[Ataxia due to vitamin E deficiency]. / Ataksi pa grunn av vitamin E-mangel.

ABSTRACT

Few metabolic or degenerative ataxias can be treated pharmacologically. However ataxia due to vitamin E deficiency can be treated effectively with vitamin E supplementation if it is diagnosed early. We describe two ataxic patients with vitamin E deficiency where this was the definite or probable cause of the ataxia. Both polyneuropathy and cerebellar dysfunction were found. The deficiency was due to intestinal resection in one case, whereas the exact mechanism was unknown in the other case. In one of the patients there was a clear improvement of the ataxia after vitamin E supplementation, but this had to be taken for about six to 12 months. In the other patient the symptom progression was halted, but only slight improvement was observed. This patient therefore underwent leftsided stereotaxic thalamotomy, which markedly alleviated his rightsided ataxic symptoms. We stress the importance of testing for vitamin E (alpha-tocopherol) in all patients with ataxia where there is no other known cause.