title: Efficacy of therapeutic plasma exchange for treatment of stiff-person syndrome.
abstract: BACKGROUND
  The efficacy of therapeutic plasma exchange (TPE) in stiff-person syndrome (SPS) is unclear.
  
  
  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.
  
  
  CONCLUSIONS
  TPE may benefit patients with SPS who are not responsive to first-line therapy, and it is well tolerated.
authors: Puget Sound Blood Center, Seattle, Washington.
authors: Monica B
authors: MB
authors: Pagano
authors: Beth B
authors: BB
authors: Murinson
authors: Aaron A R
authors: AA
authors: Tobian
authors: Karen E
authors: KE
authors: King
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010951:Plasma Exchange / Q000009:adverse effects / Q000706:statistics & numerical data; D011379:Prognosis; D012189:Retrospective Studies; D016750:Stiff-Person Syndrome / Q000097:blood / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy*; D016896:Treatment Outcome