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Stiff-Person Syndrome

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Neurorheumatology

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Abstract

Stiff person syndrome spectrum disorder (SPSSD) consists of a rare set of diseases characterized by severe muscle stiffness and rigidity with decline in function. Although poorly understood, an underlying autoimmune disorder interfering with the CNS inhibitory mechanism is likely the culprit. The spectrum ranges from a mild disease affecting focal muscles to a severe form that also includes the development of seizures and myoclonus.

 GAD-abs are frequently positive, especially in classic SPS. In SPS-plus/PERM, GlyR-abs are more commonly found. Patients with amphiphysin-ab should be screened for cancer. Treatment includes the GABA agonists, especially diazepam and baclofen. Patients with SPS-plus or refractory forms of the disease should be treated with immunomodulation, especially IVIG.

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Authors and Affiliations

  1. ALS and Neuromuscular Disease Center, Department of Neurology, Oregon Health & Science University, Portland, OR, USA

    Chafic Karam

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  1. Chafic Karam
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Correspondence to Chafic Karam .

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Editors and Affiliations

  1. Division of Neuroimmunology, University of Iowa, Iowa City, IA, USA

    Tracey A. Cho

  2. Department of Neurology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA

    Shamik Bhattacharyya

  3. Division of Rheumatology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA

    Simon Helfgott

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Karam, C. (2019). Stiff-Person Syndrome. In: Cho, T., Bhattacharyya, S., Helfgott, S. (eds) Neurorheumatology. Springer, Cham. https://doi.org/10.1007/978-3-030-16928-2_21

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  • DOI: https://doi.org/10.1007/978-3-030-16928-2_21

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-16927-5

  • Online ISBN: 978-3-030-16928-2

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