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Önceden CIDP tanısı almış bir hastada ortaya çıkan miyastenia gravis: Örtüşen otoimmün hastalıklarda klinik zorluklar

Yıl 2025, Cilt: 64 Sayı: 4, 716 - 720, 08.12.2025

Şeyma Aykaç , Abdullah Arı , Birgül Dere , Yaprak Seçil , Ayşe Nur Yüceyar

https://doi.org/10.19161/etd.1736794

Öz

Kronik inflamatuar demiyelinizan polinöropati (CIDP) ve miyastenia gravis (MG), nadiren birlikte görülen iki farklı otoimmün nöromüsküler hastalıktır. Stabil CIDP’si olan 52 yaşındaki erkek hasta, ilerleyici alt ekstremite ve fasyal güçsüzlükle başvurdu. Elektrofizyolojik incelemelerde nöromüsküler kavşak disfonksiyonu saptandı ve asetilkolin reseptör antikorlarının yüksek olması MG tanısını doğruladı. Piridostigmin, intravenöz immünglobulin ve rituksimab tedavisiyle klinik stabilite sağlandı. Bu olgu, CIDP’li hastalarda yeni veya atipik semptomlarda eşlik eden otoimmün hastalıkların düşünülmesinin önemini vurgulamaktadır. Elektrofizyolojik ve serolojik testlerle erken tanı, eşzamanlı CIDP ve MG yönetiminde etkilidir.

