ИНТЕНСИВНАЯ ТЕРАПИЯ МИАСТЕНИЧЕСКИХ КРИЗОВ
Обзорная статья
Дата публикации: августа 26, 2024
Ключевые слова
миастения гравис
миастенический криз
интенсивная терапия
миастенический криз
интенсивная терапия
Как цитировать
Быков Ю. В., Обедин А. Н., Фишер В. В., Яцук И. В., Волков Е. В., Зинченко О. В., Муравьёва А. А. ИНТЕНСИВНАЯ ТЕРАПИЯ МИАСТЕНИЧЕСКИХ КРИЗОВ // Кремлевская медицина. Клинический вестник. 2024. Т. № 3. С. 42-47.
Аннотация
Миастенический криз (МК) – редкое и потенциально опасное осложнение миастении гравис, требующее интенсивной терапии в условиях отделения анестезиологии и реанимации (ОАИР). МК проявляется нарастанием острой дыхательной недостаточности на фоне прогрессирующего снижения тонуса дыхательной мускулатуры и параллельной гипотонией бульбарных мышц. К симптоматической терапии относят введение ингибиторов ацетилхолинэстеразы, быстрая иммуномодулирующая терапия (иммуноадсорбция и плазмаферез), назначение глюкокортикостероидов и иммунодепрессантов (азатиоприн, ритуксимаб, экулизумаб). При отсутствии эффекта переходят к респираторной поддержке – неинвазивной или искусственной вентиляции лёгких с интубацией трахеи. Повышение информированности о МК и чёткое следование терапевтическим алгоритмам улучшат качество оказания экстренной помощи данному контингенту пациентов. Цель обзора – анализ и обсуждение современной зарубежной литературы, посвящённой вопросам интенсивной терапии МК в условиях ОАИР.Литература
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2. Prado MB Jr, Adiao KJ. Acetylcholinesterase Inhibitors in Myasthenic Crisis: A Systematic Review of Observational Studies. Neurocrit Care. – 2021. – V. 35. –No. 2. – Р. 528-544. doi: 10.1007/s12028-021-01259-4.
3. Mishra AK et al. Myasthenia Gravis: A Systematic Review. Cureus. – 2023. – V. 15. – No. 12. – Р. 50017. doi: 10.7759/cureus.50017.
4. Gilhus NE. Myasthenia gravis, respiratory function, and respiratory tract disease. J Neurol. – 2023. – V. 270. – No. 7. – P. 3329-3340. doi: 10.1007/s00415-023-11733-y.
5. Alhaidar MK et al. Current Treatment of Myasthenia Gravis. J Clin Med. –2022. – V. 11. – No. 6. Р. – 1597. doi: 10.3390/jcm11061597.
6. Hogan C et al. Acute Myasthenia Crisis: A Critical Emergency Department Differential. Cureus. – 2020. – V. 12. – No. 8. – Р. 9760. doi: 10.7759/cureus.9760.
7. Payus AO et al. Myasthenic Crisis as the First Presentation of Myasthenia Gravis: A Case Report. Am J Case Rep. – 2021. – V. 22. – P. e928419-1–e928419-4. doi: 10.12659/AJCR.928419.
8. Spillane J et al. Easily missed? Myasthenia gravis. BMJ. – 2013. – V. 19. – P. 36–38.
9. Tran D et al. Myasthenic crisis as an initial presentation of myasthenia gravis in an 81-year-old following endoscopic myotomy for Zenker’s diverticulum. Proc (Bayl Univ Med Cent). – 2023. – V. 36. – No. 4. – P. 514–517. doi: 10.1080/08998280.2023.2201657.
10. Prabhu SS et al. Anesthetic considerations for a patient with myasthenia gravis undergoing deep sedation in an outpatient oral surgery setting. J Dent Anesth Pain Med. – 2019. – V. 19. - No. 1. – Р. 67–72. doi: 10.17245/jdapm.2019.19.1.67.
11. Bershad EM et al. Myasthenia gravis crisis. South Med J. – 2008. – V. 10. – No. 11. – P. 63–69. doi: 10.1097/SMJ.0b013e31815d4398.
12. Claytor B et al. Myasthenic crisis. Muscle Nerve. 2023. – V. 68. – No.1. – P. 8-19. doi: 10.1002/mus.27832.
13. Munot P et al. 242nd ENMC international diagnosis and management of juvenile myasthenia gravis Hoofddorp, the Netherlands, 1-3 March 2019. Neuromuscul Disord. – 2020. – V. 30. – P. 254–64. doi: 10.1016/j.nmd.2020.02.001.
