Sensitivity of patients with neuromuscular disease to neuromuscular blocking agents

Disorder type	Response to succinylcholine	Response to nondepolarizing NMBA
Myasthenic syndromes
Myasthenia gravis	Resistant	Sensitive
Lambert-Eaton myasthenic syndrome	Sensitive	Sensitive
Congenital myasthenic syndromes	Variable, not recommended due to potential hyperkalemic response	Sensitive
Malignant hyperthermia, central core disease, King-Denborough syndrome	Contraindicated	Normal
Demyelinating diseases
Multiple sclerosis	Avoid use	Sensitive
Motor neuron diseases	Avoid use	Sensitive
Guillain-Barré syndrome	Avoid use	Sensitive
Charcot-Marie-Tooth disease (CMTD)	Avoid use	Normal
Primary muscle diseases
Muscular dystrophies	Contraindicated	Sensitive
Duchenne muscular dystrophy	Contraindicated	Sensitive
Becker muscular dystrophy	Contraindicated	Sensitive
Limb-girdle dystrophy	Contraindicated	Sensitive
Congenital muscular dystrophy	Contraindicated	Sensitive
Facioscapulohumeral muscular dystrophy	Contraindicated	Sensitive
Myotonic dystrophy	Contraindicated	Sensitive*
Mitochondrial myopathies	Sensitive	Sensitive
Motor neuron diseases
Amyotrophic lateral sclerosis (Lou Gehrig's disease)	Avoid use	Sensitive
Channelopathies
Acquired neuromyotonia	Avoid use	Normal*
Myotonia congenita	Avoid use
Paramyotonia congenita	Avoid use
Potassium aggravated myotonia	Avoid use
Hyperkalemic periodic paralysis	Avoid use
Hypokalemic periodic paralysis	Avoid use
Spinal cord injury, stroke, immobility, ICU patients	Avoid use	Resistant
Metabolic myopathies
Storage disorders (lipid, glycogen)^¶	Avoid use	Variable – avoid use if possible

NMBA: neuromuscular blocking agent; ICU: intensive care unit.
* Anticholinesterase drugs should be avoided because they may provoke a myotonic reaction.
¶ Carnitine palmitoyltransferase II (CPT II) deficiency; myophosphorylase deficiency (McArdle disease).

Adapted from: Brull SJ. Neuromuscular blocking agents. In: Clinical Anesthesia, 8th ed, Barash PG, Cullen BF, Stoelting RK, et al (Eds), Wolters Kluwer 2017. p.527.

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Sensitivity of patients with neuromuscular disease to neuromuscular blocking agents