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

• Published in: Annals of neurology Vol. 12; no. 5; p. 419
• Main Authors: Harding, A E, Muller, D P, Thomas, P K, Willison, H J
• Format: Journal Article
• Language: English
• Published: United States 01.11.1982
• Subjects: Adult; Celiac Disease - complications; Cerebellar Ataxia - etiology; Cerebellar Diseases - diagnosis; Evoked Potentials, Somatosensory; Female; Humans; Malabsorption Syndromes - complications; Male; Middle Aged; Nerve Degeneration; Neural Conduction; Short Bowel Syndrome - complications; Spinal Cord Diseases - etiology; Vitamin E - blood; Vitamin E Deficiency - complications
• ISSN: 0364-5134
• DOI: 10.1002/ana.410120503

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.