A prospective double‐blind study of plasma exchange therapy for the acroparesthesia of Fabry's disease

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Abstract
During a study of the effect of plasma exchange on glycosphingolipid metabolism, a patient with Fabry''s disease noted a dramatic improvement in his painful acroparesthesia. A controlled study was undertaken. Observations were made of nerve conduction times, graded exercise testing and psychometric evaluations during and after 2 planned series of 3 plasma exchanges: one a true plasma exchange and the other a sham control in which the patient received his own plasma. All observers and the patient were blinded and unanimously attributed beneficial results to the sham procedure. This study demonstrates the need for controlled studies in diseases prone to unpredictable exacerbation or spontaneous remission, and outlines 1 possible technique of controlling studies involving plasma exchange.