Top

The Egyptian Journal of Neurology, Psychiatry and Neurosurgery

Published in:

Open Access 01-12-2023 | Myasthenia Gravis | Case Report

Myasthenia gravis with achalasia secondary to thymoma: a case report and literature review

Authors: Nourelhoda A. Haridy, Eman M. Khedr, Asmaa M. Hasan, Ahmed A. Maghraby, Essam Abdelmohsen, AbdelHamid M. Aly

Published in: The Egyptian Journal of Neurology, Psychiatry and Neurosurgery | Issue 1/2023

Abstract

Background

Myasthenia gravis is an autoimmune neuromuscular junction disorder characterized by fatigable muscle weakness and autoantibodies. Frequent associations exist between myasthenia gravis and thymic abnormalities, including hyperplasia and thymoma. Several autoimmune illnesses have been identified to be associated with thymoma; however, a few case reports have linked thymoma and achalasia, and the underlying mechanism is unknown.

Case report

A 43-year-old man with thymoma-associated myasthenia gravis presented with dysphagia that was refractory to conventional treatment of myasthenia gravis. This dysphagia was challenging to diagnose even after multiple gastroenterology consults and upper endoscopy. The diagnosis of achalasia type II was established after a comprehensive evaluation, including upper endoscopy, barium swallow, and high-resolution esophageal manometry. The patient underwent elective pneumatic balloon dilatation, which successfully alleviated his dysphagia.

Conclusion

This case confirmed the association between myasthenia gravis secondary to thymoma and achalasia and showed how the diagnosis of achalasia was challenging. Awareness of this association is crucial for early diagnosis and treatment, improving affected patients’ quality of life.

Gilhus NE, Tzartos S, Evoli A, Palace J, Burns TM, Verschuuren JJGM. Myasthenia gravis. Nat Rev Dis Primers. 2019;5(1):30. https://doi.org/10.1038/s41572-019-0079-y.CrossRefPubMed

Damato V, Viegas S, Vincent A. Chapter 53—myasthenia gravis and related disorders. In: Rose NR, Mackay IR, editors. The Autoimmune Diseases (Sixth Edition). Academic Press; 2020. p. 1011–33.CrossRef

Scorsetti M, Leo F, Trama A, D’Angelillo R, Serpico D, Macerelli M, et al. Thymoma and thymic carcinomas. Crit Rev Oncol Hematol. 2016;99:332–50. https://doi.org/10.1016/j.critrevonc.2016.01.012.CrossRefPubMed

Detterbeck FC, Parsons AM. Thymic tumors. Ann Thorac Surg. 2004;77(5):1860–9. https://doi.org/10.1016/j.athoracsur.2003.10.001.CrossRefPubMed

Jamilloux Y, Frih H, Bernard C, Broussolle C, Petiot P, Girard N, et al. Thymoma and autoimmune diseases. Rev Med Interne. 2018;39(1):17–26. https://doi.org/10.1016/j.revmed.2017.03.003.CrossRefPubMed

Shelly S, Agmon-Levin N, Altman A, Shoenfeld Y. Thymoma and autoimmunity. Cell Mol Immunol. 2011;8(3):199–202. https://doi.org/10.1038/cmi.2010.74.CrossRefPubMedPubMedCentral

Demos NJ, Yadusky RJ, Timmes JJ, Poulos PP. Thymoma associated with megaesophagus. J Thorac Cardiovasc Surg. 1966;51(5):708–13. https://doi.org/10.1016/s0022-5223(19)43299-6.CrossRefPubMed

Ribolsi M, Andrisani G, Di Matteo FM, Cicala M. Achalasia, from diagnosis to treatment. Expert Rev Gastroenterol Hepatol. 2023;17(1):21–30. https://doi.org/10.1080/17474124.2022.2163236.CrossRefPubMed

Savarino E, Bhatia S, Roman S, Sifrim D, Tack J, Thompson SK, et al. Achalasia. Nat Rev Dis Primers. 2022;8(1):28. https://doi.org/10.1038/s41572-022-00356-8.CrossRefPubMed

10.

Pandolfino JE, Gawron AJ. Achalasia: a systematic review. JAMA. 2015;313(18):1841–52. https://doi.org/10.1001/jama.2015.2996.CrossRefPubMed

11.

Desuter G, Van Parijs V, Gardiner Q. Megaesophagus associated with myasthenia gravis: a rare condition in humans. B-ENT. 2015;11(1):63–6.PubMed

12.

Kempf J, Beckmann K, Kook PH. Achalasia-like disease with esophageal pressurization in a myasthenic dog. J Vet Intern Med. 2014;28(2):661–5. https://doi.org/10.1111/jvim.12329.CrossRefPubMedPubMedCentral

13.

Kornizky Y, Heller I, Isakov A, Shapira I, Topilsky M. Dysphagia with multiple autoimmune disease. Clin Rheumatol. 2000;19(4):321–3. https://doi.org/10.1007/s100670070055.CrossRefPubMed

14.

Rison RA. Reversible oropharyngeal dysphagia secondary to cricopharyngeal sphincter achalasia in a patient with myasthenia gravis: a case report. Cases J. 2009;2(1):1–3.CrossRef

15.

Vinod D. Autoimmunity strikes neuromuscular junction: myasthenia gravis & achalasia cardia. Indian J Med Res. 2020;152(Suppl 1):S146.PubMedPubMedCentral

16.

Kaminski HJ. Achalasia and myasthenia gravis in a patient with thymoma. Neurology. 1999;52(2):425.CrossRefPubMed

17.

Seabi M, Mokgoko D. A case of achalasia and thymoma-associated myasthenia gravis. South Afr Gastroenterol Rev. 2020;18(3):18–9.

18.

