Current management of myasthenia gravis in Belgium: a single-center experience.

Publisher: Springer Country of Publication: Italy NLM ID: 0247035 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 2240-2993 (Electronic) Linking ISSN: 03009009 NLM ISO Abbreviation: Acta Neurol Belg Subsets: MEDLINE

Publication: 2012- : Milan : Springer
Original Publication: 1970-2011: Bruxelles : Acta Medica Belgica,

Myasthenia Gravis*/diagnosis ; Myasthenia Gravis*/drug therapy ; Myasthenia Gravis*/epidemiology ; Blepharoptosis*/drug therapy; Humans ; Female ; Male ; Belgium ; Pyridostigmine Bromide/therapeutic use ; Diplopia/drug therapy

Introduction: As new treatments are becoming available for patients with myasthenia gravis (MG), it is worth reflecting on the actual status of MG treatment to determine which patients would most likely benefit from the new treatments.
Methods: We reviewed the clinical files of all MG patients seen at the Department of Neurology of the Antwerp University Hospital during the years 2019, 2020 and 2021.
Results: 163 patients were included. Age at diagnosis varied from the first to the eighth decades, with a peak of incidence from 60 to 70 years for both genders, and an additional peak from 20 to 30 years in women. Diplopia and ptosis were by far the most common onset symptom. At maximum disease severity, 24% of the patients still had purely ocular symptoms and 4% needed mechanical ventilation. 97% of the patients received a treatment with pyridostigmine and 68% with corticosteroids, often in combination with immunosuppressants. More than half reported side effects. At the latest visit, 50% of the patients were symptom-free. Also, half of the symptomatic patients were fulltime at work or retired with no or mild limitations in daily living. The remaining patients were working part-time, on sick leave, or retired with severe limitations.
Discussion and Conclusion: The majority of MG patients are doing well with currently available treatments, but often at the cost of side effects in the short and in the long term. A significant group is in need of better treatments.
(© 2023. The Author(s) under exclusive licence to Belgian Neurological Society.)

