Differential Diagnosis

Besides Huntington disease, other less common hereditary choreas include benign familial chorea, familial parox ysmal choreoathetosis, and neuroacanthocytosis. Other forms of hereditary neurodegeneration that share the same pathogenesis of polyG aggregation and may be confused with HD include dentatorubropallidoluysian atrophy (DRPLA) and spinocerebellar ataxia (SCA) types 1, 2, 3, 6, 7, and 17 (Stacy and Jankovic 1992).

If the patient lacks a family history for a choreic or psychiatric disorder, then the clinician should consider the following disorders: senile chorea, tardive dyskinesia, central nervous system (CNS) vasculitis, subdural hematoma, Wilson disease, pantothenate kinase-associated neurodegeneration, Sydenham chorea, antiphospholipid antibody syndrome, Creutzfeldt-Jakob disease, and various toxic and metabolic disorders. The specific toxins causing chorea include oral contraceptives, levodopa, CNS stimulants, neuroleptics, phenytoin, carbamazepine, ethosuximide, and other drugs. Metabolic-endocrine disorders associated with chorea include chorea gravidarum, thyrotoxicosis, hypoparathyroidism, hypernatremia, Addison disease, and chronic hepatocerebral degeneration, among others (Stacy and Jankovic 1992b).

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