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Plasma exchange in the treatment of Refsum's disease (heredopathia atactica polyneuritiformis).
  1. D Harari,
  2. F B Gibberd,
  3. J P Dick,
  4. M C Sidey
  1. Department of Neurology, Westminster Hospital, London, UK.


    Five cases of heredopathia atactica polyneuritiformis (HAP--Refsum's disease) were treated by serial plasma exchanges. In all patients a reduction in calorie intake and body weight had been associated with a rise in plasma phytanic acid, followed by an exacerbation of the ataxia and neuropathy. Lowering the plasma phytanic acid by plasma exchange produced a rapid clinical improvement. The main indication for plasma exchange in HAP is a severe or rapidly worsening clinical condition. A lesser indication is failure of dietary management to reduce a high plasma phytanic acid level.

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