r-n-jРоссийский неврологический журналRussian neurological journal2658-79472686-7192МИА10.30629/2658-7947-2025-30-6-47-55r-n-j-777Research ArticleИССЛЕДОВАНИЯ И КЛИНИЧЕСКИЕ НАБЛЮДЕНИЯCLINICAL RESEARCHES AND CASE REPORTSПреимущественно проксимальный моторный вариант хронической воспалительной демиелинизирующей полирадикулоневропатииPredominantly proximal motor variant of chronic inflammatory demyelinating polyradiculoneuropathyhttps://orcid.org/0000-0002-7924-3405ГришинаД. А.GrishinaD. A.

Москва

Moscow

dgrishina82@gmail.comhttps://orcid.org/0000-0003-3956-6362СупоневаН. А.SuponevaN. A.

Москва

Moscow

https://orcid.org/0000-0001-8070-7644ФедотоваЕ. Ю.FedotovaE. Yu.

Москва

Moscow

https://orcid.org/0000-0003-3179-7668НужныйЕ. П.NuzhnyiE. P.

Москва

Moscow

https://orcid.org/0000-0002-9093-344XМорозоваС. Н.MorozovaS. N.

Москва

Moscow

ФГБНУ «Российский центр неврологии и нейронаук»РоссияRussian Сenter of Neurology and NeurosciencesRussian Federation2025120220263064755Copyright © Гришина Д.А., Супонева Н.А., Федотова Е.Ю., Нужный Е.П., Морозова С.Н., 20262026Гришина Д.А., Супонева Н.А., Федотова Е.Ю., Нужный Е.П., Морозова С.Н.Grishina D.A., Suponeva N.A., Fedotova E.Y., Nuzhnyi E.P., Morozova S.N.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.r-n-j.com/jour/article/view/777

   Гетерогенность клинической и нейрофизиологической картины при хронической воспалительной демиелинизирующей полирадикулоневропатии (ХВДП), широкий спектр ХВДП-подобных заболеваний, а также отсутствие биомаркеров значительно затрудняют своевременную диагностику болезни. В статье дается подробное описание особенностей клинической, нейрофизиологической и нейровизуализационной картины преимущественно проксимального моторного варианта ХВДП, проанализированы причины изначально ошибочной диагностики первично-мышечного заболевания у пациентки.

   The heterogeneity of the clinical and neurophysiological presentation of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), the wide spectrum of conditions mimicking CIDP, and the absence of specific biomarkers significantly complicate timely diagnosis. The article provides a detailed description of the clinical, neurophysiological, and neuroimaging features of predominantly proximal motor CIDP, as well as a review of the causes of initial misdiagnosis of a primary neuromuscular disease in the patient.

