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Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 1991 Jul;54(7):614–617. doi: 10.1136/jnnp.54.7.614

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

D Harari 1, F B Gibberd 1, J P Dick 1, M C Sidey 1
PMCID: PMC1014432  PMID: 1716665

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.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

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