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September 1930

La maladie de Friedreich.

Arch NeurPsych. 1930;24(3):665-666. doi:10.1001/archneurpsyc.1930.02220150228022

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Abstract

This is a monographic consideration of the disease based on the personal study of twenty-one cases, which apparently have been collected from many of the hospitals in Paris although the work has been done under the aegis of Guillain, Mollaret being his chef de clinique. Although he limits his study to the strictly familial disease originally described by Friedreich, he promises more in a future communication and states that he has come to the conclusion that "there is no sharp boundary separating Friedreich's disease from heredo-ataxia of Pierre Marie."

In Friedreich's disease there are three elementary pathologic traits that may be mixed in various proportions: (1) the cerebellar disturbances manifested in nystagmus and scanning speech; (2) disease of the posterior columns and roots, producing ataxia, abolition of reflexes and sensory disorders, and (3) indications of pyramidal defect resulting in extensor response with reflexes of medullary automatism. The last is usually

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