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June 5, 1967

Periodic Ataxia

Author Affiliations

From the Good Samaritan Hospital (Dr. DeCastro), and the University of Cincinnati College of Medicine (Dr. Campbell), Cincinnati.

JAMA. 1967;200(10):892-894. doi:10.1001/jama.1967.03120230144031

IN 1946 Parker1 described a bizarre but clearcut symptom complex consisting of acute transient general cerebellar dyssynergia, affecting all muscles of the body, including those of articulation. These attacks last no longer than a few seconds to a few minutes, and disappear just as suddenly as they come on. The patient then reverts to his former condition, normal or otherwise. He described 11 cases, six associated with some phase of multiple sclerosis. The condition in another four was due to familial cerebellar ataxia, but their periodic attacks were more violent and bitterly complained of. One unusual case was an instance of degenerative spinocerebellar ataxia. This syndrome was termed "periodic ataxia."

In 1959, Andermann et al2 studied two patients with multiple sclerosis who had paroxysmal attacks of dysarthria and ataxia. The cardinal features were the sudden paroxysmal occurrence and brief duration of the attacks.3 The cerebellum or its connections