Abstract
Although paroxysmal choreo-athetosis has been known for 100 years, it is still not universally accepted as an entity and is still usually misconstrued as psychogenic, even by sophisticated neurologists. Recognition of this syndrome and accumulation of valid statistics has been obstructed by its alleged rarity and also by the variety of labels under which it is often disguised. As recent as 1958, under the title "A Novel Case of Conditionally Responsive Extra-Pyramidal Syndrome," Kishimoto1 reported a four-generation pedigree and also three other pedigrees of patients with typical paroxysmal choreo-athetosis. He had studied these patients over a period of 20 years but states that no Japanese neurologist could identify the malady, nor could several European and American neurologists to whom he showed the data and films recognize the disorder. In the concluding sentence of his article he plaintively expresses his perplexity: "Any suggestions of the possible identity of this

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