хроническая воспалительная демиелинизирующая полирадикулоневропатиянетипичный вариантмоторный вариантмиопатияэлектронейромиографияМРТ сплетенийдифференциальный диагнозchronic inflammatory demyelinating polyradiculoneuropathyCIDPatypical variantmotor variantmyopathyelectroneuromyographyplexus MRIdifferential diagnosisИсследование выполнено без финансовой поддержкиThe study had no sponsorshipReferencesStino AM, Naddaf E, Dyck PJ, Dyck PJB. Chronic infl ammatory demyelinating polyradiculoneuropathy-Diagnostic pitfalls and treatment approach. Muscle Nerve. 2021;63(2):157–169. doi: 10.1002/mus.27046Stino AM, Naddaf E, Dyck PJ, Dyck PJB. Chronic infl ammatory demyelinating polyradiculoneuropathy-Diagnostic pitfalls and treatment approach. Muscle Nerve. 2021;63(2):157–169. doi: 10.1002/mus.27046Van den Bergh PYK, van Doorn PA, Hadden RDM, Avau B, Vankrunkelsven P, Allen JA, Attarian S, Blomkwist-Markens PH, Corndlath DR, Eftimov F, Goedee HS, Harbo T, Kuwabara S, Lewis RA, Lunn MP, Nobile-Orazio E, Querol L, Rajebally YA, Sommer C, Topaloglu HA. European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint Task Force-Second revision. Journal of the Peripheral Nervous System. 2021;26(3):242–268. doi: 10.1111/jns.12455Van den Bergh PYK, van Doorn PA, Hadden RDM, Avau B, Vankrunkelsven P, Allen JA, Attarian S, Blomkwist-Markens PH, Corndlath DR, Eftimov F, Goedee HS, Harbo T, Kuwabara S, Lewis RA, Lunn MP, Nobile-Orazio E, Querol L, Rajebally YA, Sommer C, Topaloglu HA. European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint Task Force-Second revision. Journal of the Peripheral Nervous System. 2021;26(3):242–268. doi: 10.1111/jns.12455Eftimov F, Lucke IM, Querol LA, Rajabally YA, Verhamme C. Diagnostic challenges in chronic inflammatory demyelinating polyradiculoneuropathy. Brain. 2020;5;143(11):3214–3224. doi: 10.1093/brain/awaa265Eftimov F, Lucke IM, Querol LA, Rajabally YA, Verhamme C. Diagnostic challenges in chronic inflammatory demyelinating polyradiculoneuropathy. Brain. 2020;5;143(11):3214–3224. doi: 10.1093/brain/awaa265Allen JA, Gorson KC, Gelinas D. Challenges in the diagnosis of chronic infl ammatory demyelinating polyneuropathy. Brain Behav. 2018;7;8(3):e00932. doi: 10.1002/brb3.932Allen JA, Gorson KC, Gelinas D. Challenges in the diagnosis of chronic infl ammatory demyelinating polyneuropathy. Brain Behav. 2018;7;8(3):e00932. doi: 10.1002/brb3.932Grishina DA, Suponeva NA, Arestova AS, Melnik EA, Tumilovich TA, Sinkova VV, Piradov MA. Chronic inflammatory demyelinating polyradiculoneuropathy overdiagnosis in Russia. Annals of Clinical and Experimental Neurology (Annaly klinicheskoj i eksperimentalʹnoj nevrologii). 2023;17(3):5–13. (In Russ.). doi: 10.54101/ACEN.2023.3.1Grishina DA, Suponeva NA, Arestova AS, Melnik EA, Tumilovich TA, Sinkova VV, Piradov MA. Chronic inflammatory demyelinating polyradiculoneuropathy overdiagnosis in Russia. Annals of Clinical and Experimental Neurology (Annaly klinicheskoj i eksperimentalʹnoj nevrologii). 2023;17(3):5–13. (In Russ.). doi: 10.54101/ACEN.2023.3.1Almoallim H, Albar H, Mehdawi F. Diagnostic Approach to Proximal Myopathy. 2021 Jan 6. In: Almoallim H, Cheikh M, editors. Skills in Rheumatology [Internet]. Singapore: Springer; 2021. Chapter 9. Available from: https://www.ncbi.nlm.nih.gov/books/NBK585737/ doi: 10.1007/978-981-15-8323-0_9Almoallim H, Albar H, Mehdawi F. Diagnostic Approach to Proximal Myopathy. 