A PRESENTATION ON ABNORMAL MOVEMENT DISORDERS

Dr. Aarthy Joseph Prof. Dr. Mageshkumar’s Unit

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

DIAGNOSIS AND MANAGEMENT

PATIENT1 PATIENT2RHEUMATIC CHOREA HYPERGLYCEMIA INDUCED CHOREA

TREATMENTPenicillin Glycemic control with insulinHaloperidol Fluid correction

CHOREA

•Greek word meaning DANCE•Involuntary, rapid, irregular, jerky, purposeless, random, nonrhythmic hyperkinesia•Present at rest, increased by activity, tension, emotional stress and self consciousness, and disappear in sleep•Most characteristic in the distal parts of upper extrimities

CHARACTERISTIC FEATURES:

• One extremity, hemichorea or generalised• Piano playing movements• Milkmaid grip• Parakinesia• Hypotonia and pendular DTR• Hyperpronation• Snake, darting, flycatcher tongue- Motor

impersistence

CAUSES• Hereditary – Huntington’s disease, benign

hereditary chorea , neuroacanthocytosis , dentatorubro-pallidoluysian atrophy

• Metabolic and endocrine disorders - Wilson disease, nonketotic hyperglycemia, hypoglycemia hypobetalipoproteinemia, lipid storage diseases, Kernicterus, hyperthyroidism

• Paroxysmal - Paroxysmal kinesogenic choreoathetosis, paroxysmal dystonic choreoathetosis

• Rheumatic choreaSyndenham’s chorea, Chorea gravidarum

• Drug induced - Neuroleptics, levodopa, oral contraceptives, cocaine, phenytoin

• Toxins - Alcohol intoxication and withdrawal, carbon monoxide , manganese, mercury

• Other choreas associated with systemic disease- SLE, antiphospholipid antibodies, Polycythemia vera,

AIDS• Rarely- Stroke, Vascular malformation, tumours

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

• 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 yearsSteroidsHaloperidol, Valproate

CHOREA WITH NONKETOTIC HYPERGLYCEMIA

• Focal neurological symptoms may provide the first clinical clue for the presence of non ketotic hyperglycemia

• Seizures, chorea, Chorea – ballismus syndrome &focal neurological deficits

• Pathogenesis- depletion of GABA, Cerebral hypoperfusion• T1 weighted MRI- hyperintense basal ganglia lesion• SPECT studies- Striatal hypoperfusion and striatal

hypometaboli• Neurological symptoms improve with correction of

hyperglycemia.

REFERENCES-DeJong’s The Nurological Examination

-Harrison’s Principles of Internal Medicine-Web references from medscape

THANK YOU!!