Application of the Technique of Extracorporeal Membrane Oxygenation in a Patient With Respiratory Distress Syndrome Associated With Myasthenia Gravis

Myasthenia gravis is an autoimmune neuromuscular disease characterized by pathologically rapid fatigue of striated muscles [1]. The main symptom of myasthenia gravis is the presence of pathological muscle weakness with involvement of the ocular, bulbar and skeletal muscles in the pathological process. The provoking factors for the development of myasthenia gravis can be infectious diseases, surgery, drugs [2, 3]. The main danger is represented by myasthenic and cholinergic crises, which are characterized by a severe course and high mortality; therefore, the problems of treating myasthenia gravis are still of high medical and social significance. The prevalence of myasthenia gravis is 17.5–20.3 per 100 thousand population, and the number of patients is increasing by 5–10% annually [4, 5]. In recent years, there has been a steady increase in morbidity with an increase in age over 50 years [6, 7]. Myasthenia gravis is a serious disease with a high mortality rate of up to 30–40% [3]. There are difficulties in the early differential diagnosis of muscle weakness in patients with respiratory failure between myasthenia gravis, myasthenic syndrome and critical illness polyneuropathy. These difficulties and insufficient awareness of patients and doctors of various specialties about myasthenia gravis can lead to the choice of the wrong treatment tactics and the development of myasthenic crisis, which is manifested by respiratory failure, requiring respiratory support. The progression of respiratory failure against the background of myasthenic crisis may require the use of extracorporeal membrane oxygenation (ECMO).

It is necessary to expand the differential diagnosis of muscle weakness in a patient during the period of resolution of respiratory failure, allowing to move away from compulsory respiratory support, termination of ECMO. 

1. Sanadze AG. Miasteniya i miastenicheskie sindromy. Moscow: Littera Publ.; 2012. (in Russ.)

2. Lapshina OV, Sedyshev DV, Belyakov KM, Antipenko EA, Gustov AV. Peculiarities of Myasthenic Crisis in the Late Onset of the Disease. Modern problems of science and education. 2017;(4):9. (in Russ.)

3. Gusev EI, Konovalov AN, Skvortsova VI, Gekht AB (eds.) Nevrologiya. 2nd ed. Moscow: GEOTAR–Media Publ.; 2018. (in Russ.)

4. Ponomareva EN. Miasteniya: klinika, patogenez, differentsial’naya diagnostika, taktika vedeniya. Minsk: MET Publ.; 2002. (in Russ.)

5. Carr AS, Cardwell CR, McCarron PO, Mc Conville J. A systematic review of population based epidemiological studies in Myasthenia Gravis. BMC Neurol. 2010;10:46. PMID: 20565885 https://doi.org/10.1186/1471-2377-10-46

6. Heldal AT, Owe JF, Gilhus NE, Romi F. Seropositive myasthenia gravis a nationwide epidemiologic study. Neurology. 2009;73(2):150–151. PMID: 19597135 https://doi.org/10.1212/WNL.0b013e3181ad53c2

7. Gattellari M, Goumas C, Worthington JM. A national epidemiological study of Myasthenia Gravis in Australia. Eur J Neurol. 2012;19(11):1413– 1420. PMID: 22469211 https://doi.org/10.1111/j.1468-1331.2012.03698.x

8. Gel’fand BR, Kassil’ VL. (eds.) Ostryy respiratornyy distress-sindrom. Moscow: Littera Publ.; 2007. (in Russ.)

9. Extracorporeal Life Support Organization (ELSO) Available at: https:// xn--j1aeg1d.xn--p1ai/rekomendatsii/ekmo-obshchie [Accessed 17 May 2021] (in Russ.)

