologic deficit • Often hemiplegia • Seizures • Involuntary movement disorders • Headache • Transient Ischemic Attacks • Precipitated by crying, coughing, blowing

  13. Imaging • CT  MRI  CT Angiogram (gold standard) • “Puff of Smoke”…Moyamoya

  14. 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

  15. Thanks!! Noon conference = YOGA (12:15)

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