Preview

Medical alphabet

Advanced search
Open Access Open Access  Restricted Access Subscription or Fee Access

Pain syndrome, “dropped head” and myasthenia gravis: a case report and literature review

https://doi.org/10.33667/2078-5631-2024-21-7-12

Abstract

The objective of the study is to present a description of the first domestic clinical observation of the painful dropped head syndrome in a patient with myasthenia gravis (MG).

Materials and Methods. Clinical, laboratory and instrumental data were analyzed over the course of MG symptoms with late onset in combination with thymoma, manifesting with pain and weakness in the neck extensors.

Results. The characteristic and unique features of diagnostics and treatment of MG developed in a 59-year-old patient associated with antecollis and significant pain syndrome, initially considered as manifestations of osteochondrosis, are observed in comparison with the literature data. The information on the occurrence, demographic origin, association with the type of receptor targets, thymus pathology and reversibility of these disorders in MG is systematized, and the importance of addressing the concepts of pain in MG is emphasized. The positive effect of ipidacrine in the symptomatic treatment of MG in a patient with marked cholinergic manifestations induced by conventional anticholinergic agents (AA) therapy is confirmed.

Conclusions. MG can cause axial muscle impairment leading to the phenomenon of “dropped head” and the development of pain syndrome atypical for the classical course of the disease. Pathogenetic treatment of seropositive MG in most cases leads to the elimination of its clinical manifestations, including pain. In some cases, the use of the reversible AA ipidacrine is an additional advantage in the therapy of such conditions, taking into account its concomitant proven efficacy as an adjuvant analgesic.

About the Authors

Yu. A. Merkulov
Scientific Research Institute of General Pathology and Pathophysiology
Russian Federation

Yury A. Merkulov - DM Sci (habil.), chief researcher at the Laboratory of Fundamental and Applied Problems of Pain.

Moscow



E. L. Plieva
B.M. Guekht Neurology center, Central clinical hospital “RZD-Medicine”
Russian Federation

Elizaveta L. Plieva – neurologist.

Moscow



D. M. Merkulova
B.M. Guekht Neurology center, Central clinical hospital “RZD-Medicine”; I.M. Sechenov First Moscow State Medical University of the Ministry of Healthcare of the Russian Federation (Sechenovskiy University)
Russian Federation

Dina M. Merkulova - DM Sci (habil.), head of the Center, B.M. Guekht Neurology center, Central clinical hospital «RZD-Medicine»; professor at Dept of Neurology at the Institute of Professional Education, I.M. Sechenov First Moscow State Medical University of the Ministry of Healthcare of the Russian Federation (Sechenovskiy University).

Moscow



References

1. Gekht B. M., Ilyina N. A. Neuromuscular diseases. M.: Medicine, 1982. 352 p. (In Russ.).

2. Burakgazi A. Z., Richardson P. K., Abu-Rub M. Dropped head syndrome due to neuromuscular disorders: Clinical manifestation and evaluation. Neurol. Int. 2019; 11 (3): 8198. DOI: 10.4081/ni.2019.8198

3. Kamchatnov P. R. Spondylogenic dorsalgia: tactics of conservative therapy. Consilium Medicum. 2007; 9 (2): 33–38. (In Russ.).

4. Merkulov Yu.A., Hamburg A. M., Lezina D. S., Fedorova A. N., Onsin A. A., Merkulova D. M. Optimization of diagnostics and treatment of dorsalgia in real world clinical practice: secondary endpoint of the DORISS multicenter observational study. Korsakov Journal of Neurology and Psychiatry. 2023; 123 (2): 1–10. DOI: 10.17116/jnevro20231230211

5. Mikhailyuk I. G. Analysis of the correctness of diagnostics of the causes of back pain in a neurological hospital in accordance with the international classification of diseases. Neuromuscular diseases. 2014; (3): 20–27. (In Russ.). DOI: 10.17650/2222-8721-2014-0-3-20-27

