February 1987

Intramuscular Vitamin E Repletion in Children With Chronic Cholestasis

Author Affiliations

From the Divisions of Gastroenterology and Nutrition (Dr Perlmutter), Neurology (Drs Gross and Jones), and Ophthalmology (Dr Fulton), Children's Hospital; Department of Pediatrics, Harvard Medical School (Drs Perlmutter, Gross, Jones, and Fulton); Division of Pediatric Gastroenterology and Nutrition, Floating Hospital, New England Medical Center (Dr Grand); and Department of Pediatrics, Tufts University School of Medicine (Dr Grand), Boston. Dr Perlmutter is now with the Department of Pediatrics, Washington University School of Medicine, St Louis.

Am J Dis Child. 1987;141(2):170-174. doi:10.1001/archpedi.1987.04460020060027

• 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.

(AJDC 1987;141:170-174)