Efficacy of therapeutic plasma exchange for treatment of stiff-person syndrome.

BACKGROUND: The efficacy of therapeutic plasma exchange (TPE) in stiff-person syndrome (SPS) is unclear. STUDY DESIGN AND METHODS: A retrospective analysis of patients diagnosed with SPS who underwent TPE and a systematic literature review were conducted. RESULTS: 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 percent (n = 7) of patients demonstrated at least minimal clinical improvement and 56% (n = 5) had a significant response. Most of the patients who demonstrated a significant response to treatment improved and their symptoms stabilized. Two patients (22%) developed adverse events, including catheter-associated infection and transient hypotension. Eighteen publications were found from the literature review, which resulted in a total of 26 patients diagnosed with SPS. Forty-two percent (n = 11) of patients had a significant symptomatic improvement after TPE treatment, and two patients (8%) developed adverse events. CONCLUSION: TPE may benefit patients with SPS who are not responsive to first-line therapy, and it is well tolerated.