Morning ReportAugust 7, 2012

Good Morning

Chorea

**Show video**

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

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 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

ImagingCT 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)