Intramuscular vitamin E repletion in children with chronic cholestasis
D. H. Perlmutter, P. Gross, H. R. Jones, A. Fulton and R. J. Grand
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.