14. Stetefeld HR, Schroeter M. [Myasthenic Crisis]. Fortschr Neurol Psychiatr. – 2018. – V. 86. – No. 5. – Р. 301-307. doi: 10.1055/a-0599-0811.
15. Alshekhlee A et al. Incidence and mortality rates of myasthenia gravis and myasthenic crisis in US hospitals. Neurology. – 2009. – V. 72. – P. 1548–1554. doi: 10.1212/WNL.0b013e3181a41211.
16. Farrugia ME, Goodfellow JA. A Practical Approach to Managing Patients With Myasthenia Gravis-Opinions and a Review of the Literature. Front Neurol. – 2020. – V. 11. – P. 604. doi: 10.3389/fneur.2020.00604.
17. Popperud TH et al. Juvenile myasthenia gravis in Norway: clinical characteristics, treatment, and long-term outcome in a nationwide population-based cohort. Eur J Paediatr Neurol. – 2017. – V. 21. - Р. 707–714. doi: 10.1016/j.ejpn.2017.04.003.
18. Tomschik M et al. Subgroup stratification and outcome in recently diagnosed generalized myasthenia gravis. Neurology. – 2020. – V. 95. – P. –1426–1436. doi: 10.1212/WNL.0000000000010209.
19. Neumann B et al. Myasthenic crisis demanding mechanical ventilation: a multicenter analysis of 250 cases. Neurology. – 2020. – V. 94. – P. 299–313. doi: 10.1212/WNL.0000000000008688.
20. Liu F et al. Myasthenic crisis treated in a Chinese neurological intensive care unit: clinical features, mortality, outcomes, and predictors of survival. Bmc Neurol. – 2019. – V. – 19. – P. 22. doi: 10.1186/s12883-019-1384-5.
21. Godoy DA et al. The myasthenic patient in crisis: an update of the management in neurointensive care unit. Arq Neuropsiquiatr. – 2013. – V. 71. – P. 627–639. doi: 10.1590/0004-282X20130108.
22. Stetefeld H, Schroeter M. SOP myasthenic crisis. Neurol Res Pract. –2019. – V. 1. – P. 19. doi: 10.1186/s42466-019-0023-3.
23. Wendell LC, Levine JM. Myasthenic crisis. Neurohospitalist. – 2011. – V. 1. – P. 16–22. doi: 10.1177/1941875210382918.
24. Ramos-Fransi A et al. Myasthenia gravis: Descriptive analysis of life-threatening events in a recent nationwide registry. Eur J Neurol. – 2015. V. 22. – P. 1056– 1061. doi: 10.1111/ene.12703
25. Evoli A et al. Myasthenia gravis with antibodies to MuSK: an update. Ann N Y Acad Sci. – 2018. – V. 1412. – P. 82–89. doi: 10.1111/nyas.13518.
26. Walters J. Weakness in the intensive care unit. Pract Neurol. – 2022. – V. 22. – P. 358. doi: 10.1136/pn-2022-003422.
27. Wiendl H. Diagnostik und Therapie der Myasthenia gravis und des Lambert- Eaton- Syndroms Die wichtigsten Empfehlungen auf einen Blick Definition und Klassifikation. DGN Leitlinien. – 2012. – P. 1–29.
28. Wang Y et al. Plasma exchange versus intravenous immunoglobulin in AChR subtype myasthenic crisis: a prospective cohort study. Clin Immunol. – 2022. – V. 241. – P. 109058. doi: 10.1016/j.clim.2022.109058.
29. Köhler W et al. A randomized and controlled study comparing immunoadsorption and plasma exchange in myasthenic crisis. J Clin Apher. – 2011. – V. 26. – P. 347–355. doi: 10.1002/jca.20317.
30. Schneider-Gold C et al. Immunoadsorption versus plasma exchange versus combination for treatment of myasthenic deterioration. Ther Adv Neurol Disord. – 2016. – V. 9. – P. 297–303. doi: 10.1177/1756285616637046.
31. Dhawan PS et al. IVIG versus PLEX in the treatment of worsening myasthenia gravis: what is the evidence? A critically appraised topic. Neurologist. – 2015. – V. 19. – P. 145–148. doi: 10.1097/NRL.0000000000000026.
