At this time the patient's serum lactate, pyruvate, folate, red blood cell folate serum B12, zinc, copper, cholesterol, and the lipoprotein values were all normal. Activities of alanine aminotransferase, aspartate aminotransferase, alkaline phosphatase, and y-glutamyltransferase were raised.After supplementation with vitamin E for 6 months the nystagmus had resolved. After 18 months of treatment the ataxia had lessened, hand coordination was improved, and Romberg's sign was negative. She was still areflexic. DiscussionA neurological syndrome secondary to vitamin E deficiency which has responded to vitamin E supple-, ments has been reported in adults with cystic fibrosis.4 5 Our patient resembled these previous reports but presented much earlier. This was presumably because of impaired bile salt solubility of the vitamin in addition to pancreatic insufficiency. Luminal bile salt concentrations were likely to have been reduced in our patient. Firstly she had multiple resections of the ileum, the site of the active reabsorption of bile salts; secondly, the situation would be exacerbated by stasis in the dilated jejunum producing a 'blind loop' effect; and finally biliary fibrosis would result in impaired biliary excretion.Although this neurological complication seems to be relatively uncommon in cystic fibrosis, it is important to be aware of it. A careful neurological examination of these patients for evidence of cerebellar, posterior column, and peripheral nerve dysfunction is emphasised. Monitoring of serum vitamin E concentrations in all patients would be ideal and is recommended if possible, yet limited laboratory facilities for measurement make this impractical for many paediatricians. In patients with cystic fibrosis, 50 to 400 mg daily of a water miscible product has generally resulted in normal plasma and tissue levels of ct-tocopherol.' 3 6 In the absence of laboratory facilities regular vitamin E supplementation of a water miscible preparation of vitamin E is recommended in a dosage of 100 to 200 mg daily.
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