Prieto JL, Soria MJ, Peña JA, Infantes JM, Navas CJ, Martín L. Achalasia secondary to radiotherapy in a patient with thymoma and myasthenia gravis. Rev Esp Enferm Dig. 2001;93(2):125–6.PubMed

19.

Chen K, Li Y, Yang H. Poor responses and adverse outcomes of myasthenia gravis after thymectomy: predicting factors and immunological implications. J Autoimmun. 2022;132:102895. https://doi.org/10.1016/j.jaut.2022.102895.CrossRefPubMed

20.

Hsu H-S, Huang C-S, Huang B-S, Lee H-C, Kao K-P, Hsu W-H, et al. Thymoma is associated with relapse of symptoms after transsternal thymectomy for myasthenia gravis. Interact Cardiovasc Thorac Surg. 2006;5(1):42–6. https://doi.org/10.1510/icvts.2005.116616.CrossRefPubMed

21.

Xue L, Wang L, Dong J, Yuan Y, Fan H, Zhang Y, et al. Risk factors of myasthenic crisis after thymectomy for thymoma patients with myasthenia gravis†. Eur J Cardiothorac Surg. 2017;52(4):692–7. https://doi.org/10.1093/ejcts/ezx163.CrossRefPubMed

22.

Farrugia ME, Goodfellow JA. A practical approach to managing patients with myasthenia gravis—opinions and a review of the literature. Front Neurol. 2020. https://doi.org/10.3389/fneur.2020.00604.CrossRefPubMedPubMedCentral

23.

Geng Y, Zhang H, Wang Y. Risk factors of myasthenia crisis after thymectomy among myasthenia gravis patients: a meta-analysis. Medicine. 2020;99(1):e18622. https://doi.org/10.1097/md.0000000000018622.CrossRefPubMedPubMedCentral

24.

Trillenberg P, Thern J. Antibiotic-induced myasthenia worsening—an estimation of risk based on reporting frequency. J Antimicrob Chemother. 2020;75(11):3408–10. https://doi.org/10.1093/jac/dkaa298.CrossRefPubMed

25.

Jones SC, Sorbello A, Boucher RM. Fluoroquinolone-associated myasthenia gravis exacerbation: evaluation of postmarketing reports from the US FDA adverse event reporting system and a literature review. Drug Saf. 2011;34(10):839–47. https://doi.org/10.2165/11593110-000000000-00000.CrossRefPubMed

26.

Stavropoulos SN, Friedel D, Modayil R, Parkman HP. Diagnosis and management of esophageal achalasia. BMJ. 2016;354:i2785. https://doi.org/10.1136/bmj.i2785.CrossRefPubMed

27.

Mari A, Patel K, Mahamid M, Khoury T, Pesce M. Achalasia: insights into diagnostic and therapeutic advances for an ancient disease. Rambam Maimonides Med J. 2019. https://doi.org/10.5041/RMMJ.10361.CrossRefPubMedPubMedCentral

28.

Storch WB, Eckardt VF, Wienbeck M, Eberl T, Auer PG, Hecker A, et al. Autoantibodies to Auerbach’s plexus in achalasia. Cell Mol Biol. 1995;41(8):1033–8.PubMed

29.

Patel DA, Lappas BM, Vaezi MF. An overview of achalasia and its subtypes. Gastroenterol Hepatol (N Y). 2017;13(7):411–21.PubMed

30.

Rieder E, Fernandez-Becker NQ, Sarosiek J, Guillaume A, Azagury DE, Clarke JO. Achalasia: physiology and diagnosis. Ann N Y Acad Sci. 2020;1482(1):85–94. https://doi.org/10.1111/nyas.14510.CrossRefPubMed

31.

Hasner E. Thymomas. Acta Chir Scand. 1963;126:58–65.PubMed

32.

Ferguson DD, Foxx-Orenstein AE. Eosinophilic esophagitis: an update. Dis Esophagus. 2007;20(1):2–8. https://doi.org/10.1111/j.1442-2050.2007.00649.x.CrossRefPubMed

33.

Albinsson Högberg S. A study of eosinophils–from the human thymus to eosinophilic esophagitis [Doctoral thesis]: University of Gothenburg; 2022.

Title: Myasthenia gravis with achalasia secondary to thymoma: a case report and literature review
Authors: Nourelhoda A. Haridy
Eman M. Khedr
Asmaa M. Hasan
Ahmed A. Maghraby
Essam Abdelmohsen
AbdelHamid M. Aly
Publication date: 01-12-2023
Publisher: Springer Berlin Heidelberg
Keywords: Myasthenia Gravis
Thymoma
Thymoma
Achalasia
Dysphagia
Dysphagia
Dysphagia
Thymectomy
Thymectomy
Published in: The Egyptian Journal of Neurology, Psychiatry and Neurosurgery / Issue 1/2023
Electronic ISSN: 1687-8329
DOI: https://doi.org/10.1186/s41983-023-00636-4

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Myasthenia gravis with achalasia secondary to thymoma: a case report and literature review

Abstract

Background

Case report

Conclusion

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Background

Case report

Conclusion

Please log in to get access to this content

Other articles of this Issue 1/2023

Assessment of the role of telemedicine in the outcome of multiple sclerosis patients

The frequency and impact of tramadol addiction on acute aneurysmal subarachnoid hemorrhage: cross-sectional multicenter study

Idiopathic intracranial hypertension: from concise history to current management

Emerging hallmark of gliomas microenvironment in evading immunity: a basic concept

Neurotrophic factors-based therapeutic strategies in the spinal cord injury: an overview of recent preclinical studies in rodent models

Determinants of quality of life in Nigerian female patients with epilepsy on carbamazepine and levetiracetam monotherapy