title: Intramuscular vitamin E repletion in children with chronic cholestasis.
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.
authors.forename: D H
authors.initials: DH
authors.lastname: Perlmutter
authors.forename: P
authors.initials: P
authors.lastname: Gross
authors.forename: H R
authors.initials: HR
authors.lastname: Jones
authors.forename: A
authors.initials: A
authors.lastname: Fulton
authors.forename: R J
authors.initials: RJ
authors.lastname: Grand
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D002779:Cholestasis / Q000150:complications*; D004334:Drug Administration Schedule; D006801:Humans; D007273:Injections, Intramuscular; D013132:Spinocerebellar Degenerations / Q000209:etiology*; D013997:Time Factors; D014810:Vitamin E / Q000008:administration & dosage* / Q000627:therapeutic use; D014811:Vitamin E Deficiency / Q000188:drug therapy* / Q000209:etiology
chemical_list: D014810:Vitamin E