PDF herunterladen
CC BY-NC-ND 4.0 · Neurology International Open 2018; 02(01): E10-E15
DOI: 10.1055/s-0043-118441
Review
Eigentümer und Copyright ©Georg Thieme Verlag KG 2018

Myasthenia Gravis – Exacerbation and Crisis

Michael Schroeter
1   Department of Neurology, University Hospital, University of Cologne, Cologne, Germany
,
Günther Thayssen
2   Department of Neurology, University Hospital Hamburg-Eppendorf, Hamburg, Germany
,
Julia Kaiser
3   Department of Neurology, LVR Clinic Bonn, Germany
› Institutsangaben
Weitere Informationen

Publikationsverlauf

Publikationsdatum:
08. Januar 2018 (online)

   Lizenzen und Reprints

Abstract

Myasthenic exacerbation and crisis are most critical incidences in myasthenia gravis. Even nowadays myasthenic crisis is a life-threatening condition, with a lethality of 2–3%. We review means of avoiding myasthenic exacerbation and crisis, elaborate on red flags and how to establish highly-active therapy in a timely manner. This includes the reasonable use of cholinesterase inhibitors, immunoadsorption or plasma exchange, as well as immunoglobulins and steroids. Immunosuppressive agents and monoclonal antibody therapy add to the therapeutic options.

Intensive care of myasthenic patients includes the management of dysphagia and delirium. Importantly, the perioperative management of patients undergoing thymectomy and weaning are specific challenges in the treatment of myasthenic patients in the ICU.

Establishing timely consequent immunosuppression and treatment of myasthenic patients in specialized outpatient centres help to avoid repetitive exacerbations and crises.

Key words

myasthenia gravis therapy - review - myasthenia gravis crisis - intensive care unit
 

  • References

  • 1 Meriggioli MN, Sanders DB. Autoimmune myasthenia gravis: Emerging clinical and biological heterogeneity. Lancet Neurol 2009; 8: 475-490
    CrossrefPubMedGoogle Scholar
  • 2 Lacomis D. Myasthenic crisis. Neurocrit Care 2005; 3: 189-194
    CrossrefPubMedGoogle Scholar
  • 3 Alshekhlee A, Miles JD, Katirji B. et al. Incidence and mortality rates of myasthenia gravis and myasthenic crisis in US hospitals. Neurology 2009; 72: 1548-1554
    CrossrefPubMedGoogle Scholar
  • 4 Thomas CE, Mayer SA, Gungor Y. et al. Myasthenic crisis: clinical features, mortality, complications, and risk factors for prolonged intubation. Neurology 1997; 48: 1253-1260
    CrossrefPubMedGoogle Scholar
  • 5 Bungard S, Rohn H. Döbler: Zertifizierung von Myasthenie-Zentren - Entwicklung und Umsetzung eines Zertifizierungsverfahrens für Patientenorganisationen. Z Eval Fortb Qual Gesundhwes 2011; 105: 49-53
    PubMedGoogle Scholar
  • 6 Zinman L, Ng E, Bril V. IV immunoglobulin in patients with myasthenia gravis: A randomized controlled trial. Neurology 2007; 68: 837-841
    CrossrefPubMedGoogle Scholar
  • 7 Alabdali M, Barnett C, Katzberg H. et al. Intravenous immunoglobulin as treatment for myasthenia gravis: Current evidence and outcomes. Expert Rev Clin Immunol 2014; 10: 1659-1665
    CrossrefPubMedGoogle Scholar
  • 8 Anonym Bundesausschuss AG. Verordnungsfähigkeit von zugelassenen Arzneimitteln in nicht zugelassenen Anwendungsgebieten (off-label use). BAnz AT 10.07.2014 B4
    PubMed
  • 9 Bedlack RS, Simel DL, Bosworth H. et al. Quantitative myasthenia gravis score: assessment of responsiveness and longitudinal validity. Neurology 2005; 64: 1968-1970
    CrossrefPubMedGoogle Scholar
  • 10 Jamal BT, Herb K. Perioperative management of patients with myasthenia gravis: Prevention, recognition, and treatment. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2009; 107: 612-615
    CrossrefPubMedGoogle Scholar
  • 11 Watanabe A, Watanabe T, Obama T. et al. Prognostic factors for myasthenic crisis after transsternal thymectomy in patients with myasthenia gravis. J Thorac Cardiovasc Surg 2004; 127: 868-876
    CrossrefPubMedGoogle Scholar
  • 12 Gritti P, Sgarzi M, Carrara B. et al. A standardized protocol for the perioperative management of myasthenia gravis patients. Experience with 110 patients. Acta Anaesthesiol Scand 2012; 56: 66-75
    CrossrefPubMedGoogle Scholar
  • 13 Wiendl H. (federführend). Diagnostik und Therapie der Myasthenia gravis und des Lambert-Eaton-Syndroms. https://www.dgn.org/leitlinien/3005-ll-68-ll-diagnostik-und-therapie-der-myastheniagravis-und-des-lambert-eaton-syndroms. Last access 22.07.2017
    PubMed
  • 14 Loschiavo C, Grecò M, Polo A et al. The use of therapeutic apheresis in neurological diseases and comparison between plasma exchange and immunoadsorption. G Ital Nefrol 2015; 32: pii: gin/32.1.10
    PubMed
  • 15 Stangel M, Baumann U, Borte M. et al. Treatment of neurological autoimmune diseases with immunoglobulins: First insights from the prospective SIGNS registry. J Clin Immunol 2013; 33 (Suppl. 01) S67-S71
    CrossrefPubMedGoogle Scholar
  • 16 Iorio R, Damato V, Alboini PE. et al. Efficacy and safety of rituximab for myasthenia gravis: A systematic review and meta-analysis. J Neurol 2015; 262: 1115-1119
    CrossrefPubMedGoogle Scholar
  • 17 Melzer N, Ruck T, Fuhr P. et al. Clinical features, pathogenesis, and treatment of myasthenia gravis: A supplement to the Guidelines of the German Neurological Society. J Neurol 2016; 263: 1473-1494
    CrossrefPubMedGoogle Scholar
  • 18 Rabinstein AA. Noninvasive ventilation for neuromuscular respiratory failure: when to use and when to avoid. Curr Opin Crit Care 2016; 22: 94-91
    PubMedGoogle Scholar
  • 19 Godoy DA, Mello LJ, Masotti L. et al. The myasthenic patient in crisis: An update of the management in Neurointensive Care Unit. Arq Neuropsiquiatr 2013; 71: 627-639
    CrossrefPubMedGoogle Scholar
  • 20 Higo R, Nito T, Tayama N. Videofluoroscopic assessment of swallowing function in patients with myasthenia gravis. J Neurol Sci 2005; 231: 45-48
    CrossrefPubMedGoogle Scholar
  • 21 Dziewas R, Glahn J, Helfer C. et al. Flexible endoscopic evaluation of swallowing (FEES) for neurogenic dysphagia: training curriculum of the German Society of Neurology and the German stroke society. BMC Med Educ 2016; 16: 70
    PubMedGoogle Scholar