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Wetherbee Ail*

Published online by Cambridge University Press:  18 September 2015

James M. Powers ,
Dikran S. Horoupian  and
Herbert H. Schaumburg
James M. Powers*
Affiliation:
departments of Pathology (Neuropathology), Medical University of South Carolina and Veterans Administration Hospital, Charleston, South Carolina and the departments of Pathology (Neuropathology) and Saul R. Korey, department of Neurology, Albert Einstein College of Medicine, The Bronx, New York
Dikran S. Horoupian
Affiliation:
departments of Pathology (Neuropathology), Medical University of South Carolina and Veterans Administration Hospital, Charleston, South Carolina and the departments of Pathology (Neuropathology) and Saul R. Korey, department of Neurology, Albert Einstein College of Medicine, The Bronx, New York
Herbert H. Schaumburg
Affiliation:
departments of Pathology (Neuropathology), Medical University of South Carolina and Veterans Administration Hospital, Charleston, South Carolina and the departments of Pathology (Neuropathology) and Saul R. Korey, department of Neurology, Albert Einstein College of Medicine, The Bronx, New York
*
Medical University of South Carolina, 80 Barre St., Charleston, South Carolina 29401, U.S.A.
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Summary

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The neuropathological findings of a Farr family member consist of neuronal loss in the anterior horns and dorsal nuclei of Clarke, neuronal intracytoplasmic inclusions and posterior and lateral column demyelination. This report supports the role of familial amyotrophic lateral sclerosis as a link between common motor neuron disease and classical spinocerebellar degeneration.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 1 , Issue 2 , May 1974 , pp. 139 - 140
Copyright
Copyright © Canadian Neurological Sciences Federation 1974

Footnotes

*

Wetherbee was the name of the Massachusetts family described by Madelaine Brown (1951) as an example of familial progressive muscular atrophy.

References

1.Brown, M.R. (1951). “Wetherbee Ail”. The inheritance of progressive muscular atrophy as a dominant trait in two New England families. New England Journal of Medicine, 243, 645–647.CrossRefGoogle Scholar
2.Hirano, A., Kurland, L.T. and Sayre, G.P. (1967). Familial amyotrophic lateral sclerosis. Archives of Neurology, 16, 232–243.CrossRefGoogle ScholarPubMed
3.McKann, G.M. and Johnson, R.T. (1973). Amyotrophic lateral sclerosis: summary of a conference. Science, 180, 221–222.CrossRefGoogle Scholar
4.Osler, W. (1880). Heredity in progressive muscular atrophy as illustrated in the Farr family in Vermont. Archives of Medicine, 4, 316–320.Google Scholar
5.Woods, B.T. and Schaumburg, H. H. (1972). Nigro-spino-dentatal degeneration with nuclear ophthalmoplegia. Journal of Neurological Sciences, 17, 149–166.CrossRefGoogle ScholarPubMed