Anahtar Kelimeler

CIDP myasthenia gravis, tanı güçlüğü immunsupresyon rituximab

Kaynakça

  • Mathey EK, Park SB, Hughes RA, et al. Chronic inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype. J Neurol Neurosurg Psychiatry. 2015;86:973- 985. doi:10.1136/jnnp-2014.
  • Meriggioli MN, Sanders DB. Autoimmune myasthenia gravis: emerging clinical and biological heterogeneity. Lancet Neurol. 2009;8:475-490.doi:10.1016/S1474-4422(09)70063-8.
  • Sanders DB, Wolfe GI, Benatar M, et al. International consensus guidance for management of myasthenia gravis: Executive summary. Neurology. 2016;87:419-425. doi:10.1212/WNL.0000000000002790.
  • Weinreb H, Klein J, Kupersmith M. Ocular myasthenia gravis and chronic inflammatory polyradiculoneuropathy. N Y State J Med 1986;86:439 – 42.
  • Inatsu A, Ohi T, Shioya K, Matsukura S. A case of myasthenia gravis occurring in the period of remission of chronic inflammatory demyelinating polyradiculoneuropathy. Rinsho Shinkeigaku 1992;32: 878 – 879.
  • Patwa HS, Fecko JF, Goldstein JM. Concurrent myasthenia gravis and chronic inflammatory demyelinating polyneuropathy. Muscle Nerve. 1996;19:1059-1060.
  • Sempere AP, Claveria LE, Cru4Martine4 A, et al. Myasthenia gravis and chronic inflammatory demyelinating polyneuropathy. Eur J Neurol. 19964;3:397398.
  • Kimura K, Nezu A, Kimura S, et al. A case of myasthenia gravis in childhood associated with chronic inflammatory demyelinating polyradiculoneuropathy. Neuropediatrics. 1998;29:108-112. doi:10.1055/s-2007-973544.
  • Tam DA, Chalmers A. Chronic inflammatory demyelinating polyneuropathy and myasthenia gravis. J Child Neurol. 1999;14:478-479. doi:10.1177/088307389901400715.
  • 1Shankar V, Sayeed ZA. Myasthenia gravis with chronic inflammatory demyelinating polyneuropathy - A case report. Neurol India. 1999;47:78-79.
  • Magot A, Wiertlewski S, Boutoleau C, et al. Ptosis et troubles de la mastication révélateurs d'une association myasthénie et polyradiculonévrite chronique [Ptosis and mastication disorders revealing concurrent myasthenia gravis and chronic polyradiculoneuritis]. Rev Neurol (Paris). 2002;158:741- 743.
  • Mori M, Kuwabara S, Nemoto Y, et al. Concomitant chronic inflammatory demyelinating polyneuropathy and myasthenia gravis following cytomegalovirus infection. J Neurol Sci. 2006;240:103-106. doi:10.1016/j.jns.2005.08.013.
  • Quan W, Xia J, Tong Q, et al. Myasthenia gravis and chronic inflammatory demyelinating polyneuropathy in the same patient - a case report. Int J Neurosci. 2018;128:570-572. doi:10.1080/00207454.2017.1398160.
  • Sumit D, Jyoti BT, Musharraf H, et al. Myasthenia Gravis and Chronic Inflammatory Demyelinating Polyradiculoneuropathy in the Same Patient: A Clinical Rarity. International Journal of Neurology and Neurosurgery. 2018;10:277-279. doi: http://dx.doi.org/10.21088/i5nns.0975.0223.10318.23.
  • Jiang N, Kazamel M. Concomitant Chronic Inflammatory Demyelinating Polyradiculoneuropathy and Myasthenia Gravis-A Two-Case Report. Acta Scientific Neurology. 2020;3:117-120.
  • Martić V, Fejzić E, Marić N. Myasthenia Gravis and Chronic Inflammatory Demyelinating Polyradiculoneuropathy in a Patient with Recurrent Thymoma. SrpSki medicinSki čaSopiS LekarSke komore.2023;4:188-192.
  • Hickman SJ, Allen JA, Baisre A, et al. Neuro-ophthalmological Complications of Chronic Inflammatory Demyelinating Polyradiculoneuropathy. Neuroophthalmology. 2013;37:146-156. doi:10.3109/01658107.2013.809459.
  • Grüter T, Motte J, Fisse AL, et al. Pathological spontaneous activity as a prognostic marker in chronic inflammatory demyelinating polyneuropathy. Eur J Neurol. 2020;27:2595-2603. doi:10.1111/ene.14476.
  • Mausberg AK, Dorok M, Stettner M, Muller M, Hartung HP, Dehmel T, Warnke C, Meyer Zu Horste G, Kieseier BC. Recovery of the T-cell repertoire in CIDP by IV immunoglobulins. Neurology. 2013;80:296–303. doi: 10.1212/WNL.0b013e31827debad].
  • Dalakas MC. Pathogenesis of immune-mediated neuropathies. Biochim Biophys Acta. 2015;1852:658–666. doi: 10.1016/j.bbadis.2014.06.013.].
  • Dalakas MC. Future perspectives in target-specific immunotherapies of myasthenia gravis. Ther Adv Neurol Disord. 2015;8:316–327. doi: 10.1177/1756285615605700.].
  • Krampfl K, Mohammadi B, Buchwald B, et al. IgG from patients with Guillain-Barré syndrome interact with nicotinic acetylcholine receptor channels. Muscle Nerve. 2003;27:435-441. doi:10.1002/mus.10349.
  • Shevach EM. CD4+ CD25+ suppressor T cells: more questions than answers. Nat Rev Immunol. 2002;2: 389-400
  • Sawhney S, Asranna A, Sureshbabu S, et al. Malignant thymoma and chronic inflammatory demyelinating neuropathy. Muscle Nerve. 2021;63:E10-E13. doi:10.1002/mus.27132.
  • Mathew T, Thomas K, K John S, et al. Effective Early Treatment of AChR Antibody-Positive Myasthenia Gravis with Rituximab; the Experience from a Neuroimmunology Clinic in a Developing Country. J Cent Nerv Syst Dis. 2021;13:11795735211016080. doi:10.1177/11795735211016080
  • Piehl F, Eriksson-Dufva A, Budzianowska A, et al. Efficacy and Safety of Rituximab for New-Onset Generalized Myasthenia Gravis: The RINOMAX Randomized Clinical Trial. JAMA Neurol. 2022;79(11):1105-1112. doi:10.1001/jamaneurol.2022.2887
  • Roux T, Debs R, Maisonobe T, et al. Rituximab in chronic inflammatory demyelinating polyradiculoneuropathy with associated diseases. J Peripher Nerv Syst. 2018;23:235-240. d Du Y, Yan
  • Du Y, Yan Q, Li C, et al. Efficacy and safety of combined low-dose rituximab regimen for chronic inflammatory demyelinating polyradiculoneuropathy. Ann Clin Transl Neurol. 2025;12:180-191. doi:10.1002/acn3.52270oi:10.1111/jns.12287.

