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October 1965

A Kinship With the Roussy-Levy Syndrome: A Clinical and Electrophysiologic Study

Author Affiliations

From the Mayo Clinic and Mayo Foundation, Section of Neurology (Dr. Dyck) and Section of Physiology (Dr. Lambert). Mayo Graduate School of Medicine, University of Minnesota, Resident in Neurology (Dr. Yudell).

Arch Neurol. 1965;13(4):432-440. doi:10.1001/archneur.1965.00470040098016

IN 1926, Roussy and Lévy1 described a familial syndrome characterized by (1) clumsy gait, (2) abnormalities of equilibrium which were evident on standing and more pronounced with eye closure, (3) areflexia, (4) clubfeet, (5) awkwardness and weakness of the hands with slight atrophy of the small muscles of the hand, (6) decreased faradic and galvanic excitability of muscles, and (7) atypical tremor of the hands, made evident by tensing the muscles, increased with movement but absent at rest. In 1932, they2 included the signs that Popow3 had reported as part of the syndrome in five affected patients—symmetric thinning of the lower one third of the legs and impaired vibratory sensibility. In another article on this syndrome, Roussy and Levy4 stressed the absence of: characteristic cerebellar signs, speech disturbances, Babinski signs, nystagmus, cranial nerve abnormalities, definite sensory loss, intellectual impairment, and marked muscle atrophy. The

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