Therapeutic Plasma Exchange in the Management of Stiff Person Syndrome Spectrum Disorders: A Case Series and Review of the Literature

Objective To describe the safety, tolerability, and response to TPE in patients with SPSD. Background Stiff person syndrome spectrum disorders (SPSD) are a rare group of disabling neuroimmunological disorders. SPSD often require immune therapies especially setting inadequate symptomatic treatments. The safety efficacy therapeutic plasma exchange (TPE) is unclear. Design/Methods A retrospective review medical records for seen at Johns Hopkins Hospital was performed. Patient characteristics, exam findings, diagnostic studies, treatment TPE-related complications were recorded. We also conducted linear regression models assess possible predictive factors response. Literature conducted. Results Thirty-six treated TPE; mean age 48 years, 81% female, average anti-GAD65 antibody titer 42352 U/mL (range, 0-309,902). Twenty-two had classic SPS, 10 SPS-plus, 3 other phenotypes. Thirty-three acute exacerbations, on maintenance TPE. There 4 (11.1%) (catheter infection, catheter thrombosis, hemorrhage), but no deaths or anaphylaxis. Twenty (55.6%) reported improvement symptoms after TPE, 13 change, worsening symptoms. Of 36 total who received 21 an exacerbation their condition, 12 requiring fewer anti-spasmodic medications months treatment. positive identified 42 more patients; 69% these temporary condition. Conclusions tolerability show that uncommon manageable. Additionally, many derived Further studies could help inform clinicians when use