32. Sanders DB et al. International consensus guidance for management of myasthenia gravis: executive summary. Neurology. – 2016. – V. 87. – P. 419–425. doi: 10.1212/WNL.0000000000002790.
33. Gilhus NE. Myasthenia Gravis. N Engl J Med. – 2016. – V. 375. – No. 26. – P. 2570–81. doi: 10.1056/NEJMra1602678.
34. Murthy JM. Myasthenic Crisis – Comorbidities, Complications, Long-Term Outcomes: The Challenges. Ann Indian Acad Neurol. – 2019. – V. 22. – No. 4. – P. 472–473. doi: 10.4103/aian.AIAN_472_19.
35. Guptill JT et al. A Retrospective study of complications of therapeutic plasma exchange in myasthenia. Muscle Nerve. – 2013. – V. 47. – P. 170–176. doi: 10.1002/mus.23508.
36. Heatwole C et al. Plasma exchange versus intravenous immunoglobulin for myasthenia gravis crisis: an acute hospital cost comparison study. J Clin Neuromuscul Dis. – 2011. – V. 13. – P. 85–94. doi: 10.1097/CND.0b013e31822c34dd
37. Melzer N et al. Clinical features, pathogenesis, and treatment of myasthenia gravis: a supplement to the Guidelines of the German Neurological Society. J Neurol. – 2016. – V. 263. – P. 1473–1494. doi: 10.1007/s00415-016-8045-z.
38. Sussman J et al. Myasthenia gravis: association of British neurologists' management guidelines. Pract Neurol. – 2015. – V. 15. – P. 199–206. doi: 10.1136/practneurol-2015-001126.
39. Lacomis D. Myasthenic crisis. Neurocrit Care. – 2005. – V. 3. – P. 189–194.
40. Roper J et al. Myasthenia gravis and crisis: evaluation and management in the emergency department. J Emerg Med. – 2017. – V. 53. – P. 843–853. doi: 10.1016/j.jemermed.2017.06.009.
41. Wu JY et al. The role of non-invasive ventilation and factors predicting extubation outcome in myasthenic crisis. Neurocrit Care. – 2009. – V. 10. – P. 35–42. doi: 10.1007/s12028-008-9139-y.
42. Seneviratne J et al. Noninvasive ventilation in myasthenic crisis. Arch Neurol. – 2008. – V. 65. – P. 54–58. doi: 10.1001/archneurol.2007.1.
43. Wu JY et al. The role of noninvasive ventilation and factors predicting extubation outcome in myasthenic crisis. Neurocrit Care. 2009. – V. 10. –P. 35–42. doi: 10.1007/s12028-008-9139-y.
44. Liu CC et al. Post-traumatic stress disorder symptoms after respiratory insufficiency in patients with myasthenia gravis. Psychol Health Med. –2021. – V. 26. – P. 221–227. doi: 10.1080/13548506.2020.1807577.
45. Jamal BT, Herb K. Perioperative management of patients with myasthenia gravis: Prevention, recognition, and treatment. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. – 2009. – V. 1. – P. 612–615. doi: 10.1016/j.tripleo.2009.01.015.
2. Prado MB Jr, Adiao KJ. Acetylcholinesterase Inhibitors in Myasthenic Crisis: A Systematic Review of Observational Studies. Neurocrit Care. – 2021. – V. 35. –No. 2. – Р. 528-544. doi: 10.1007/s12028-021-01259-4.
3. Mishra AK et al. Myasthenia Gravis: A Systematic Review. Cureus. – 2023. – V. 15. – No. 12. – Р. 50017. doi: 10.7759/cureus.50017.
4. Gilhus NE. Myasthenia gravis, respiratory function, and respiratory tract disease. J Neurol. – 2023. – V. 270. – No. 7. – P. 3329-3340. doi: 10.1007/s00415-023-11733-y.
5. Alhaidar MK et al. Current Treatment of Myasthenia Gravis. J Clin Med. –2022. – V. 11. – No. 6. Р. – 1597. doi: 10.3390/jcm11061597.
6. Hogan C et al. Acute Myasthenia Crisis: A Critical Emergency Department Differential. Cureus. – 2020. – V. 12. – No. 8. – Р. 9760. doi: 10.7759/cureus.9760.
7. Payus AO et al. Myasthenic Crisis as the First Presentation of Myasthenia Gravis: A Case Report. Am J Case Rep. – 2021. – V. 22. – P. e928419-1–e928419-4. doi: 10.12659/AJCR.928419.
