Treatment of Stiff-Person Spectrum Disorders

Abstract

Treatment of stiff-person spectrum disorders is usually divided into symptomatic and specific therapies. Symptomatic therapies include benzodiazepines, baclofen, tizanidine, and, less commonly, antiepileptic medications. The most commonly used medication for symptomatic treatment is diazepam. Given autoimmunity as a main pathophysiological basis, specific immunotherapies therapies include corticosteroids, corticosteroid-sparing agents, intravenous immunoglobulin, plasma exchange, and rituximab. Data from clinical trials to suggest treatment selection in clinical practice remain lacking, given the rarity of SPSD. Multiple different approaches are based on clinical experience. The targeted approach with stratification of patients by severity and clinical phenotypes is recommended. In patients with severe symptoms or aggressive phenotypes such as progressive encephalomyelitis with rigidity and myoclonus, immunotherapies should be considered early.