A case of myasthenia gravis emerging after prior diagnosis of CIDP: Clinical challenges in overlapping autoimmune disorders

Yıl 2025, Cilt: 64 Sayı: 4, 716 - 720, 08.12.2025

Şeyma Aykaç , Abdullah Arı , Birgül Dere , Yaprak Seçil , Ayşe Nur Yüceyar

https://doi.org/10.19161/etd.1736794

Öz

Chronic inflammatory demyelinating polyneuropathy (CIDP) and myasthenia gravis (MG) are distinct autoimmune neuromuscular disorders that rarely coexist. We present a 52-year-old man with stable CIDP who developed progressive lower limb weakness and facial involvement. Electrophysiological studies revealed neuromuscular junction dysfunction, and elevated acetylcholine receptor antibodies confirmed MG. Treatment with pyridostigmine, intravenous immunoglobulin, and rituximab resulted in clinical stabilization. This case shows the importance of considering overlapping autoimmune disorders in CIDP patients with new or atypical symptoms. Early recognition using electrophysiological and serological tests is important for accurate diagnosis and effective management of coexisting CIDP and MG.

Anahtar Kelimeler

CIDP myasthenia gravis diagnostic challenge immunosuppression rituximab.

Kaynakça

  • Mathey EK, Park SB, Hughes RA, et al. Chronic inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype. J Neurol Neurosurg Psychiatry. 2015;86:973- 985. doi:10.1136/jnnp-2014.
  • Meriggioli MN, Sanders DB. Autoimmune myasthenia gravis: emerging clinical and biological heterogeneity. Lancet Neurol. 2009;8:475-490.doi:10.1016/S1474-4422(09)70063-8.
  • Sanders DB, Wolfe GI, Benatar M, et al. International consensus guidance for management of myasthenia gravis: Executive summary. Neurology. 2016;87:419-425. doi:10.1212/WNL.0000000000002790.
  • Weinreb H, Klein J, Kupersmith M. Ocular myasthenia gravis and chronic inflammatory polyradiculoneuropathy. N Y State J Med 1986;86:439 – 42.
  • Inatsu A, Ohi T, Shioya K, Matsukura S. A case of myasthenia gravis occurring in the period of remission of chronic inflammatory demyelinating polyradiculoneuropathy. Rinsho Shinkeigaku 1992;32: 878 – 879.
  • Patwa HS, Fecko JF, Goldstein JM. Concurrent myasthenia gravis and chronic inflammatory demyelinating polyneuropathy. Muscle Nerve. 1996;19:1059-1060.
  • Sempere AP, Claveria LE, Cru4Martine4 A, et al. Myasthenia gravis and chronic inflammatory demyelinating polyneuropathy. Eur J Neurol. 19964;3:397398.
  • Kimura K, Nezu A, Kimura S, et al. A case of myasthenia gravis in childhood associated with chronic inflammatory demyelinating polyradiculoneuropathy. Neuropediatrics. 1998;29:108-112. doi:10.1055/s-2007-973544.
  • Tam DA, Chalmers A. Chronic inflammatory demyelinating polyneuropathy and myasthenia gravis. J Child Neurol. 1999;14:478-479. doi:10.1177/088307389901400715.
  • 1Shankar V, Sayeed ZA. Myasthenia gravis with chronic inflammatory demyelinating polyneuropathy - A case report. Neurol India. 1999;47:78-79.
  • Magot A, Wiertlewski S, Boutoleau C, et al. Ptosis et troubles de la mastication révélateurs d'une association myasthénie et polyradiculonévrite chronique [Ptosis and mastication disorders revealing concurrent myasthenia gravis and chronic polyradiculoneuritis]. Rev Neurol (Paris). 2002;158:741- 743.
  • Mori M, Kuwabara S, Nemoto Y, et al. Concomitant chronic inflammatory demyelinating polyneuropathy and myasthenia gravis following cytomegalovirus infection. J Neurol Sci. 2006;240:103-106. doi:10.1016/j.jns.2005.08.013.
  • Quan W, Xia J, Tong Q, et al. Myasthenia gravis and chronic inflammatory demyelinating polyneuropathy in the same patient - a case report. Int J Neurosci. 2018;128:570-572. doi:10.1080/00207454.2017.1398160.
