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Anesthetic management of the patient with amyotrophic lateral sclerosis

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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.

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The authors have no relationships with pharmaceutical companies or products to disclose, nor do they discuss off-label or investigative products in this lesson.

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Correspondence to Alan David Kaye.

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Prabhakar, A., Owen, C.P. & Kaye, A.D. Anesthetic management of the patient with amyotrophic lateral sclerosis. J Anesth 27, 909–918 (2013). https://doi.org/10.1007/s00540-013-1644-2

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