Verschuuren JJ, Palace J, Murai H, Tannemaat MR, Kaminski HJ, Bril V (2022) Advances and ongoing research in the treatment of autoimmune neuromuscular junction disorders. Lancet Neurol 21(2):189–202. https://doi.org/10.1016/S1474-4422(21)00463-4. (PMID: 10.1016/S1474-4422(21)00463-435065041)
Punga AR, Maddison P, Heckmann JM, Guptill JT, Evoli A (2022) Epidemiology, diagnostics, and biomarkers of autoimmune neuromuscular junction disorders. Lancet Neurol 21(2):176–188. https://doi.org/10.1016/S1474-4422(21)00297-0. (PMID: 10.1016/S1474-4422(21)00297-035065040)
Westerberg E, Punga AR (2020) Mortality rates and causes of death in Swedish Myasthenia Gravis patients. Neuromuscul Disord 30(10):815–824. https://doi.org/10.1016/j.nmd.2020.08.355. (PMID: 10.1016/j.nmd.2020.08.35532962871)
Cortés-Vicente E et al (2022) Drug-refractory myasthenia gravis: Clinical characteristics, treatments, and outcome. Ann Clin Transl Neurol 9(2):122–131. https://doi.org/10.1002/acn3.51492. (PMID: 10.1002/acn3.51492350801538862423)
Jaretzki A et al (2000) Myasthenia gravis: recommendations for clinical research standards. Neurology 55:16–23. https://doi.org/10.1212/WNL.55.1.16. (PMID: 10.1212/WNL.55.1.1610891897)
Vincent A et al (2012) Antibodies identified by cell-based assays in myasthenia gravis and associated diseases. Ann NY Acad Sci 1274:92–98. https://doi.org/10.1111/j.1749-6632.2012.06789.x. (PMID: 10.1111/j.1749-6632.2012.06789.x23252902)
Mercelis R, Merckaert V (2011) Diagnostic utility of stimulated single-fiber electromyography of the orbicularis oculi muscle in patients with suspected ocular myasthenia. Muscle Nerve 43(2):168–170. https://doi.org/10.1002/mus.21853. (PMID: 10.1002/mus.2185321254079)
Sanders DB, Raja SM, Guptill JT, Hobson-Webb LD, Juel VC, Massey JM (2021) The Duke myasthenia gravis clinic registry: I. Description and demographics. Muscle Nerve 63(2):209–216. https://doi.org/10.1002/mus.27120. (PMID: 10.1002/mus.2712033205437)
Evoli A, Iorio R (2020) Controversies in Ocular Myasthenia Gravis. Front Neurol. https://doi.org/10.3389/fneur.2020.605902. (PMID: 10.3389/fneur.2020.605902333293687734350)
Lehnerer S et al (2021) Burden of disease in myasthenia gravis: taking the patient’s perspective. J Neurol 269(6):3050. https://doi.org/10.1007/s00415-021-10891-1. (PMID: 10.1007/s00415-021-10891-1348001679120127)
Muppidi S, Silvestri NJ, Tan R, Riggs K, Leighton T, Phillips GA (2022) Utilization of MG-ADL in myasthenia gravis clinical research and care. Muscle Nerve. https://doi.org/10.1002/mus.27476. (PMID: 10.1002/mus.27476349894279302997)
Barnett C, Bril V, Kapral M, Kulkarni AV, Davis AM (2017) Myasthenia gravis impairment index. Neurology 89(23):2357–2364. https://doi.org/10.1212/WNL.0000000000004676. (PMID: 10.1212/WNL.0000000000004676291012745719924)
Abraham A, Breiner A, Barnett C, Katzberg HD, Bril V (2017) The utility of a single simple question in the evaluation of patients with myasthenia gravis. Muscle Nerve. https://doi.org/10.1002/mus.25720. (PMID: 10.1002/mus.2572028590545)
Mendoza M, Tran C, Bril V, Katzberg HD, Barnett C (2020) Patient-acceptable symptom states in myasthenia gravis. Neurology 95(12):e1617–e1628. https://doi.org/10.1212/WNL.0000000000010574. (PMID: 10.1212/WNL.000000000001057432759200)
Anil R et al (2020) Exploring outcomes and characteristics of myasthenia gravis: Rationale, aims and design of registry – The EXPLORE-MG registry. J Neurol Sci 414:116830. https://doi.org/10.1016/j.jns.2020.116830. (PMID: 10.1016/j.jns.2020.11683032388060)
Imai T et al (2014) Oral corticosteroid therapy and present disease status in myasthenia gravis. Muscle Nerve. https://doi.org/10.1002/mus.24438. (PMID: 10.1002/mus.2443824741683)
