Plasma exchange in the treatment of Refsum's disease (heredopathia atactica polyneuritiformis).

Abstract

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.

• Authors: D Harari, F B Gibberd, J P Dick, M C Sidey
• Journal: Journal of neurology, neurosurgery, and psychiatry (J Neurol Neurosurg Psychiatry) Vol. 54 Issue 7 Pg. 614-7 (Jul 1991) ISSN: 0022-3050 [Print] England
• PMID: 1716665 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Phytic Acid

Topics

• Adult
• Body Weight (physiology)
• Female
• Humans
• Male
• Middle Aged
• Neurologic Examination
• Phytic Acid (administration & dosage, blood)
• Plasma Exchange
• Refsum Disease (blood, diet therapy, therapy)