Stiff Person Spectrum Disorders—An Update and Outlook on Clinical, Pathophysiological and Treatment Perspectives

Vlad, Benjamin; Wang, Yujie; Newsome, Scott D.; Balint, Bettina

doi:10.3390/biomedicines11092500

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Stiff Person Spectrum Disorders—An Update and Outlook on Clinical, Pathophysiological and Treatment Perspectives

¹

Department of Neurology, University Hospital Zurich, 8091 Zurich, Switzerland

²

Department of Neurology, University of Washington, Seattle, WA 98195, USA

³

Division of Neuroimmunology and Neurological Infections, Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA

⁴

Faculty of Medicine, University of Zurich, 8091 Zurich, Switzerland

^*

Author to whom correspondence should be addressed.

Biomedicines 2023, 11(9), 2500; https://doi.org/10.3390/biomedicines11092500

Submission received: 1 August 2023 / Revised: 6 September 2023 / Accepted: 7 September 2023 / Published: 10 September 2023

(This article belongs to the Special Issue Immune-Mediated Neurological Disorders)

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Abstract

Stiff person spectrum disorders (SPSD) are paradigm autoimmune movement disorders characterized by stiffness, spasms and hyperekplexia. Though rare, SPSD represent a not-to-miss diagnosis because of the associated disease burden and treatment implications. After decades as an enigmatic orphan disease, major advances in our understanding of the evolving spectrum of diseases have been made along with the identification of multiple associated autoantibodies. However, the most important recent developments relate to the recognition of a wider affection, beyond the classic core motor symptoms, and to further insights into immunomodulatory and symptomatic therapies. In this review, we summarize the recent literature on the clinical and paraclinical spectrum, current pathophysiological understanding, as well as current and possibly future therapeutic strategies.

Keywords: stiff person spectrum disorders; stiff person syndrome; glutamic acid decarboxylase; autoimmunity; autoimmune movement disorders

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MDPI and ACS Style

Vlad, B.; Wang, Y.; Newsome, S.D.; Balint, B. Stiff Person Spectrum Disorders—An Update and Outlook on Clinical, Pathophysiological and Treatment Perspectives. Biomedicines 2023, 11, 2500. https://doi.org/10.3390/biomedicines11092500

AMA Style

Vlad B, Wang Y, Newsome SD, Balint B. Stiff Person Spectrum Disorders—An Update and Outlook on Clinical, Pathophysiological and Treatment Perspectives. Biomedicines. 2023; 11(9):2500. https://doi.org/10.3390/biomedicines11092500

Chicago/Turabian Style

Vlad, Benjamin, Yujie Wang, Scott D. Newsome, and Bettina Balint. 2023. "Stiff Person Spectrum Disorders—An Update and Outlook on Clinical, Pathophysiological and Treatment Perspectives" Biomedicines 11, no. 9: 2500. https://doi.org/10.3390/biomedicines11092500

APA Style

Vlad, B., Wang, Y., Newsome, S. D., & Balint, B. (2023). Stiff Person Spectrum Disorders—An Update and Outlook on Clinical, Pathophysiological and Treatment Perspectives. Biomedicines, 11(9), 2500. https://doi.org/10.3390/biomedicines11092500

Note that from the first issue of 2016, this journal uses article numbers instead of page numbers. See further details here.

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Stiff Person Spectrum Disorders—An Update and Outlook on Clinical, Pathophysiological and Treatment Perspectives

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