Amyotrophic Lateral Sclerosis (ALS)

Succinylcholine is contraindicated - Denervation up-regulates acetylcholine receptors, risking hyperkalemic cardiac arrest. Avoid it.

Profound NMB sensitivity - Use markedly reduced doses of nondepolarizing blockers with quantitative train-of-four, and confirm full, sustained reversal - recurarization after apparent recovery has been reported. Consider avoiding neuromuscular blockade altogether where the surgery allows.

Aspiration risk - Bulbar weakness and a poor cough make aspiration likely; protect the airway and plan a rapid-sequence approach that does not rely on succinylcholine.

Respiratory reserve - Reserve is minimal; dose opioids and sedatives conservatively, anticipate difficulty weaning, and plan for postoperative noninvasive or invasive ventilation and monitored recovery.

Regional and local - Regional or local techniques avoid neuromuscular blockade and the airway risk and are attractive when feasible; document the pre-existing deficit first.

A progressive degeneration of upper and lower motor neurons producing relentless weakness and wasting, bulbar dysfunction (dysarthria, dysphagia, a weak cough), and ultimately respiratory muscle failure. Sensation and, usually, cognition are spared. For the anesthesia provider the disease combines the hyperkalemia risk of denervated muscle, marked sensitivity to neuromuscular blockers and respiratory depressants, and a bulbar-driven aspiration risk with almost no respiratory reserve.