8. Spillane J et al. Easily missed? Myasthenia gravis. BMJ. – 2013. – V. 19. – P. 36–38.
9. Tran D et al. Myasthenic crisis as an initial presentation of myasthenia gravis in an 81-year-old following endoscopic myotomy for Zenker’s diverticulum. Proc (Bayl Univ Med Cent). – 2023. – V. 36. – No. 4. – P. 514–517. doi: 10.1080/08998280.2023.2201657.
10. Prabhu SS et al. Anesthetic considerations for a patient with myasthenia gravis undergoing deep sedation in an outpatient oral surgery setting. J Dent Anesth Pain Med. – 2019. – V. 19. - No. 1. – Р. 67–72. doi: 10.17245/jdapm.2019.19.1.67.
11. Bershad EM et al. Myasthenia gravis crisis. South Med J. – 2008. – V. 10. – No. 11. – P. 63–69. doi: 10.1097/SMJ.0b013e31815d4398.
12. Claytor B et al. Myasthenic crisis. Muscle Nerve. 2023. – V. 68. – No.1. – P. 8-19. doi: 10.1002/mus.27832.
13. Munot P et al. 242nd ENMC international diagnosis and management of juvenile myasthenia gravis Hoofddorp, the Netherlands, 1-3 March 2019. Neuromuscul Disord. – 2020. – V. 30. – P. 254–64. doi: 10.1016/j.nmd.2020.02.001.
14. Stetefeld HR, Schroeter M. [Myasthenic Crisis]. Fortschr Neurol Psychiatr. – 2018. – V. 86. – No. 5. – Р. 301-307. doi: 10.1055/a-0599-0811.
15. Alshekhlee A et al. Incidence and mortality rates of myasthenia gravis and myasthenic crisis in US hospitals. Neurology. – 2009. – V. 72. – P. 1548–1554. doi: 10.1212/WNL.0b013e3181a41211.
16. Farrugia ME, Goodfellow JA. A Practical Approach to Managing Patients With Myasthenia Gravis-Opinions and a Review of the Literature. Front Neurol. – 2020. – V. 11. – P. 604. doi: 10.3389/fneur.2020.00604.
17. Popperud TH et al. Juvenile myasthenia gravis in Norway: clinical characteristics, treatment, and long-term outcome in a nationwide population-based cohort. Eur J Paediatr Neurol. – 2017. – V. 21. - Р. 707–714. doi: 10.1016/j.ejpn.2017.04.003.
18. Tomschik M et al. Subgroup stratification and outcome in recently diagnosed generalized myasthenia gravis. Neurology. – 2020. – V. 95. – P. –1426–1436. doi: 10.1212/WNL.0000000000010209.
19. Neumann B et al. Myasthenic crisis demanding mechanical ventilation: a multicenter analysis of 250 cases. Neurology. – 2020. – V. 94. – P. 299–313. doi: 10.1212/WNL.0000000000008688.
20. Liu F et al. Myasthenic crisis treated in a Chinese neurological intensive care unit: clinical features, mortality, outcomes, and predictors of survival. Bmc Neurol. – 2019. – V. – 19. – P. 22. doi: 10.1186/s12883-019-1384-5.
21. Godoy DA et al. The myasthenic patient in crisis: an update of the management in neurointensive care unit. Arq Neuropsiquiatr. – 2013. – V. 71. – P. 627–639. doi: 10.1590/0004-282X20130108.
22. Stetefeld H, Schroeter M. SOP myasthenic crisis. Neurol Res Pract. –2019. – V. 1. – P. 19. doi: 10.1186/s42466-019-0023-3.
23. Wendell LC, Levine JM. Myasthenic crisis. Neurohospitalist. – 2011. – V. 1. – P. 16–22. doi: 10.1177/1941875210382918.
24. Ramos-Fransi A et al. Myasthenia gravis: Descriptive analysis of life-threatening events in a recent nationwide registry. Eur J Neurol. – 2015. V. 22. – P. 1056– 1061. doi: 10.1111/ene.12703
25. Evoli A et al. Myasthenia gravis with antibodies to MuSK: an update. Ann N Y Acad Sci. – 2018. – V. 1412. – P. 82–89. doi: 10.1111/nyas.13518.
26. Walters J. Weakness in the intensive care unit. Pract Neurol. – 2022. – V. 22. – P. 358. doi: 10.1136/pn-2022-003422.
