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July 1968

Hereditary Ataxia of Animals

Author Affiliations

St. Louis
From the Department of Neurology and Neurosurgery, Washington University School of Medicine, St. Louis, and the Jackson Laboratory, Bar Harbor, Me.

Arch Neurol. 1968;19(1):34-46. doi:10.1001/archneur.1968.00480010052003

EXAMPLES of hereditary ataxia in animals provide profitable material for study of the evolution of the causative lesions. Human familial ataxia ordinarily follows a protracted course as do some of the sporadic manifestations of ataxia in animals. Even where a postmortem provides the opportunity for studying the end effects of the pathological process in man, much doubt can remain concerning both the genesis of the lesions and the concordance between the clinical criteria of regression and the distribution and extent of lesions. The brains of animals are eminently suitable for neurochemical studies; correlations between chemical and histological data may be sought under the most favorable circumstances.

The extraordinary complexity of the genetic mechanism makes it more improbable that all human and animal familial ataxias could be traced to a single gene. Ataxia is but a symptom of disorder in certain brainstem cerebellar systems, and identical symptoms could be produced

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