PMID- 37114503
OWN - NLM
STAT- MEDLINE
DCOM- 20230615
LR  - 20230616
IS  - 1097-4598 (Electronic)
IS  - 0148-639X (Linking)
VI  - 68
IP  - 1
DP  - 2023 Jul
TI  - Myasthenic crisis.
PG  - 8-19
LID - 10.1002/mus.27832 [doi]
AB  - Myasthenic crisis (MC) is a life-threatening manifestation of myasthenia gravis 
      (MG) defined by respiratory insufficiency that requires the use of invasive or 
      non-invasive ventilation. This is often the result of respiratory muscle weakness 
      but can also be due to bulbar weakness with upper airway collapse. MC occurs in 
      approximately 15%-20% of patients with MG usually within the first 2 to 3 y of 
      the disease course. Many crises have a specific trigger with respiratory 
      infections being most common; however, no specific trigger is found in 30%-40% of 
      patients. MG patients with a history of MC, severe disease, oropharyngeal 
      weakness, muscle-specific kinase (MuSK) antibodies and thymoma appear to be at 
      higher risk. Most episodes of MC do not occur suddenly, providing a window of 
      opportunity for prevention. Immediate treatment is directed toward airway 
      management and removing any identified triggers. Plasmapheresis is preferred over 
      intravenous immune globulin as the treatments of choice for MC. The majority of 
      patients are able to be weaned from mechanical ventilation within 1 mo and the 
      outcomes of MC are generally favorable. The mortality rate in United States 
      cohorts is less than 5% and mortality in MC seems to be driven by age and other 
      medical co-morbidities. MC does not appear to affect long-term prognosis as many 
      patients are able to eventually achieve good MG control.
CI  - (c) 2023 The Authors. Muscle & Nerve published by Wiley Periodicals LLC.
FAU - Claytor, Benjamin
AU  - Claytor B
AD  - Neuromuscular Center, Department of Neurology, Neurological Institute, Cleveland 
      Clinic Foundation, Cleveland, Ohio, USA.
FAU - Cho, Sung-Min
AU  - Cho SM
AD  - Division of Neuroscience Critical Care, Departments of Neurology, Neurosurgery, 
      Anesthesiology and Critical Care Medicine, Johns Hopkins University School of 
      Medicine, Baltimore, Maryland, USA.
FAU - Li, Yuebing
AU  - Li Y
AUID- ORCID: 0000-0002-4248-8927
AD  - Neuromuscular Center, Department of Neurology, Neurological Institute, Cleveland 
      Clinic Foundation, Cleveland, Ohio, USA.
LA  - eng
GR  - K23 HL157610/HL/NHLBI NIH HHS/United States
PT  - Journal Article
PT  - Research Support, N.I.H., Extramural
PT  - Research Support, Non-U.S. Gov't
PT  - Review
DEP - 20230428
PL  - United States
TA  - Muscle Nerve
JT  - Muscle & nerve
JID - 7803146
SB  - IM
MH  - Humans
MH  - *Myasthenia Gravis/diagnosis/therapy
MH  - Muscle Weakness
MH  - Plasmapheresis
MH  - Respiration, Artificial
MH  - *Thymus Neoplasms/therapy
OTO - NOTNLM
OT  - mechanical ventilation
OT  - myasthenia gravis
OT  - myasthenic crisis
OT  - plasmapheresis
EDAT- 2023/04/28 06:41
MHDA- 2023/06/15 06:42
CRDT- 2023/04/28 05:26
PHST- 2023/04/06 00:00 [revised]
PHST- 2022/12/26 00:00 [received]
PHST- 2023/04/09 00:00 [accepted]
PHST- 2023/06/15 06:42 [medline]
PHST- 2023/04/28 06:41 [pubmed]
PHST- 2023/04/28 05:26 [entrez]
AID - 10.1002/mus.27832 [doi]
PST - ppublish
SO  - Muscle Nerve. 2023 Jul;68(1):8-19. doi: 10.1002/mus.27832. Epub 2023 Apr 28.