Lotan I, Hellmann MA, Wilf-Yarkoni A, Steiner I (2020) Exacerbation of myasthenia gravis following corticosteroid treatment: what is the evidence? A systematic review. J Neurol. https://doi.org/10.1007/s00415-020-10264-0. (PMID: 10.1007/s00415-020-10264-033064188)
Sanders DB et al (2016) International consensus guidance for management of myasthenia gravis: Executive summary. Neurology 87(4):419–425. https://doi.org/10.1212/WNL.0000000000002790. (PMID: 10.1212/WNL.0000000000002790273583334977114)
Gotterer Do L, Li Y (2016) Maintenance immunosuppression in myasthenia gravis. J Neurol Sci 369:294–304. https://doi.org/10.1016/j.jns.2016.08.057. (PMID: 10.1016/j.jns.2016.08.057)
Jack KL, Koopman WJ, Hulley D, Nicolle MW (2016) A Review of Azathioprine-Associated hepatotoxicity and myelosuppression in myasthenia gravis. J Clin Neuromuscul Dis 18(1):12–20. https://doi.org/10.1097/CND.0000000000000133. (PMID: 10.1097/CND.000000000000013327552384)
Lorenzoni PJ, Kay CSK, Zanlorenzi MF, Ducci RDP, Werneck LC, Scola RH (2020) Myasthenia gravis and azathioprine treatment: Adverse events related to thiopurine S-methyl-transferase (TPMT) polymorphisms. J Neurol Sci 412:116734. https://doi.org/10.1016/j.jns.2020.116734. (PMID: 10.1016/j.jns.2020.11673432070863)
Pedersen EG et al (2014) Risk of non-melanoma skin cancer in myasthenia patients treated with azathioprine. Eur J Neurol 21(3):454–458. https://doi.org/10.1111/ene.12329. (PMID: 10.1111/ene.1232924330255)
Sanders DB et al (2008) An international, phase III, randomized trial of mycophenolate mofetil in myasthenia gravis. Neurology 71(6):400–406. https://doi.org/10.1212/01.wnl.0000312374.95186.cc. (PMID: 10.1212/01.wnl.0000312374.95186.cc18434638)
Hehir MK et al (2017) Rituximab as treatment for anti-MuSK myasthenia gravis. Neurology 89(10):1069–1077. https://doi.org/10.1212/WNL.0000000000004341. (PMID: 10.1212/WNL.000000000000434128801338)
Nowak RJ et al (2021) Phase 2 Trial of rituximab in acetylcholine receptor antibody-positive generalized myasthenia gravis: The BeatMG Study. Neurology. https://doi.org/10.1212/wnl.0000000000013121. (PMID: 10.1212/wnl.000000000001312134857535)
Guptill JT et al (2013) A retrospective study of complications of therapeutic plasma exchange in myasthenia. Muscle Nerve 47(2):170–176. https://doi.org/10.1002/mus.23508. (PMID: 10.1002/mus.2350823168720)
Jayam Trouth A, Dabi A, Solieman N, Kurukumbi M, Kalyanam J (2012) Myasthenia gravis: a review. Autoimmun Dis 1(1):10. https://doi.org/10.1155/2012/874680. (PMID: 10.1155/2012/874680)
Marx A et al (2015) Thymoma related myasthenia gravis in humans and potential animal models. Exp Neurol. https://doi.org/10.1016/j.expneurol.2015.02.010. (PMID: 10.1016/j.expneurol.2015.02.01026708556)
Wolfe GI et al (2016) Randomized trial of thymectomy in myasthenia gravis. N Engl J Med 375(6):511–522. https://doi.org/10.1056/NEJMoa1602489. (PMID: 10.1056/NEJMoa1602489275091005189669)
Maggi L et al (2008) Thymoma-associated myasthenia gravis: outcome, clinical and pathological correlations in 197 patients on a 20-year experience. J Neuroimmunol 201–202:237–244. https://doi.org/10.1016/j.jneuroim.2008.07.012. (PMID: 10.1016/j.jneuroim.2008.07.01218722676)
Sanders DB, Stålberg EV (1996) AAEM minimonograph 25: Single-fiber electromyography. Muscle Nerve 19(9):1069–1083. https://doi.org/10.1002/(SICI)1097-4598(199609)19. (PMID: 10.1002/(SICI)1097-4598(199609)198761262)
Padua L, Stalberg E, LoMonaco M, Evoli A, Batocchi A, Tonali P (2000) SFEMG in ocular myasthenia gravis diagnosis. Clin Neurophysiol 111(7):1203–1207. (PMID: 10.1016/S1388-2457(00)00307-210880794)

Keywords: Autoantibodies; Electromyography; Myasthenia gravis; Treatment

KVI301NA53 (Pyridostigmine Bromide)

Date Created: 20230119 Date Completed: 20230428 Latest Revision: 20230428

20230428

PMC9851893

10.1007/s13760-023-02187-0

36658451