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Article
October 22, 1927

FRIEDREICH'S ATAXIA IN TEN MEMBERS OF A FAMILY

Author Affiliations

ATLANTA, GA.

JAMA. 1927;89(17):1395-1397. doi:10.1001/jama.1927.02690170019006
Abstract

Friedreich's ataxia may be defined as a familial disease manifesting itself at any age during childhood, characterized by imperfect muscular coordination, speech disturbance, and a variety of symptoms and signs varying with different cases. Anatomically, it is characterized by a defective development of the spinal cord. While the disease itself is not rare, the ten cases that I present occurred in one family and constitute, I believe, one of the largest series yet reported.

Direct inheritance is not the rule. This is shown not only by the series of cases on which this report is based but also in those mentioned by others, notably Sachs and Oppenheim. A study of these cases seems to indicate that the percentage of diseased individuals in a family increases with each generation. In the first generation of this series four cases were observed, while in the second generation six cases have so far developed.

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