6. Lloyd J. M., Mitchell R. G. Myasthenia gravis as a cause of facial pain. Oral Surg. Oral Med. Oral Pathol. 1988l; 66 (1): 45–6. DOI: 10.1016/0030–4220(88)90065-5

7. Rostedt A., Stålberg E. Joint pain and hyperalgesia due to pyridostigmine bromide in a patient with myasthenia gravis. Neurology. 2004; 62 (5): 835–6. DOI: 10.1212/01.wnl.0000113756.23340.2f

8. Tong O., Delfiner L., Herskovitz S. Pain, Headache, and Other Non-motor Symptoms in Myasthenia Gravis. Curr Pain Headache Rep. 2018; 22 (6): 39. DOI: 10.1007/s11916-018-0687-3

9. Scott K. R., Kothari M. J. Self-reported Pain Affects Quality of Life in Myasthenia Gravis. J. Clin. Neuromuscul. Dis. 2006; 7 (3): 110–4. DOI: 10.1097/01.cnd.0000202223.39994.c0

10. Dewilde S., Philips G., Paci S., Beauchamp J., Chiroli S., Quinn C., Day L., Larkin M., Palace J., Berrih-Aknin S., Claeys K. G., Muppidi S., Mantegazza R., Saccà F., Meisel A., Bassez G., Murai H., Janssen M. F. Patient-reported burden of myasthenia gravis: baseline results of the international prospective, observational, longitudinal real-world digital study MyRealWorld-MG. BMJ Open. 2023; 13 (1): e066445. DOI: 10.1136/bmjopen-2022–066445

11. Guy-Coichard C., Nguyen D. T., Delorme T., Boureau F. Pain in hereditary neuromuscular disorders and myasthenia gravis: a national survey of frequency, characteristics, and impact. J. Pain. Symptom Manage. 2008; 35 (1): 40–50. DOI: 10.1016/j.jpainsymman.2007.02.041

12. Padua L., Evoli A., Aprile I., Caliandro P., Mazza S., Padua R., Tonali P. Health-related quality of life in patients with myasthenia gravis and the relationship between patient-oriented assessment and conventional measurements. Neurol Sci. 2001; 22 (5): 363–9. DOI: 10.1007/s100720100066

13. https://myastheniagravisnews.com/columns/pain-real-as-myasthenia-gravis/

14. https://www.newswise.com/articles/pain-often-overlooked-in-myasthenia-gravis-patients

15. https://www.institut-myologie.org/en/2021/09/22/myasthenia-gravis-a-very-often-painful-disease/

16. Leon-Sarmiento F.E., Leon-Ariza J.S., Prada D., Leon-Ariza D.S., Rizzo-Sierra C. V. Sensory aspects in myasthenia gravis: A translational approach. J. Neurol. Sci. 2016; 368: 379–88. DOI: 10.1016/j.jns.2016.07.014

17. D'Amelio M., Di Benedetto N., Ragonese P., Daniele O., Brighina F., Fierro B., Savettieri G. Dropped head as an unusual presenting sign of myasthenia gravis. Neurol. Sci. 2007; 28 (2): 104–6. DOI: 10.1007/s10072–007–0796-y

18. Drain J. P., Virk S. S., Jain N., Yu E. Dropped Head Syndrome: A Systematic Review. Clin Spine Surg. 2019; 32 (10): 423–429. DOI: 10.1097/BSD.0000000000000811

19. Kreinter-Rosembaun H., Moutran-Barroso H., Augusto-Forero C., Gómez-Mazuera A., Martínez-Rubio C. Dropped Head Syndrome in Myasthenia Gravis after a SARS-Cov2 Infection. Rev Ecuat Neurol [online]. 2023; 32 (1): 81–84. DOI: 10.46997/revecuatneurol32100081

20. Hansraj K. K. Assessment of stresses in the cervical spine caused by posture and position of the head. Surg Technol Int. 2014; 25: 277–9.

