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October 7, 1905

ATAXIA OF CENTRAL ORIGIN APPEARING IN CHILDHOOD.

JAMA. 1905;XLV(15):1075-1078. doi:10.1001/jama.1905.52510150039002i
Abstract

This paper is a brief consideration of a form of central nervous disease previously described under the titles of hereditary ataxia, cerebellar ataxia, and not infrequently under the heading of Friedreich's disease. It varies, in certain particulars, however, as we shall see, from the usual conception of what should constitute the affection bearing Friedreich's name. In view of its relative rarity, the number of cases of this affection coming under the observation of any single observer is naturally small.

The clinical characteristics of this affection stated briefly are: Muscular inco-ordination commencing usually in childhood, and, as a rule, first affecting the muscles of the lower extremities, thence extending to the upper extremities, and to the muscles of the trunk, head, larynx, tongue and eyes; slowness of muscular response to volitional impulse; swaying, unsteady gait; slow, hesitating or deliberate speech, sometimes of nasal character and occasionally explosive; nystagmus; oscillation or tremor

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