uzspbgmuУчёные записки Первого Санкт-Петербургского государственного медицинского университета имени академика И. П. ПавловаThe Scientific Notes of the Pavlov University1607-41812541-8807Academician I.P. Pavlov First St. Petersburg State Medical University10.24884/1607-4181-2025-32-4-65-76uzspbgmu-1201Research ArticleОРИГИНАЛЬНЫЕ РАБОТЫORIGINAL PAPERSЛабораторная и инструментальная диагностика хронической воспалительной демиелинизирующей полиневропатии: анализ 158 случаевLaboratory and instrumental diagnostics of chronic inflammatory demyelinating polyneuropathy: analysis of 158 caseshttps://orcid.org/0000-0001-7891-9883КушнирЯ. Б.KushnirYa. B.

Кушнир Яна Богдановна, врач-невролог 1 неврологического отделения

197022, Санкт-Петербург, ул. Льва Толстого, д. 6-8

Kushnir Yana B., Neurologist of the 1st Neurological Department

6-8, L’va Tolstogo str., Saint Petersburg, 197022

kushnir.yana2014@yandex.ruhttps://orcid.org/0000-0003-2846-6411БезводинскихА. И.BezvodinskikhA. I.

Безводинских Александр Игоревич, врач-невролог 1 неврологического отделения

197022, Санкт-Петербург, ул. Льва Толстого, д. 6-8

Bezvodinskikh Aleksandr I., Neurologist of the 1st Neurological Department

6-8, L’va Tolstogo str., Saint Petersburg, 197022

https://orcid.org/0000-0003-2967-4378КулагинП. А.KulaginP. A.

Кулагин Павел Александрович, врач-невролог 1 неврологического отделения

197022, Санкт-Петербург, ул. Льва Толстого, д. 6-8

Kulagin Pavel A., Neurologist of the 1st Neurological Department

6-8, L’va Tolstogo str., Saint Petersburg, 197022

https://orcid.org/0000-0002-8193-0116БубноваЕ. В.BubnovaE. V.

Бубнова Евгения Викторовна, кандидат медицинских наук, доцент кафедры рентгенологии и радиационной медицины с рентгенологическим и радиологическим отделениями

197022, Санкт-Петербург, ул. Льва Толстого, д. 6-8

Bubnova Evgeniya V., Cand. of Sci. (Med.), Associate Professor of the Department of X-ray and Radiation Medicine with X-ray and Radiological Departments

6-8, L’va Tolstogo str., Saint Petersburg, 197022

https://orcid.org/0000-0002-6715-8203ТотолянН. А.TotolyanN. A.

Тотолян Наталья Агафоновна, доктор медицинских наук, профессор кафедры неврологии

197022, Санкт-Петербург, ул. Льва Толстого, д. 6-8

Totolyan Nataliya A., Dr. of Sci. (Med.), Professor of the Department of Neurology

6-8, L’va Tolstogo str., Saint Petersburg, 197022

Первый Санкт-Петербургский государственный медицинский университет имени академика И. П. ПавловаРоссияPavlov UniversityRussian Federation2025080420263246576Copyright © Кушнир Я.Б., Безводинских А.И., Кулагин П.А., Бубнова Е.В., Тотолян Н.А., 20262026Кушнир Я.Б., Безводинских А.И., Кулагин П.А., Бубнова Е.В., Тотолян Н.А.Kushnir Y.B., Bezvodinskikh A.I., Kulagin P.A., Bubnova E.V., Totolyan N.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.sci-notes.ru/jour/article/view/1201Введение

Введение. В связи с разнообразием клинико-электрофизиологических фенотипов хронической воспалительной демиелинизирующей полиневропатии (ХВДП) и вариабельностью их ответа на патогенетическую терапию, вероятно, существуют различия в иммунопатологических механизмах при разных формах заболевания. Актуальной является задача поиска ключевых лабораторных и инструментальных особенностей различных клинико-патогенетических вариантов спектра ХВДП.

Цель – сравнить данные лабораторных анализов сыворотки крови, цереброспинальной жидкости и данных инструментальных исследований при типичной ХВДП и ее вариантах.

Методы и материалы

Методы и материалы. В ретроспективно-проспективном исследовании проанализированы клинические, лабораторные, инструментальные данные 158 пациентов с диагнозом достоверной ХВДП, соответствующей критериям European Federation of Neurological Societies/Peripheral Nervous System (EAN/PNS) 2021, разделенных на группу типичной ХВДП и ее вариантов.