21. Mahmoud N. F., Hassan K. A., Abdelmajeed S. F., Moustafa I. M., Silva A. G. The Relationship Between Forward Head Posture and Neck Pain: a Systematic Review and Meta-Analysis. Curr Rev Musculoskelet Med. 2019; 12 (4): 562–577. DOI: 10.1007/s12178–019–09594-y

22. Mujawar J. C., Sagar J. H. Prevalence of Upper Cross Syndrome in Laundry Workers. Indian J. Occup Environ Med. 2019; 23 (1): 54–56. DOI: 10.4103/ijoem.IJOEM_169_18

23. Aoki K. Short history of epidemiology for noninfectious diseases in Japan. Part 1: selected diseases and related episodes from 1880 through 1944. J. Epidemiol. 2007; 17 (1): 1–18. DOI: 10.2188/jea.17.1

24. Grascenkov N. I. Tick-Borne Encephalitis in the USSR. Bull World Health Organ. 1964; 30 (2): 187–96.

25. Gómez-Piña J. J. Characterizing Dropped Head Syndrome across neurologic disorders, the hidden epidemic of dropped head syndrome. Int J. Res Med. Sci. 2023; 11 (12): 4306–11. DOI: 10.18203/2320–6012.ijrms20233693

26. Puruckherr M., Pooyan P., Dube D., Byrd R. P., Roy T. M. The dropped head sign: an unusual presenting feature of myasthenia gravis. Neuromuscul Disord. 2004; 14 (6): 378–9. DOI: 10.1016/j.nmd.2004.01.009

27. Tamai M., Hashimoto T., Isobe T., Sato H., Doden T., Nakano T. Treatment of myasthenia gravis with dropped head: a report of 2 cases and review of the literature. Neuromuscul Disord. 2015;25 (5): 429–31. DOI: 10.1016/j.nmd.2015.01.014

28. Sih M., Soliven B., Mathenia N., Jacobsen J., Rezania K. Head-drop: A frequent feature of late-onset myasthenia gravis. Muscle Nerve. 2017; 56 (3): 441–444. DOI: 10.1002/mus.25526

29. Bardakov S. N., Polushin A. Yu., Tsargush V. A., Carlier P., Emelyantsev A. A., Lapin S. V., Moshnikova A. N., Skiba Ya.B., Belskikh A. N., Kulagin A. D. Anti-MuSK myasthenia gravis as a complication of allogeneic transplantation. Russian neurological journal. 2022; 27 (4): 44–54. (In Russ.). DOI: 10.30629/2658-7947-2022-27-4-44-54

30. Sanadze A. G., Sidnev D. V., Galkina O. I., Kasatkina L. F., Davydova T. V., Rudnichenko V. A. Myasthenic myopathy. S. S. Korsakov Journal of Neurology and Psychiatry. 2007; 107 (9): 11–16. (In Russ.).

31. Okubo S., Kainaga M., Tokushige S., Uchibori A., Oishi C., Hirano T., Ichikawa Y. Dropped head syndrome in anti-MuSK antibody-positive myasthenia gravis with possible concurrent axial myopathy. Clin. Exp. Neuroimmunol. 2024. DOI: 10.1111/cen3.12782

32. Mincă A., Mincă D. I., Calinoiu A. L., Gheorghiă V., Popescu C. C., Rusu A., Cristea A. M., Mincă D. G. Myasthenia Gravis Triggered by a COVID-19 Infection: A Case Report and Literature Review. Cureus. 2024; 16 (5): e59538. DOI: 10.7759/cureus.59538

33. Gekht B. M. Neuromidin in the treatment of diseases of the peripheral neuromotor apparatus. Doctor.Ru. 2003; 2: 3. (In Russ.).


Review

For citations:


Merkulov Yu.A., Plieva E.L., Merkulova D.M. Pain syndrome, “dropped head” and myasthenia gravis: a case report and literature review. Medical alphabet. 2024;(21):7-12. (In Russ.) https://doi.org/10.33667/2078-5631-2024-21-7-12

Views: 220


ISSN 2078-5631 (Print)
ISSN 2949-2807 (Online)