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January 1943

FAMILIAL TYPE OF PARALYSIS IN INFANTS AND ITS RELATIONSHIP TO OTHER HEREDOFAMILIAL DISORDERS: A CLINICOPATHOLOGIC STUDY

Author Affiliations

SAN FRANCISCO; NEW YORK; SAN FRANCISCO

From the Neurological Institute of New York, the Department of Neurology of Columbia University College of Physicians and Surgeons and the Division of Neuropsychiatry of the Stanford University School of Medicine.

Arch NeurPsych. 1943;49(1):27-42. doi:10.1001/archneurpsyc.1943.02290130035004
Abstract

In spite of the clear differentiation between various heredofamilial neurologic syndromes, many instances of transitional forms have been described from both the clinical and the pathologic viewpoint, particularly among the syndromes of Friedreich's ataxia, Marie's heredocerebellar ataxia, Charcot-Marie-Tooth peroneal muscular atrophy, hereditary spastic paralysis and Leber's hereditary optic nerve atrophy. This report concerns a disease occurring in 3 siblings which does not easily fall under any previously described disease entity. Clinically the condition in 1 case resembled infantile progressive spinal atrophy (Werdnig-Hoffmann disease). Histopathologically it was related to amaurotic family idiocy (Tay-Sachs disease) but presented changes that are also seen in other, supposedly unrelated, forms of heredofamilial disorder.

REPORT OF CASES  The 3 cases occurred in a family in which no previous nervous disorder was known. Both parents were born in Ireland, of Irish stock. During the course of twenty years the mother had been pregnant fourteen times, eleven of

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