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March 1938

HEREDITARY CEREBELLAR ATAXIA: Report of a Case and Genetic Study†

Author Affiliations

Ann Arbor, Mich.; Orono, Maine

From the Neuropsychiatric Institute of the University of Michigan.

Arch NeurPsych. 1938;39(3):570-586. doi:10.1001/archneurpsyc.1938.02270030148011

There is no chapter of neurology and neuropathology which is more contradictory than that on the cerebellar ataxias. Strictly speaking, there are three types: (1) the heredocerebellar ataxia of Pierre Marie; (2) the olivopontocerebellar ataxia of Dejerine and Thomas, and (3) the ataxia with cerebellar cortical atrophy. The classification and differentiation of these conditions are difficult because the same parts of the brain stem and cerebellum are frequently affected. The most important property of Marie's type is its outspoken hereditary tendency. This property justifies its classification as a separate entity.

In this report we will consider only the hereditary form.

I. CLINICAL AND PATHOLOGIC STUDY  By Drs. Waggoner and LöwenbergREPORT OF CASEWell developed symptoms of the disease have been observed through five generations in 26 members of a white, American family of 180 members.

History.  —P. M., a man aged 42, was first seen on Feb. 18, 1933.

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