10. Mehndiratta M, Pandey S, Kuntzer T. Acetylcholinesterase inhibitor treatment for myasthenia gravis. Cochrane Database Syst Rev. 2011;(2): CD006986. PMID: 21328290 https://doi.org/10.1002/14651858. CD006986.pub2

11. Skeie GO, Apostolski S, Evoli A, Gilhus NE, Illa I, Harms L, et al. Guidelines for treatment of autoimmune neuromuscular transmission disorders. Eur J Neurol. 2010;17:893–902. PMID: 20402760 https://doi.org/10.1111/j.1468-1331.2010.03019.x

12. Punga AR, Sawada M, Stalberg EV. Electrophysiological signs and the prevalence of adverse effects of acetylcholinesterase inhibitors in patients with myasthenia gravis. Muscle Nerve. 2008;37(3):300–307. PMID: 18069667 https://doi.org/10.1002/mus.20935

13. Sanadze AG. Miasteniya i miastenicheskie sindromy. 2nd ed. Moscow: GEOTAR-Media Publ.; 2017. (in Russ.)

14. Dedaev SI. Antibodies to autoantigen targets in myasthenia and their value in clinical practice. Neuromuscular Diseases. 2014;(2):6–15. (In Russ.) https://doi.org/10.17650/2222-8721-2014-0-2-6-15

15. Matney SE, Huff DR. Diagnosis and treatment of myasthenia gravis. Consult Pharm. 2007; 22(3):239–248. PMID: 17658970 http://doi.org/10.4140/TCP.n.2007.239

16. Shcherbakova NI, Piradov MA, Pavlova EM, Ryabinkina YuV, Pirogov VN, Gurkina GT, et al . The Causes, Risk Factors and Predictors of Myasthenia Gravis Crisis. The Neurological Journal. 2013;(2):11–19. (in Russ.)

17. Vasilyeva EB, Kartavenko VI, Petrikov SS, Badygov SA, Sidnev DV, Savin LA. Diagnosis and Treatment of New-Onset Myasthenia Gravis in Critically Ill Patients. Russian Sklifosovsky Journal Emergency Medical Care. 2014;(3):34–37. (in Russ.)

18. Wiktor AJ, Haft JW, Bartlett RH, Park PK, Raghavendran K, Napolitano LM. Prolonged VV ECMO (265 Days) for ARDS without technical complications. ASAIO J. 2015;61(2):205–206. PMID: 25423122 https://doi.org/10.1097/mat.0000000000000181

19. Posluszny J, Rycus PT, Bartlett RH, Engoren M, Haft JW, Lynch WR, et al. Outcome of Adult Respiratory Failure Patients Receiving Prolonged (≥14 Days) ECMO. Ann Surg. 2016;263(3):573–581. PMID: 26625136 https://doi.org/10.1097/sla.0000000000001176

20. Oude Lansink-Hartgring A, de Vries AJ, Droogh JM, van den Bergh WM. Hemorrhagic complications during extracorporeal membrane oxygenation – The role of anticoagulation and platelets. J Crit Care. 2019;54:239–243. PMID: 31630073 https://doi.org/10.1016/j.jcrc.2019.09.013

21. Shah AG, Peahota M, Thoma BN, Kraft WK. Medication Complications in Extracorporeal Membrane Oxygenation. Crit Care Clin. 2017;33(4):897– 920. PMID: 28887935 https://doi.org/10.1016/j.ccc.2017.06.010

22. Sutter R, Tisljar K, Marsch S. Acute Neurologic Complications During Extracorporeal Membrane Oxygenation: A Systematic Review. Crit Care Med. 2018;46(9):1506–1513. PMID: 29782356 https://doi.org/10.1097/ccm.0000000000003223

23. Vellipuram AR, Cruz-Flores S, Chaudhry MRA, Rawla P, Maud A, Rodriguez GJ, et al. Comparative Outcomes of Respiratory Failure Associated with Common Neuromuscular Emergencies: Myasthenia Gravis versus Guillain-Barré Syndrome. Medicina (Kaunas). 2019;55(7):375. PMID: 31311172 https://doi.org/10.3390/medicina55070375