Abstract |
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 (range, 15 to 44 months), 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.
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Authors | D H Perlmutter, P Gross, H R Jones, A Fulton, R J Grand |
Journal | American journal of diseases of children (1960)
(Am J Dis Child)
Vol. 141
Issue 2
Pg. 170-4
(Feb 1987)
ISSN: 0002-922X [Print] United States |
PMID | 3468801
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)
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Chemical References |
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Topics |
- Adolescent
- Child
- Child, Preschool
- Cholestasis
(complications)
- Drug Administration Schedule
- Humans
- Injections, Intramuscular
- Spinocerebellar Degenerations
(etiology)
- Time Factors
- Vitamin E
(administration & dosage, therapeutic use)
- Vitamin E Deficiency
(drug therapy, etiology)
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