  • Sumit D, Jyoti BT, Musharraf H, et al. Myasthenia Gravis and Chronic Inflammatory Demyelinating Polyradiculoneuropathy in the Same Patient: A Clinical Rarity. International Journal of Neurology and Neurosurgery. 2018;10:277-279. doi: http://dx.doi.org/10.21088/i5nns.0975.0223.10318.23.
  • Jiang N, Kazamel M. Concomitant Chronic Inflammatory Demyelinating Polyradiculoneuropathy and Myasthenia Gravis-A Two-Case Report. Acta Scientific Neurology. 2020;3:117-120.
  • Martić V, Fejzić E, Marić N. Myasthenia Gravis and Chronic Inflammatory Demyelinating Polyradiculoneuropathy in a Patient with Recurrent Thymoma. SrpSki medicinSki čaSopiS LekarSke komore.2023;4:188-192.
  • Hickman SJ, Allen JA, Baisre A, et al. Neuro-ophthalmological Complications of Chronic Inflammatory Demyelinating Polyradiculoneuropathy. Neuroophthalmology. 2013;37:146-156. doi:10.3109/01658107.2013.809459.
  • Grüter T, Motte J, Fisse AL, et al. Pathological spontaneous activity as a prognostic marker in chronic inflammatory demyelinating polyneuropathy. Eur J Neurol. 2020;27:2595-2603. doi:10.1111/ene.14476.
  • Mausberg AK, Dorok M, Stettner M, Muller M, Hartung HP, Dehmel T, Warnke C, Meyer Zu Horste G, Kieseier BC. Recovery of the T-cell repertoire in CIDP by IV immunoglobulins. Neurology. 2013;80:296–303. doi: 10.1212/WNL.0b013e31827debad].
  • Dalakas MC. Pathogenesis of immune-mediated neuropathies. Biochim Biophys Acta. 2015;1852:658–666. doi: 10.1016/j.bbadis.2014.06.013.].
  • Dalakas MC. Future perspectives in target-specific immunotherapies of myasthenia gravis. Ther Adv Neurol Disord. 2015;8:316–327. doi: 10.1177/1756285615605700.].
  • Krampfl K, Mohammadi B, Buchwald B, et al. IgG from patients with Guillain-Barré syndrome interact with nicotinic acetylcholine receptor channels. Muscle Nerve. 2003;27:435-441. doi:10.1002/mus.10349.
  • Shevach EM. CD4+ CD25+ suppressor T cells: more questions than answers. Nat Rev Immunol. 2002;2: 389-400
  • Sawhney S, Asranna A, Sureshbabu S, et al. Malignant thymoma and chronic inflammatory demyelinating neuropathy. Muscle Nerve. 2021;63:E10-E13. doi:10.1002/mus.27132.
  • Mathew T, Thomas K, K John S, et al. Effective Early Treatment of AChR Antibody-Positive Myasthenia Gravis with Rituximab; the Experience from a Neuroimmunology Clinic in a Developing Country. J Cent Nerv Syst Dis. 2021;13:11795735211016080. doi:10.1177/11795735211016080
  • Piehl F, Eriksson-Dufva A, Budzianowska A, et al. Efficacy and Safety of Rituximab for New-Onset Generalized Myasthenia Gravis: The RINOMAX Randomized Clinical Trial. JAMA Neurol. 2022;79(11):1105-1112. doi:10.1001/jamaneurol.2022.2887
  • Roux T, Debs R, Maisonobe T, et al. Rituximab in chronic inflammatory demyelinating polyradiculoneuropathy with associated diseases. J Peripher Nerv Syst. 2018;23:235-240. d Du Y, Yan
  • Du Y, Yan Q, Li C, et al. Efficacy and safety of combined low-dose rituximab regimen for chronic inflammatory demyelinating polyradiculoneuropathy. Ann Clin Transl Neurol. 2025;12:180-191. doi:10.1002/acn3.52270oi:10.1111/jns.12287.
Toplam 28 adet kaynakça vardır.

Ayrıntılar

Birincil Dil İngilizce
Konular Nöroloji ve Nöromüsküler Hastalıklar
Bölüm Olgu Sunumu
Yazarlar

Şeyma Aykaç 0000-0003-3994-0019

Abdullah Arı 0009-0006-1995-210X

Birgül Dere 0000-0002-0565-4922

Yaprak Seçil 0000-0002-8283-9680

Ayşe Nur Yüceyar 0000-0003-4590-6423

Yayımlanma Tarihi 8 Aralık 2025
Gönderilme Tarihi 11 Temmuz 2025
Kabul Tarihi 30 Eylül 2025
Yayımlandığı Sayı Yıl 2025 Cilt: 64 Sayı: 4

Kaynak Göster

Vancouver Aykaç Ş, Arı A, Dere B, Seçil Y, Yüceyar AN. A case of myasthenia gravis emerging after prior diagnosis of CIDP: Clinical challenges in overlapping autoimmune disorders. ETD. 2025;64(4):716-20.
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