Myasthenia gravis - NYSORA

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Myasthenia gravis

Learning objectives

  • Know the challenges in the perioperative management of these patients
  • Recognise a myasthenic or cholinergic crisis

Definition and mechanisms

  • Myasthenia Gravis (MG) is an autoimmune disease of the neuromuscular junction at the postsynaptic membrane
  • Autoantibodies to AchR disrupt its function by receptor blockade and conformational change leading to increased degradation which results in decreased motor end-plate potential amplitude and failure in the initiation of muscle contraction
  • It is the most common disorder of the neuromuscular junction

Signs and symptoms 

  • Muscle weakness and fatigue of:
Extraocular muscle groupPtosis
Diplopia
Bulbar muscle groupDysarthria
Dysphagia
Dysphonia
Axial-limb muscle groupSymmetrical, more often in proximal muscles
Respiratory musclesDyspnea
  • Symptoms vary over the day and from day to day, often with normal or near-normal muscle strength in the morning
  • Clinical presentation varies from mild and localized to severe, generalized weakness of multiple muscle groups

Myasthenic crisis is a life-threatening condition in which severe respiratory muscle insufficiency leads to respiratory failure

  • Precipitants
    • Infection 
    • Surgery 
    • Residual neuromuscular block 
    • Pain 
    • Hypo- and hyperthermia
    • Reduction or withdrawal of treatment 
    • Pregnancy
    • Stress
    • Sleep deprivation 
    • Agents/drugs:
Antibiotics Aminoglycoside
Fluoroquinolones
Tetracycline
Macrolides
Sulfonamides
Penicillin
Vancomycin
Clindamycin
Ketolides
Non-depolarizing neuromuscular-blocking agentsPancuronium
Vecuronium
Atracurium
Cardiovascular agentsBeta-blockers
Calcium channel blockers
Procainamide
Quinidine
Beryllium
Anesthetic agentsLocal: procaine, lidocaine, bupivacaine
Inhalation: halothane, isoflurane
Neuromuscular blockers
AnticonvulsantsCarbamazepine
Gabapentine
Phenobarbital
Phenytoin
Ethosuximide
Other agentsBotulinum toxin
Chloroquine
Hydroxychloroquine
Magnesium
Penicillamine
Quinine
Iodinated contrast agents
Cisplatinum
Riluzole
Interferon-alfa
Interleukin-2

Treatment

  • Respiratory support 
  • Urgent neurological input 
  • High-dose steroids/intravenous immunoglobulins  

Anesthetic management 

Myasthenia gravis, myasthenic crises, anticholinesterase agents, immunoglobuline, glucocorticoids, plasma exchange

Myasthenia gravis, bulbar weakness, muscarinic activity, NMBDs, rucoronium-suggammadex

Suggested reading

  • P. Daum, J. Smelt and I.R. Ibrahim. Perioperative management of myasthenia gravis. 2021, BJA Education, 21(11): 414-429. 
  • Dhallu MS, Baiomi A, Biyyam M, Chilimuri S. Perioperative Management of Neurological Conditions. Health Serv Insights. 2017;10:1178632917711942.
  • Dillon FX. Anesthesia issues in the perioperative management of myasthenia gravis. Semin Neurol. 2004;24(1):83-94.

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