[Refsum's disease: 10 years of a diet low in phytanic acid and phytol].

The authors report the evolution of two cases of Refsum's disease, treated with a specific phytol and phytanic acid diet for ten years. This treatment is effective leading to a rapid and stable fall of phytanemia, and a progressive improvement of the peripheral neuropathy and cerebellous symptoms, then prevent from quite new aggravation. Any symptom in favour of a cardiac, pulmonary or renal disease was observed during the past ten years. On the other hand, the sight and hearing disorders remained unchanged and are responsible to a major discomfort in this long-dated evolution. The strict liquid diet is quickly abandoned on account of a bad tolerance (diarrhea), but the ordinary diet is better endured, and is sufficient to keep a low phytanemia; this need a permanent continuation.