May 1963

Vestibulocerebellar AtaxiaA Newly Defined Hereditary Syndrome with Periodic Manifestations

Author Affiliations

From the Division of Neurology, Department of Medicine, University of North Carolina School of Medicine.

Arch Neurol. 1963;8(5):471-480. doi:10.1001/archneur.1963.00460050021002

A familial disorder is described which is characterized by the initial development in early adult life of periodic attacks of vertigo, diplopia, and ataxia, which recur over a period of many years. These attacks may be followed by the development of a slowly, progressive, cerebellar disease, which eventually may produce motor incapacity. In the many family groups with cerebellar degeneration reported in the literature, no families are described with acute symptoms of vertigo, diplopia, and unsteadiness occurring in periodic attacks over a period of many years and preceding the onset of cerebellar dysfunction.1

Clinical Picture  This disorder has been traced through four generations with involvement of 16 members of a white, rural, North Carolina family group. The pedigree of the family is presented in Fig 1. Four affected members (II-1, III-4, 7, and 8) had died before the onset of this study, and histories concerning them were obtained from

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