Vitamin E adminstration (100 U/kg daily) resulted in improvements in ataxia, motor strength, and mobility in 2 siblings of a consanguineous Bedouin family with Friedreich ataxia and low serum vitamin E levels treated at Soroka Medical Center, Beer Sheva, Israel. The 2 siblings who responded to vitamin E had spinocerebellar symptoms but no peripheral neuropathy. Two siblings complicated by neuropathy failed to respond. Erythrocytes of vitamin E-deficent patients had reduced permeability to acidified glycerol that was partially corrected by vitamin E. [1]
COMMENT. Ataxia with isolated vitamin E deficiency is a rare type of Friedreich ataxia which may be benefited by early administration of large doses of vitamin E. Patients may be vitamin E deficient despite normal intake in the diet. (For further cases and commentary, see Progress in Pediatric Neurology II, PNB Publ, 1994:p497).