27. Wiendl H. Diagnostik und Therapie der Myasthenia gravis und des Lambert- Eaton- Syndroms Die wichtigsten Empfehlungen auf einen Blick Definition und Klassifikation. DGN Leitlinien. – 2012. – P. 1–29.
28. Wang Y et al. Plasma exchange versus intravenous immunoglobulin in AChR subtype myasthenic crisis: a prospective cohort study. Clin Immunol. – 2022. – V. 241. – P. 109058. doi: 10.1016/j.clim.2022.109058.
29. Köhler W et al. A randomized and controlled study comparing immunoadsorption and plasma exchange in myasthenic crisis. J Clin Apher. – 2011. – V. 26. – P. 347–355. doi: 10.1002/jca.20317.
30. Schneider-Gold C et al. Immunoadsorption versus plasma exchange versus combination for treatment of myasthenic deterioration. Ther Adv Neurol Disord. – 2016. – V. 9. – P. 297–303. doi: 10.1177/1756285616637046.
31. Dhawan PS et al. IVIG versus PLEX in the treatment of worsening myasthenia gravis: what is the evidence? A critically appraised topic. Neurologist. – 2015. – V. 19. – P. 145–148. doi: 10.1097/NRL.0000000000000026.
32. Sanders DB et al. International consensus guidance for management of myasthenia gravis: executive summary. Neurology. – 2016. – V. 87. – P. 419–425. doi: 10.1212/WNL.0000000000002790.
33. Gilhus NE. Myasthenia Gravis. N Engl J Med. – 2016. – V. 375. – No. 26. – P. 2570–81. doi: 10.1056/NEJMra1602678.
34. Murthy JM. Myasthenic Crisis – Comorbidities, Complications, Long-Term Outcomes: The Challenges. Ann Indian Acad Neurol. – 2019. – V. 22. – No. 4. – P. 472–473. doi: 10.4103/aian.AIAN_472_19.
35. Guptill JT et al. A Retrospective study of complications of therapeutic plasma exchange in myasthenia. Muscle Nerve. – 2013. – V. 47. – P. 170–176. doi: 10.1002/mus.23508.
36. Heatwole C et al. Plasma exchange versus intravenous immunoglobulin for myasthenia gravis crisis: an acute hospital cost comparison study. J Clin Neuromuscul Dis. – 2011. – V. 13. – P. 85–94. doi: 10.1097/CND.0b013e31822c34dd
37. Melzer N et al. Clinical features, pathogenesis, and treatment of myasthenia gravis: a supplement to the Guidelines of the German Neurological Society. J Neurol. – 2016. – V. 263. – P. 1473–1494. doi: 10.1007/s00415-016-8045-z.
38. Sussman J et al. Myasthenia gravis: association of British neurologists' management guidelines. Pract Neurol. – 2015. – V. 15. – P. 199–206. doi: 10.1136/practneurol-2015-001126.
39. Lacomis D. Myasthenic crisis. Neurocrit Care. – 2005. – V. 3. – P. 189–194.
40. Roper J et al. Myasthenia gravis and crisis: evaluation and management in the emergency department. J Emerg Med. – 2017. – V. 53. – P. 843–853. doi: 10.1016/j.jemermed.2017.06.009.
41. Wu JY et al. The role of non-invasive ventilation and factors predicting extubation outcome in myasthenic crisis. Neurocrit Care. – 2009. – V. 10. – P. 35–42. doi: 10.1007/s12028-008-9139-y.
42. Seneviratne J et al. Noninvasive ventilation in myasthenic crisis. Arch Neurol. – 2008. – V. 65. – P. 54–58. doi: 10.1001/archneurol.2007.1.
43. Wu JY et al. The role of noninvasive ventilation and factors predicting extubation outcome in myasthenic crisis. Neurocrit Care. 2009. – V. 10. –P. 35–42. doi: 10.1007/s12028-008-9139-y.
44. Liu CC et al. Post-traumatic stress disorder symptoms after respiratory insufficiency in patients with myasthenia gravis. Psychol Health Med. –2021. – V. 26. – P. 221–227. doi: 10.1080/13548506.2020.1807577.
45. Jamal BT, Herb K. Perioperative management of patients with myasthenia gravis: Prevention, recognition, and treatment. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. – 2009. – V. 1. – P. 612–615. doi: 10.1016/j.tripleo.2009.01.015.