A case of severe generalized myasthenia with late onset and predominant lesion of bulbar muscles: the case report and review

Cover Page

Cite item

Full Text

Abstract

Myasthenia gravis according to modern concepts is a heterogeneous group of autoimmune pathology with a postsynaptic neuromuscular transmission defectsts and can be classified by the prevalence of the lesion, the detection of specific antibodies, the age at onset of the disease, the presence of thymus pathology. Such a clinico-pathogenetic heterogencity creates difficulties in the diagnostics and treatment of the disease. With late (over 50 years) myasthenia gravis debilily, the disease often has flow characteristics: a predominat lesion of bulbar musculature, a severe progressive course, a lack of proper effect from the therapy. If in patients with myastenia gravis of older age there is vascular load, then it is often mistakenly diagnosed with cerebral stroke. Presented case severe generalized myasthenia gravis with bulbar syndrome in a patient of 60 years old, shows the features of the cours of this disease with late debute.

About the authors

A. V Serdyuk

N.I.Pirogov Russian National Research Medical University of the Ministry of Health of the Russian Federation

Email: aserdyuk@gmail.com
канд. мед. наук, ассистент каф. неврологии, нейрохирургии и мед. генетики 117997, Russian Federation, Moscow, ul. Ostrovitianova, d. 1

E. A Kovrazhkina

N.I.Pirogov Russian National Research Medical University of the Ministry of Health of the Russian Federation

канд. мед. наук, ст. науч. сотр. НИИ цереброваскулярной патологии и инсульта 117997, Russian Federation, Moscow, ul. Ostrovitianova, d. 1

N. V Vyatkina

City Clinical Hospital №31 of the Department of Health of Moscow

врач-невролог 119415, Russian Federation, Moscow, ul. Lobachevskogo, d. 42

References

  1. Sieb J.P. Myasthenia gravis: an update for the clinician. Clin Exp Immunol 2013; 175: 408-18. doi: 10.1111/cei.12217
  2. Farrugia M.E, Robson M.D, Clover L et al. MRI and clinical studies of facial and bulbar muscle involvement in MuSK antibody-associated myasthenia gravis. Brain 2006; 129: 1481-92.
  3. Baggi F, Andreetta F, Maggi L et al. Complete stable remission and autoantibody specificity in myasthenia gravis. Neurology 2013; 80: 188-95. doi: 10.1212/WNL.0b013e31827b907b
  4. Pasnoor M, Wolfe G.I, Nations S et al. Clinical findings in MuSK antibody positive myasthenia gravis: a U.S. experience. Muscle Nerve 2010; 41: 370-4. doi: 10.1002/mus.21533
  5. Pakzad Z, Aziz T, Oger J. Increasing incidence of myasthenia gravis among elderly in British Columbia, Canada. Neurology 2011; 76: 1526-8. doi: 10.1212/WNL.0b013e318217e735
  6. Matsuda M, Dohi-Iijima N, Nakamura A et al. Increase in incidence of elderly-onset patients with myasthenia gravis in Nagano Prefecture, Japan. Intern Med 2005; 44: 572-7.
  7. Libman R, Benson R, Einberg K. Myasthenia mimicking vertebrobasilar stroke. J Neurol 2002; 249: 1512-4.
  8. Basiri K, Ansari B, Okhovat A.A. Life-threatening misdiagnosis of bulbar onset myasthenia gravis as a motor neuron disease: How much can one rely on exaggerated deep tendon reflexes. Adv Biomed Res 2015; 4: 58. doi: 10.4103/2277-9175.151874
  9. Kim S.J, Park G.Y, Choi Y.M et al. Bulbar myasthenia gravis superimposed in a medullary infarction diagnosed by a fiberoptic endoscopic evaluation of swallowing with simultaneous tensilon application. Ann Rehabil Med 2017; 41 (6): 1082-7. doi: 10.5535/arm.2017.41.6.1082
  10. Kleiner-Fisman G, Kott H.S. Myasthenia gravis mimicking stroke in elderly patients. Mayo Clin Proc 1998; 73: 1077-8.
  11. Vincent A, McConville J, Farrugia M.E, Newsom-Davis J. Seronegative myasthenia gravis. Semin Neurol 2004; 24: 125-33.
  12. Baggi F, Andreetta F, Maggi L et al. Complete stable remission and autoantibody specificity in myasthenia gravis. Neurology 2013; 80: 188-95. doi: 10.1212/WNL.0b013e31827b907b
  13. Deymeer F, Gungor-Tuncer O, Yilmaz V et al. Clinical comparison of anti-MuSK- vs anti-AChR-positive and seronegative myasthenia gravis. Neurology 2007; 68: 609-11.
  14. Бардаков С.Н., Живолупов С.А., Рашидов Н.А. Иммунологическая и клиническая гетерогенность миастении. Вестн. Российской Военно-медицинской академии. 2016; 1 (53): 154-64.
  15. Щербакова Н.И., Пирадов М.А., Павлова Е.М. и др. Причины, факторы риска, клинические предикторы развития кризов у больных миастенией. Неврол. журн. 2013; 2: 11-9
  16. Романова Т.В. Миастения с ранним и поздним началом заболевания. Саратов. науч.-мед. журн. 2016; 12 (2): 287-92.
  17. Супонева Н.А., Гришина Д.А. Внутривенная высокодозная иммунотерапия: практические рекомендации по применению в лечении дизиммунных заболеваний периферического нейромоторного аппарата. Нервно-мышечные болезни. 2015; 4: 16-23. DOI: 10.17 650 / 2222-8721-2015-5-4-16-23
  18. Санадзе А.Г. Эффективность и целесообразность внутривенного введения человеческого иммуноглобулина в патогенетической терапии больных с генерализованной миастенией. Журн. неврологии и психиатрии им. С.С.Корсакова. 2011; 6: 29-32.

Supplementary files

Supplementary Files
Action
1. JATS XML

Copyright (c) 2018 Consilium Medicum

Creative Commons License
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.

Согласие на обработку персональных данных

 

Используя сайт https://journals.rcsi.science, я (далее – «Пользователь» или «Субъект персональных данных») даю согласие на обработку персональных данных на этом сайте (текст Согласия) и на обработку персональных данных с помощью сервиса «Яндекс.Метрика» (текст Согласия).