Morning Report August 7, 2012 Good Morning

Chorea **Show video**

Symptoms Acute /subacuteChronic LocalizedDiffuse SingleMultiple StaticProgressive ConstantIntermittent Single EpisodeRecurrent AbruptGradual SevereMild PainfulNonpainful BiliousNonbilious Sharp/StabbingDull/Vague Problem Characteristics Ill-appearing/ Toxic Well-appearing/ Non-toxic Localized problemSystemic problem AcquiredCongenital New problem Recurrence of old problem Semantic Qualifiers

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

Causes of Chorea**

Diffusion-Weighted MRI

Normal Vasculature

MRI-T2

MRI

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

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?

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

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

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

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