Systematic Review of Stiff Person Syndrome

Abstract Stiff-Person Syndrome (SPS) is a rare, autoimmune-mediated neurological condition characterized by progressive rigidity, painful spasms, and disabling functional loss. It falls within a larger stiff-person spectrum disorder (SPSD) that includes focal and paraneoplastic forms and the fulminant phenotype progressive encephalomyelitis with rigidity and myoclonus (PERM). Pathophysiology revolves around the impaired GABAergic neuronal transfer, usually by antibodies to glutamic acid decarboxylase (GAD65), but glycine receptor antibodies and amphiphysin antibodies extend the serology range. Application SPS occurs in 1-2 per million of the population, mostly in middle-aged women and often in conjunction with autoimmune diseases like type 1 diabetes and thyroid disease. Clinical vigilance, electromyography and serological diagnosis and GABAergic therapy with immunomodulation (IVIG best supported by randomized evidence) are required to support diagnosis and management. Early detection is beneficial to prognosis, especially in classic SPS, but prognosis is more promising in PERM and paraneoplastic disease. Mobility, independence, and quality of life can be optimized only with the help of multidisciplinary care. Keywords: Stiff-Person Syndrome, SPS symptoms, SPS diagnosis, SPS treatment, GAD65 antibodies, IVIG therapy, Autoimmune neurology.