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Abstract

Background: Stiff Person Syndrome (SPS) is a rare, debilitating autoimmune neurological disorder characterized by progressive muscle rigidity, painful spasms, and gait disturbances. It is strongly associated with autoantibodies against glutamic acid decarboxylase (GAD), leading to impaired GABAergic neurotransmission. SPS exists on a spectrum, with both classical and paraneoplastic variants, the latter often linked to malignancies such as breast cancer.

Methods: This review synthesizes current literature on the pathophysiology, clinical presentation, diagnostic criteria, and treatment modalities of SPS. We examine emerging therapies, including immunomodulatory approaches and antibody-targeted interventions, to address the heterogeneity of disease progression and treatment response.

Results: SPS remains a diagnostic challenge due to its overlap with other neuromuscular and psychiatric conditions. While anti-GAD65 antibodies serve as a key biomarker, seronegative cases complicate definitive diagnosis. Benzodiazepines and baclofen provide symptomatic relief, whereas intravenous immunoglobulin (IVIG) and plasmapheresis offer immunotherapeutic benefits. Novel treatments, including chimeric antigen receptor (CAR) T-cell therapy and extracorporeal photopheresis, present promising avenues for long-term disease management.

Conclusion: Advances in autoimmune neurology continue to refine our understanding of SPS, yet significant gaps persist in early diagnosis and individualized treatment strategies. A multidisciplinary approach integrating neurology, immunology, and psychiatry is imperative to improve clinical outcomes. Future research should focus on targeted immunotherapies and biomarker discovery to enhance prognosis and quality of life for affected individuals.

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