The clinical manifestations, differential diagnosis, and treatment of benign hereditary chorea (BHC) are reviewed from the Indiana University and University of Minnesota Medical Schools. The diagnosis of BHC is by exclusion. The most common age of onset is about 1 year and walking is often delayed because of clumsiness and falling. After a short period of progression in severity, movements reach a plateau and may sometimes appear to improve. IQ is normal or low-average. In most reported families, the inheritance is autosomal dominant. Treatment with various drugs, eg. haloperidol, chlorpromazine, and prednisone, is only partially effective, and the chorea is usually refractory. [1]
COMMENT. BHC is an uncommon form of childhood chorea that has been mistaken for Huntington disease. The recognition of this benign, nonprogressive form of chorea of early onset and without dementia will avoid unnecessary prolonged hospitalization and the risk of invasive diagnostic procedures.