Over the past decade it has become apparent that
vitamin E is an essential nutrient for maintaining the structural and functional integrity of the developing human nervous system, skeletal muscle, and the retina. The clinical and histologic resemblance of the human neuromuscular disorder associated with chronic fat and
vitamin E malabsorption to that observed in experimental
vitamin E-deficient animal models is striking. Because of chronic malabsorption of
vitamin E, children with CF, chronic
cholestasis,
abetalipoproteinemia, and
short bowel syndrome are at risk for the development of
neurologic deficits caused by
vitamin E deficiency. Correction of the
vitamin E deficiency state prevents, reverses, or, at least, stabilizes the
neurologic dysfunction in susceptible individuals. Advances in stable
isotope technology permit study of the hepatic discrimination among the various stereoisomers and forms of
vitamin E. Investigations into the cause of the primary form of
vitamin E deficiency, the isolated
vitamin E deficiency syndrome, promise to delineate the normal physiologic processes involved in absorption, transport, and tissue delivery of
vitamin E. Studies in progress are addressing the optimal route and form of
vitamin E therapy to be used in each predisposing condition. One major task remaining is to better define the mechanism by which
vitamin E deficiency leads to neurologic injury.