Intramuscular Vitamin E Repletion in Children With Chronic Cholestasis

David H. Perlmutter, Paul Gross, H. Roy Jones, Anne Fulton, Richard J. Grand

Research output: Contribution to journalArticlepeer-review

19 Scopus citations


Progressive spinocerebellar degeneration was identified in six children with chronic cholestatic liver disease and attributed to severe vitamin E deficiency. In addition to areflexia, ataxia, dysmetria, and diminished vibratory and position sense, three patients had pigmentary retinopathy. Abnormalities were present on electromyography, nerve conduction studies, and electroretinography. Because the vitamin E deficiency was not corrected by oral administration of massive doses of vitamin E, vitamin E was administered by the intramuscular route. With doses of 50 to 100 mg of vitamin E every three to seven days, over a 32-month interval vitamin E deficiency and abnormal red blood cell peroxide hemolysis were corrected. Other than discomfort and occasional edema at the site of injection, there were no side effects of parenteral vitamin E therapy. In several other studies intramuscular vitamin E therapy has produced significant neurologic improvement in patients with similar characteristics. In this study clinical progression of spinocerebellar degeneration was arrested but improvement could not be demonstrated despite adequate vitamin E replacement.

Original languageEnglish
Pages (from-to)170-174
Number of pages5
JournalAmerican Journal of Diseases of Children
Issue number2
StatePublished - Feb 1987


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