A hereditary familial disease, transmitted as an autosomal dominant trait, characterized by attacks of choreoathetosis and torsion without loss of consciousness, each episode lasting from a few minutes to several hours. The attacks never occur during sleep and, at their height, are similar to those in Huntington’s chorea. Alcoholic beverages, coffee, hunger, fatigue, emotional stress, and tobacco are the precipitating factors. The presence of an atypical Kayser-Fleischer ring is the principal ocular feature.
This disturbance has been used as a synonym for Huntington’s disease, but these are separate entities. Huntington’s disease is entered under George Sumner Huntington, American physician, 1850-1916.
- L. A. Mount, S. Rebock:
Familial paroxysmal choreoathetosis. Preliminary report on a hitherto undescribed clinical syndrome.
Archives of Neurology, Chicago, 1940, 44: 841-847.