Vitamin E deficiency: pathogenic, clinical, histopathological and electrical features and principal causes

Vitamin E deficiency is frequently observed in human pathology. In most cas es, deficiency is moderate and asymptomatic. Severe deficiency is rare and presents as a progressive neurological syndrome including cerebellar ataxia and posterior cord injury. Neuropathological and electrophysiological feat ures confirm spinocerebellar degeneration. The pathophysiology of vitamin E deficiency nervous dysfunction is still unknown. Oxidative alterations due to the tack of the main lipid-soluble antioxidant could be involved. A few causes of vitamin E deficiency are recognized (malnutrition, impaired lipi d absorption, specific anomaly of hepatic or intestinal secretion of vitami n E, excessive endogenous consumption), but diseases associated with vitami n E deficiency are numerous and divers. Symptomatic severe deficiency is co nstantly observed in ataxia with vitamin E deficiency and abetalipoproteine mia. Intestinal, hepatobiliary, and pancreatic diseases are often associate d with more or less marked deficiency. Other pathological circumstances suc h as malnutrition, alcoholism, hemolytic anemia, renal failure and hemodial ysis could be associated with moderated and asymptomatic vitamin E deficien cy. Oral or regular administration of high-dose vitamin E is required for patie nts with symptomatic severe deficiency. An adapted supplementation is recom mended for patients with a marginal vitamin E status.