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April 1968

Acute Intermittent Familial Cerebellar Ataxia

Author Affiliations

New Orleans
From the Department of Psychiatry and Neurology, Tulane University Medical School, New Orleans.

Arch Neurol. 1968;18(4):350-357. doi:10.1001/archneur.1968.00470340036002

IN RECENT years we have had the opportunity to study a large family, many of whose members develop an acute, intermittent cerebellar ataxia. The disorder is so common in this kinskip, particularly in childhood, that it is easily recognized by the family and often no medical attention is sought, experience having taught that full recovery will follow without specific treatment. However, 11 patients have been hospitalized and studied at the Charity Hospital of Louisiana at New Orleans.

Affected members have more frequent, more prolonged, and more severe attacks in childhood. The youngest patient was 17 months old at the onset of his first attack. We do not have hospital or clinic records to document any attacks after 15 years of age, but family members give consistent and graphic reports of milder forms of the disorder occurring from the ages of 15 to 60 years.

The clinical picture in the

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