Emergencies in Stiff-Person Spectrum Disorders

  • Pichet Termsarasab
  • Thananan Thammongkolchai
  • Bashar Katirji


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.


Stiff-person syndrome Emergency Status spasticus Autonomic crisis Paroxysmal autonomic dysfunction Respiratory failure Baclofen withdrawal syndrome 


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© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Pichet Termsarasab
    • 1
  • Thananan Thammongkolchai
    • 1
  • Bashar Katirji
    • 2
  1. 1.Division of NeurologyFaculty of Medicine Ramathibodi Hospital, Mahidol UniversityBangkokThailand
  2. 2.Department of NeurologyUniversity Hospitals Cleveland Medical Center, Case Western Reserve UniversityClevelandUSA

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