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The efficacy of therapeutic plasma exchange (TPE) in stiff‐person syndrome (SPS) is unclear.

A retrospective analysis of patients diagnosed with SPS who underwent TPE and a systematic literature review were conducted.

Nine patients with the presumptive diagnosis of SPS who underwent TPE were identified. The mean age was 55 years (range, 34‐72 years) and 78% (n = 7) were female. Anti‐GAD65 was present in 89% (n = 8) of the patients (range, 1.9‐40,000 U/mL), and 33% (n = 3) had a history of diabetes. Forty‐four percent (n = 4) of patients had previously received immunosuppressive medication and 67% (n = 6) received intravenous immune globulin. The main indication for TPE was worsening of symptoms despite treatment with first‐line therapy. Seventy‐eight percent of the patients (n = 7) had five TPE procedures. Seventy‐eight …