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Ataxia with vitamin E deficiency: biochemical effects of malcompliance with vitamin E therapy
M Schuelke1, B Finckh, E A Sistermans
1Department of Neuropediatrics Charité, Humboldt University, Berlin, Germany. markus.schuelke@charite.de
Neurology
|November 30, 2000
Abstract:
To prevent neuronal damage, patients with ataxia with isolated vitamin E deficiency need lifelong supplementation with high doses of vitamin E. Short interruptions of therapy, such as occur in malcompliance, do not lead to clinical symptoms. However, the authors show that even short withdrawals may cause a prolonged decrease of the total radical trapping capacity of plasma; its major contributors, such as urate and sulfhydryl groups, fail to compensate for the missing vitamin E.