1. A PRESENTATION ON ABNORMAL MOVEMENT DISORDERS Dr. Aarthy Joseph Prof. Dr. Mageshkumar’s Unit

5. PATIENT1 PATIENT2 15 year, female, no family history •60 year, female, known diabetic 1 week H/O abnormal movements•2 day H/O abnormal movements Systemic examination •Systemic examination Biochemical and hematological • RBS- 320, urine ketones- neg, Routine investigations- normal other investigations- normal • CXR and ECG- WNL •CT brain • ASO titre- 200 •Neurology opinion •CT brain and MRI brain •Neurology opinion •Cardiology opinion •Ophthalmology opinion •Serum ceruloplasmin •Urinary copper

6. DIAGNOSIS AND MANAGEMENT PATIENT1 PATIENT2 RHEUMATIC CHOREA HYPERGLYCEMIA INDUCED CHOREA TREATMENT Penicillin Glycemic control with insulin Haloperidol Fluid correction

8. Involuntary, rapid, irregular, jerky, purposeless, random, nonrhythmichyperkinesia

9. Present at rest, increased by activity, tension, emotional stress and self consciousness, and disappear in sleep

11. CAUSES Hereditary – Huntington’s disease, benign hereditary chorea, neuroacanthocytosis, dentatorubro-pallidoluysian atrophy Metabolic and endocrine disorders - Wilson disease, nonketotichyperglycemia, hypoglycemiahypobetalipoproteinemia, lipid storage diseases, Kernicterus, hyperthyroidism Paroxysmal - Paroxysmal kinesogenicchoreoathetosis, paroxysmal dystonicchoreoathetosis

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

17. THANK YOU!!