European journal of clinical investigation
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Eur. J. Clin. Invest. · Nov 2010
ReviewA critical update on the immunopathogenesis of Stiff Person Syndrome.
Stiff Person Syndrome (SPS) is a relatively rare but often overlooked autoimmune neurological disorder that targets antigens within the brain's inhibitory pathways resulting in incapacitating stiffness and spasms that impact on the patients' quality of life. Although a number of immunomodulating therapies significantly improve the patients' symptoms, the exact pathogenic mechanisms remain unclear. ⋯ This review provides a critical assessment on the immunobiology of SPS, describes the identification of anti-GABARAP antibodies as a new antigenic target in the GABAergic synapse and identifies the areas for future research.