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Emergencies in Stiff-Person Spectrum Disorders

  • Pichet Termsarasab
  • Thananan Thammongkolchai
  • Bashar Katirji
Chapter
  • 110 Downloads

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.

Keywords

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

References

  1. 1.
    Espay AJ, Chen R. Rigidity and spasms from autoimmune encephalomyelopathies: stiff-person syndrome. Muscle Nerve. 2006;34(6):677–90.  https://doi.org/10.1002/mus.20653.CrossRefPubMedPubMedCentralGoogle Scholar
  2. 2.
    Blum P, Jankovic J. Stiff-person syndrome: an autoimmune disease. Mov Disord. 1991;6(1):12–20.  https://doi.org/10.1002/mds.870060104.CrossRefPubMedPubMedCentralGoogle Scholar
  3. 3.
    Gros AJ Jr, Thomas LC, McKinley KL, Van Gerpen JA. Prevention of an acute severe exacerbation of stiff-person syndrome during surgery. Anesthesiology. 2006;104(4):885–6.  https://doi.org/10.1097/00000542-200604000-00035.CrossRefPubMedGoogle Scholar
  4. 4.
    Maramattom BV. Status spasticus and psoas muscle edema due to anti-GAD antibody associated stiff-man syndrome. Indian J Crit Care Med. 2015;19(8):493–5.  https://doi.org/10.4103/0972-5229.162474.CrossRefPubMedPubMedCentralGoogle Scholar
  5. 5.
    Hattan E, Angle MR, Chalk C. Unexpected benefit of propofol in stiff-person syndrome. Neurology. 2008;70(18):1641–2.  https://doi.org/10.1212/01.wnl.0000284606.00074.f1.CrossRefPubMedGoogle Scholar
  6. 6.
    Maramattom BV. Intrathecal baclofen pump implantation in a case of stiff person syndrome. Neurol India. 2010;58(1):115–7.  https://doi.org/10.4103/0028-3886.60420.CrossRefPubMedGoogle Scholar
  7. 7.
    Ughratdar I, Sivakumar G, Basu S. Spinal cord stimulation to abort painful spasms of atypical stiff limb syndrome. Stereotact Funct Neurosurg. 2010;88(3):183–6.  https://doi.org/10.1159/000313871.CrossRefPubMedGoogle Scholar
  8. 8.
    Mitsumoto H, Schwartzman MJ, Estes ML, Chou SM, La Franchise EF, De Camilli P, et al. Sudden death and paroxysmal autonomic dysfunction in stiff-man syndrome. J Neurol. 1991;238(2):91–6.  https://doi.org/10.1007/bf00315688.CrossRefPubMedGoogle Scholar
  9. 9.
    Goetz CG, Klawans HL. On the mechanism of sudden death in Moersch-Woltman syndrome. Neurology. 1983;33(7):930–2.  https://doi.org/10.1212/wnl.33.7.930.CrossRefPubMedGoogle Scholar
  10. 10.
    Maccario M, Baugh JR, Mena H. Sudden death in Moersch-Woltman. Neurology. 1984;34(3):407.  https://doi.org/10.1212/wnl.34.3.407.CrossRefPubMedPubMedCentralGoogle Scholar
  11. 11.
    Guilleminault C, Sigwald J, Castaigne P. Sleep studies and therapeutic trial with L-dopa in a case of Stiffman syndrome. Eur Neurol. 1973;10(2):89–96.  https://doi.org/10.1159/000114266.CrossRefPubMedGoogle Scholar
  12. 12.
    Jachiet V, Laine L, Gendre T, Henry C, Da Silva D, de Montmollin E. Acute respiratory failure in a patient with stiff-person syndrome. Neurocrit Care. 2016;25(3):455–7.  https://doi.org/10.1007/s12028-016-0296-0.CrossRefPubMedGoogle Scholar
  13. 13.
    Termsarasab P, Frucht SJ. Dystonic storm: a practical clinical and video review. J Clin Mov Disord. 2017;4:10.  https://doi.org/10.1186/s40734-017-0057-z.CrossRefPubMedPubMedCentralGoogle Scholar
  14. 14.
    Termsarasab P, Thammongkolchai T, Frucht SJ. Medical treatment of dystonia. J Clin Mov Disord. 2016;3:19.  https://doi.org/10.1186/s40734-016-0047-6.CrossRefPubMedPubMedCentralGoogle Scholar
  15. 15.
    Watve SV, Sivan M, Raza WA, Jamil FF. Management of acute overdose or withdrawal state in intrathecal baclofen therapy. Spinal Cord. 2012;50(2):107–11.  https://doi.org/10.1038/sc.2011.112.CrossRefPubMedGoogle Scholar
  16. 16.
    Bardutzky J, Tronnier V, Schwab S, Meinck HM. Intrathecal baclofen for stiff-person syndrome: life-threatening intermittent catheter leakage. Neurology. 2003;60(12):1976–8.  https://doi.org/10.1212/wnl.60.12.1976.CrossRefGoogle Scholar
  17. 17.
    Ross JC, Cook AM, Stewart GL, Fahy BG. Acute intrathecal baclofen withdrawal: a brief review of treatment options. Neurocrit Care. 2011;14(1):103–8.  https://doi.org/10.1007/s12028-010-9422-6.CrossRefPubMedGoogle Scholar
  18. 18.
    Johnson JO, Miller KA. Anesthetic implications in stiff-person syndrome. Anesth Analg. 1995;80(3):612–3.  https://doi.org/10.1097/00000539-199503000-00032.CrossRefPubMedGoogle Scholar
  19. 19.
    Bouw J, Leendertse K, Tijssen MA, Dzoljic M. Stiff person syndrome and anesthesia: case report. Anesth Analg. 2003;97(2):486–7,. table of contents.  https://doi.org/10.1213/01.ane.0000072701.97699.79.CrossRefPubMedGoogle Scholar
  20. 20.
    Ledowski T, Russell P. Anaesthesia for stiff person syndrome: successful use of total intravenous anaesthesia. Anaesthesia. 2006;61(7):725.  https://doi.org/10.1111/j.1365-2044.2006.04699.x.CrossRefPubMedGoogle Scholar
  21. 21.
    Elkassabany N, Tetzlaff JE, Argalious M. Anesthetic management of a patient with stiff person syndrome. J Clin Anesth. 2006;18(3):218–20.  https://doi.org/10.1016/j.jclinane.2005.06.009.CrossRefPubMedGoogle Scholar
  22. 22.
    Shanthanna H. Stiff man syndrome and anaesthetic considerations: successful management using combined spinal epidural anaesthesia. J Anaesthesiol Clin Pharmacol. 2010;26(4):547–8.PubMedPubMedCentralGoogle Scholar
  23. 23.
    Aya D, Fukuda T, Ohkoshi N, Furukawa H, Toyooka H. Regional anesthesia for a patient with stiff-person syndrome. Masui. 2004;53(1):66–8.PubMedGoogle Scholar
  24. 24.
    Meinck HM, Thompson PD. Stiff man syndrome and related conditions. Mov Disord. 2002;17(5):853–66. https://doi.org/10.1002/mds.10279.

Copyright information

© 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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