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Emergencies in Stiff-Person Spectrum Disorders

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Stiff-Person Syndrome and Related Disorders

Abstract

Emergencies in stiff-person spectrum disorders (SPSD) refer to situations related to SPSD that, if not identified and treated promptly, may result in significant morbidity and mortality. These include acute severe exacerbation of spasms and rigidity, status spasticus, paroxysmal autonomic dysfunction, autonomic crisis, acute respiratory failure, and treatment-related emergencies, especially baclofen withdrawal syndrome. General management is similar among these conditions and includes initial stabilization, intensive care unit transfer, intravenous fluid hydration, sedation, and fever reduction, among others. Specific treatment, for example, immunotherapies or resuming baclofen, is then considered. There are some anesthetic considerations for SPSD patients undergoing surgeries.

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Termsarasab, P., Thammongkolchai, T., Katirji, B. (2020). Emergencies in Stiff-Person Spectrum Disorders. In: Stiff-Person Syndrome and Related Disorders. Springer, Cham. https://doi.org/10.1007/978-3-030-43059-7_13

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  • DOI: https://doi.org/10.1007/978-3-030-43059-7_13

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-43058-0

  • Online ISBN: 978-3-030-43059-7

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