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Spinocerebellar degeneration secondary to chronic intestinal malabsorption: a vitamin E deficiency syndrome

Harding, A.E., Muller, D.P., Thomas, P.K., and Willison, H.J. (1982) Spinocerebellar degeneration secondary to chronic intestinal malabsorption: a vitamin E deficiency syndrome. Annals of Neurology, 12 (5). pp. 419-424. ISSN 0364-5134

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Abstract

Two adults are described who developed a progressive neurological disorder more than 20 years after the onset of chronic fat malabsorption. The clinical features included dysarthria, cerebellar ataxia, and prominent proprioceptive loss with depressed or absent tendon reflexes. Serum vitamin E was undetectable in both cases. One patient improved clinically and electrophysiologically after oral therapy with vitamin E. The findings in these patients were similar to those in others recently reported with vitamin E deficiency associated with biliary atresia. Electrophysiological observations suggested that the human deficiency state parallels that found neuropathologically in vitamin E-deficient animals.

Item Type:Article
Status:Published
Refereed:Yes
Glasgow Author(s) Enlighten ID:Willison, Prof Hugh
Authors: Harding, A.E., Muller, D.P., Thomas, P.K., and Willison, H.J.
College/School:College of Medical Veterinary and Life Sciences > Institute of Infection Immunity and Inflammation
Journal Name:Annals of Neurology
ISSN:0364-5134

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