This Article  • References  • Full text PDF  • Reply to article  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on HighWire  • Citing articles on Web of Science (66)  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Social Bookmarking   What's this?

Report of a Family Through Six Generations

Leslie P. Weiner, MD; Bruce W. Konigsmark, MD; J. Stoll, Jr., MD; John W. Magladery, MD

Arch Neurol. 1967;16(4):364-376.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

Over 70 years ago Menzel¹ presented the anatomic findings of olivopontocerebellar atrophy in a 46-year-old man. Since then, 26 reports have appeared in the world's literature describing pathologically verified examples of this disease.^2-27 In all, the clinical features were similar, with ataxia, incoordination of the hands, and rest tremors predominating. In only two reports has there been association of olivopontocerebellar atrophy and blindness,^11-13 a feature characterizing the family described here.

About 18 years ago a Negro man, case IV-1 of this report, was admitted to the Baltimore City Hospitals because of visual difficulty and unsteady gait. Clinical examination suggested a diagnosis of olivopontocerebellar atrophy in combination with pigmentary retinal disease. To date, 27 members of the kindred over five generations have been afflicted with similar progressive ataxia and blindness. In addition to the two attended in hospital, 12 other affected members of the family have been . . . [Full Text PDF of this Article]

Author Affiliations
Baltimore

From the Division of Neurological Medicine Department of Pathology and Division of Otolaryngology, The Johns Hopkins and Baltimore City hospitals, Baltimore. Dr. Weiner is now a captain in the US Medical Corps, Womack Army Hospital, Fort Bragg, NC. Dr. Stoll is now chief of Neurological Surgery, Rhode Island Hospital, Providence.

Footnotes
Submitted for publication Oct 1, 1966; accepted Oct 25.

Read in part before the 40th Annual Meeting of the American Association of Neuropathologists, Atlantic City, NJ, June 11, 1965.

Reprint requests to The Johns Hopkins Hospital, Baltimore 21205 (Dr. Magladery).

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?