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Stiff-Person Syndrome Spectrum Disorders

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Neuroimmune Diseases

Part of the book series: Contemporary Clinical Neuroscience ((CCNE))

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Abstract

Stiff-person syndrome was first described in 1956; its further characterization as an autoimmune neurological disorder occurred more than 30 years later with the discovery of glutamic acid decarboxylase (GAD) antibodies (Abs), frequently coexisting in these patients. In the following years, clinical variants of SPS have been characterized, and a paraneoplastic presentation was also recognized, the latter mainly associated with amphiphysin antibodies. Although the presence of GAD-Abs has led to theorize that these antibodies cause disinhibition of the central nervous system through decreased production of the inhibitory neurotransmitter (GABA), the pathogenic role of GAD-Abs has not been demonstrated, although the evidence suggests that antibodies directed against amphiphysin and glycine receptor α1 are likely pathogenic. The treatment aims to attenuate the immunological response through immunotherapy, control the symptoms, mainly with GABAergic drugs, and remove an underlying tumor, if present. The course is usually chronic and the prognosis is frequently poor.

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Authors and Affiliations

  1. Department of Internal Medicine, University of Guanajuato, Guanajuato, Mexico

    José Fidel Baizabal-Carvallo

  2. National Polytechnic Institute, Mexico City, Mexico

    Marlene Alonso-Juarez

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  1. José Fidel Baizabal-Carvallo
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  2. Marlene Alonso-Juarez
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  1. Medical Education Promotion Center, Tokyo Medical University, Tokyo, Japan

    Hiroshi Mitoma

  2. Department of Neurology, CHU-Charleroi, Charleroi, Belgium, Department of Neurosciences, University of Mons, Mons, Belgium

    Mario Manto

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Baizabal-Carvallo, J.F., Alonso-Juarez, M. (2019). Stiff-Person Syndrome Spectrum Disorders. In: Mitoma, H., Manto, M. (eds) Neuroimmune Diseases. Contemporary Clinical Neuroscience. Springer, Cham. https://doi.org/10.1007/978-3-030-19515-1_20

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