title: Anesthetic management of the patient with amyotrophic lateral sclerosis.
abstract: Amyotrophic lateral sclerosis (ALS), with an incidence of 1.5-2.5 for 100 000 per year, is a rare but rapid progression neuromuscular degeneration disorder that poses unique perioperatively challenges to clinical anesthesiologists. The progressive degeneration of motor neurons causes a constellation of symptoms, including muscular weakness, atrophy, fasciculations, spasticity, and hyperreflexia. Therapeutic and experimental treatments, including riluzole, beta lactams, methylcobalamin, dexpramipexole, antiepileptics, antioxidant agents, neutrophin, antiinflammatory agents, and antiapoptosis drugs, are described. Newer therapies, such as neural stem cells and diaphragmatic pacing, are presented. Because of the inherent muscle weakness and associated respiratory insufficiency, certain precautions must be utilized during anesthetic care of ALS patients. In particular, certain neuromuscular agents are contraindicated and anesthetics that leave the body more rapidly present logical and attractive options in this population. A solid understanding of the disease process, therapeutic interventions, and anesthesia considerations are all paramount for the successful management of a patient with ALS in the perioperative setting.
authors.affiliation: Department of Anesthesiology, Louisiana State University Health Science Center, New Orleans, LA, USA.
authors.forename: Amit
authors.initials: A
authors.lastname: Prabhakar
authors.forename: Christopher Paul
authors.initials: CP
authors.lastname: Owen
authors.forename: Alan David
authors.initials: AD
authors.lastname: Kaye
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000188:drug therapy*; D000777:Anesthetics / Q000008:administration & dosage*; D006801:Humans; D009046:Motor Neurons / Q000187:drug effects
chemical_list: D000777:Anesthetics