13. RHEUMATIC CHOREA After infection with group A ß-hemolytic streptococcus 25% of cases of acute rheumatic fever Average age of onset is 5-15 years Girls > boys According to the 1992 modification of the Jones criteria, chorea alone is sufficient for diagnosis of RF Imbalance among the dopaminergic system, intrastriatal cholinergic system, and inhibitory gamma-aminobutyric acid (GABA) system
14. Cross-reactivity between group A ß-hemolytic streptococcus and the basal ganglia Diagnosis of SC may be difficult. Chorea alone is sufficient for diagnosis providing other causes of the condition have been excluded ASO titre may be elevated Echocardiogram may show carditis Volumetric MRI shows enlargement of the basal ganglia in affected patients without other abnormalities Resolves spontaneously in 3-6 months Recurrences Treatment- Chronic Penicillin prophylaxis for 10 years Steroids Haloperidol, Valproate
15. CHOREA WITH NONKETOTIC HYPERGLYCEMIA Focal neurological symptoms may provide the first clinical clue for the presence of non ketotichyperglycemia Seizures, chorea, Chorea – ballismus syndrome &focal neurological deficits Pathogenesis- depletion of GABA, Cerebral hypoperfusion T1 weighted MRI- hyperintense basal ganglia lesion SPECT studies- Striatalhypoperfusion and striatalhypometaboli Neurological symptoms improve with correction of hyperglycemia.
16. REFERENCES -DeJong’s The Nurological Examination -Harrison’s Principles of Internal Medicine -Web references from medscape