Amyotrophic lateral sclerosis and anesthesia: a case series and review of the literature
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that leads to death due to respiratory failure. This report describes the perioperative characteristics of ALS patients who underwent procedures with anesthesia at our institution.
We reviewed perioperative records of ALS patients who underwent procedures with anesthesia from January 1, 2014, through December 31, 2015.
Seventy-eight patients underwent 89 procedures (71 procedures with monitored anesthesia care and 18 with general anesthesia), including 45 gastrostomy tube placements and 18 bone marrow biopsies. Three patients had prolonged duration of postoperative intubation related to preexisting respiratory muscle weakness, and one patient with bilateral pneumothorax required tracheal reintubation for respiratory distress. Four patients had prolonged duration of hospitalization. Three patients were hospitalized for ALS-related complications, and one patient was hospitalized for respiratory distress when pneumoperitoneum developed after gastrostomy tube placement. Three of these patients died of complications attributable to ALS within 30 days of the procedure. Twenty-nine (32.6%) procedures required minimal sedation (e.g., bone marrow biopsy, cataract surgery) and were performed on an ambulatory basis.
When caring for patients with ALS, the perioperative team must be prepared to treat potentially complex medical conditions that may not be directly related to the procedure and anesthetic management. However, minor procedures performed with minimal sedation may be safely performed on an ambulatory basis.
KeywordsAmyotrophic lateral sclerosis Anesthesia Outcomes
Amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis functional rating scale-revised
Monitored anesthesia care
Percutaneous endogastric gastrostomy
Compliance with ethical standards
Conflict of interest
TNW currently serves as a consultant to Medtronic in the role as chairman of the Clinical Endpoint Committee for the Prodigy Trial; has received research support from Respiratory Motion (study equipment) and unrestricted investigator-initiated grants from Merck (active) and Baxter (completed).
- 7.Byrne S, Heverin M, Elamin M, Bede P, Lynch C, Kenna K, et al. Aggregation of neurologic and neuropsychiatric disease in amyotrophic lateral sclerosis kindreds: a population-based case-control cohort study of familial and sporadic amyotrophic lateral sclerosis. Ann Neurol. 2013;74(5):699–708.CrossRefGoogle Scholar
- 8.Marin B, Boumediene F, Logroscino G, Couratier P, Babron MC, Leutenegger AL, et al. Variation in worldwide incidence of amyotrophic lateral sclerosis: a meta-analysis. Int J Epidemiol. 2017;46(1):57–74.Google Scholar
- 9.Miller RG, Mitchell JD, Moore DH. Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND). Cochrane Database Syst Rev. 2012;14(3):CD001447.Google Scholar
- 11.Petrou P, Gothelf Y, Argov Z, Gotkine M, Levy YS, Kassis I, et al. Safety and clinical effects of mesenchymal stem cells secreting neurotrophic factor transplantation in patients with amyotrophic lateral sclerosis: results of phase 1/2 and 2a clinical trials. JAMA Neurol. 2016;73(3):337–44.CrossRefGoogle Scholar
- 13.Kirstein MM, Korner S, Schneider A, Manns MP, Petri S, Voigtlander T. Percutaneous endoscopic gastrostomy with and without jejunal extension in patients with amyotrophic lateral sclerosis. Eur J Gastroenterol Hepatol. 2018;30(3):257–62.Google Scholar
- 17.Liu S, Liu Z, Zhang J, Li Z, Hu Z, Yang W, et al. Anesthetic strategy for percutaneous endoscopic gastrostomy in amyotrophic lateral sclerosis patients. Int J Clin Exp Med. 2017;10(2):3725–30.Google Scholar
- 18.Kak M, Issa NP, Roos RP, Sweitzer BJ, Gottlieb O, Guralnick A, et al. Gastrostomy tube placement is safe in advanced amyotrophic lateral sclerosis. Neurol Res. 2017;39(1):16–22.Google Scholar
- 19.Monsma M, Osseyran F, Monzó Z, Silla I, Doménech R, Argente P. Soporte respiratorio durante procedimientos endoscópicos bajo sedación. Experiencia en 13 pacientes afectados de esclerosis lateral amiotrófica. Rev Argent Anestesiol. 2016;74(3):98–103. https://doi.org/10.1016/j.raa.2016.11.002 CrossRefGoogle Scholar
- 24.Thourot M, Cornillon B, Robert R, Royer D, Patrigeon RG. Hyperkaliémie menaçante suite à une injection de succinylcholine orientant le diagnostic vers une sclérose latérale amyotrophique. Anesth Reanim Anesth Reanim. 2017;3(2):193–7.***Google Scholar
- 26.Chang TB, Huh J. Delayed neuromuscular recovery after use of sugammadex in a patient with amyotrophic lateral sclerosis: a case report. Anaesth Pain Intensive Care Anaesth Pain Intensive Care. 2017;21(4):475–8.Google Scholar
- 39.Gu J, Lin X. Anesthesia and postoperative analgesia for a patient with amyotrophic lateral sclerosis. Miner Anestesiol. 2017;83(11):1216–7.Google Scholar
- 40.Kalava A, Clendenen S, McKinney JM, Bojaxhi E, Greengrass RA. Bilateral thoracic paravertebral nerve blocks for placement of percutaneous radiologic gastrostomy in patients with amyotrophic lateral sclerosis: a case series. Rom J Anaesth Intensive Care. 2016;23(2):149–53.Google Scholar
- 41.Agnoletti V, Corso R, Cattano D, Novi A, Piraccini E, Folli S, et al. Thoracic paravertebral block for breast surgery in a patient with amyotrophic lateral sclerosis. Miner Anestesiol. 2013;79(7):822–3.Google Scholar
- 45.Chang YJ, Jung WS, Son WR, Jo YY. Discordance between train-of-four response and clinical symptoms in a patient with amyotrophic lateral sclerosis. Acta Med Okayama. 2014;68(2):125–7.Google Scholar