2021 Jan 6. In: Almoallim H, Cheikh M, editors. Skills in Rheumatology [Internet]. Singapore: Springer; 2021. Chapter 9. Available from: https://www.ncbi.nlm.nih.gov/books/NBK585737/ doi: 10.1007/978-981-15-8323-0_9Kimura J. Electrodiagnosis in diseases of nerve and muscle: principles and practice. 4<sup>rd</sup> edn. Oxford University Press. 2013:1176.Kimura J. Electrodiagnosis in diseases of nerve and muscle: principles and practice. 4<sup>rd</sup> edn. Oxford University Press. 2013:1176.Kamath S, Venkatanarasimha N, Walsh MA, Hughes PM. MRI appearance of muscle denervation. Skeletal Radiology. 2008;37(5):397–404. doi: 10.1007/s00256-007-0409-0Kamath S, Venkatanarasimha N, Walsh MA, Hughes PM. MRI appearance of muscle denervation. Skeletal Radiology. 2008;37(5):397–404. doi: 10.1007/s00256-007-0409-0Grimm A, Vittore D, Schubert V, Lipski C, Heiling B, Décard BF, Axer H. Ultrasound pattern sum score, homogeneity score and regional nerve enlargement index for differentiation of demyelinating inflammatory and hereditary neuropathies. Clinical Neurophysiology. 2016;127(7):2618–24. doi: 10.1016/j.clinph.2016.04.009Grimm A, Vittore D, Schubert V, Lipski C, Heiling B, Décard BF, Axer H. Ultrasound pattern sum score, homogeneity score and regional nerve enlargement index for differentiation of demyelinating inflammatory and hereditary neuropathies. Clinical Neurophysiology. 2016;127(7):2618–24. doi: 10.1016/j.clinph.2016.04.009Morozova SN, Grishina DA, Orlov VA, Kartashov SI, Sinkova VV, Tsaregorodtsev DS, Krotenkova MV, Suponeva NA. Quantitative MRI assessment of peripheral nerves. Neuromuscular Diseases (Nervno-myhechnye bolezni). 2024;14(1):76–85. (In Russ.). doi: 10.17650/2222-8721-2024-14-1-76-85Morozova SN, Grishina DA, Orlov VA, Kartashov SI, Sinkova VV, Tsaregorodtsev DS, Krotenkova MV, Suponeva NA. Quantitative MRI assessment of peripheral nerves. Neuromuscular Diseases (Nervno-myhechnye bolezni). 2024;14(1):76–85. (In Russ.). doi: 10.17650/2222-8721-2024-14-1-76-85Lichtenstein T, Sprenger A, Weiss K, Slebocki K, Cervantes B, Karampinos D, Maintz D, Fink GR, Henning TD, Lehmann HC. MRI biomarkers of proximal nerve injury in CIDP. Annals of Clinical and Translational Neurology. 2017;5(1):19–28. doi: 10.1002/acn3.502Lichtenstein T, Sprenger A, Weiss K, Slebocki K, Cervantes B, Karampinos D, Maintz D, Fink GR, Henning TD, Lehmann HC. MRI biomarkers of proximal nerve injury in CIDP. Annals of Clinical and Translational Neurology. 2017;5(1):19–28. doi: 10.1002/acn3.502Grishina DA, Suponeva NA, Piradov MA. Differential diagnosis of chronic acquired demyelinating polyneuropathies. Russian neurological journal (Rossijskij Nevrologicheskiy Zhurnal). 2023;28(6):41–50. (In Russ.) doi: 10.30629/2658-7947-2023-28-6-41-50Grishina DA, Suponeva NA, Piradov MA. Differential diagnosis of chronic acquired demyelinating polyneuropathies. Russian neurological journal (Rossijskij Nevrologicheskiy Zhurnal). 2023;28(6):41–50. (In Russ.) doi: 10.30629/2658-7947-2023-28-6-41-50Rizvanova AS, Grishina DA, Suponeva NA. Clinical heterogeneity of chronic inflammatory demyelinating polyneuropathy: diagnostic challenges. Almanac of Clinical Medicine (Al’manah klinicheskoj mediciny). 2020;48(1):56–64. (In Russ.) doi: 10.18786/2072-0505-2020-48-007Rizvanova AS, Grishina DA, Suponeva NA. Clinical heterogeneity of chronic inflammatory demyelinating polyneuropathy: diagnostic challenges. Almanac of Clinical Medicine (Al’manah klinicheskoj mediciny). 2020;48(1):56–64. (In Russ.) doi: 10.18786/2072-0505-2020-48-007

The authors declare that there are no conflicts of interest present.