Результаты

Результаты. Антитела к ганглиозидам и сульфатидам чаще всего выявлялись у пациентов с сенсорным вариантом ХВДП – в 46,2 % (12/26) случаев. Парапротеин в сыворотке крови выявлен у 27,2 % (36/132) пациентов с ХВДП, причем среди них значительную часть составляли больные с сенсорным вариантом – 33 % (12/36). При анализе структуры клинических фенотипов среди пациентов, прошедших скрининг на наличие парапротеина (n=132), преобладали больные с дистальным вариантом – 40 % (8/20) случаев. Наиболее высокие показатели белка в цереброспинальной жидкости (ЦСЖ) отмечены у пациентов с дебютом заболевания в возрасте 15–29 лет (критерий Краскела–Уоллиса, p=0,028), а также у больных с рецидивирующим типом течения и острым началом (Хи-Квадрат Пирсона, p=0,01). Достоверных различий по уровню белка в ЦСЖ между группой типичной ХВДП и ее вариантов не выявлено. Патологические паттерны олигоклонального IgG в ЦСЖ выявлены у 21,7 % (20/92) пациентов, причем значительно чаще у пациентов с вариантами ХВДП – 60,0 % (12/20) случаев. По данным электронейромиографии (ЭНМГ) увеличение дистальной латентности по n. tibialis достоверно чаще регистрировалось у пациентов с типичной ХВДП по сравнению с вариантами (26,5 % против 7,3 % соответственно; Хи-квадрат Пирсона, p=0,0019). При анализе совокупных показателей (Σ показателей) по n. tibialis также выявлена более высокая частота патологических изменений у пациентов с типичной ХВДП (61,2 % против 35,8 %; Хи-квадрат Пирсона, p=0,0033). У пациентов с моторным вариантом ХВДП накопление контрастного препарата корешками и спинномозговыми нервами по данным магнитно-резонансной томографии (МРТ) выявлялось в 57,2 % (4/7) случаев, что достоверно чаще, чем при других вариантах заболевания (критерий Фишера–Фримана–Холтона, p=0,006).

Заключение

Заключение. В современных реалиях лабораторная и инструментальная диагностика у пациентов с ХВДП играет важную роль в исключении альтернативных причин полинейропатий. Выявленные особенности лабораторных и инструментальных исследований при различных вариантах ХВДП могут играть важную роль не только в постановке диагноза, но и в выборе оптимальной патогенетической терапии.

Introduction

Introduction. Given the diversity of clinical and electrophysiological phenotypes of chronic inflammatory demyelinating polyneuropathy (CIDP) and the variability of their response to disease-modifying therapy, immunopathological mechanisms likely differ across disease forms. Identifying key laboratory and instrumental features of various clinical and pathogenetic variants within the CIDP spectrum remains an important unmet need.

The objective was to compare serum and cerebrospinal fluid (CSF) laboratory findings and instrumental study results in typical CIDP and its variants.

Methods and materials

Methods and materials. This retrospective-prospective study analyzed clinical, laboratory, and instrumental data from 158 patients with definite CIDP meeting the 2021 European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) criteria, stratified into typical CIDP and CIDP variants.

Results

Results. Anti-ganglioside and anti-sulfatide antibodies were most frequently detected in the sensory CIDP variant, in 46.2 % (12/26) of cases. Serum paraprotein was identified in 27.2 % (36/132) of patients; among these, a substantial proportion had the sensory variant – 33 % (12/36). When analyzing the structure of clinical phenotypes among patients screened for the presence of paraprotein (n=132), patients with the distal variant prevailed – 40% (8/20) of cases. The highest protein levels in the cerebrospinal fluid (CSF) were observed in patients with disease onset at the age of 15-29 years (Kruskal–Wallis test, p=0.028), as well as in patients with a relapsing type of course and acute onset (Pearson’s Chi-Square Test, p=0.01). No significant differences in CSF protein levels were found between typical CIDP and CIDP variants. Abnormal CSF oligoclonal IgG patterns were detected in 21.7 % (20/92) of patients and were observed significantly more often in CIDP variants, comprising 60.0 % (12/20) of such cases. According to electroneuromyography (ENMG) data, an increase in distal latency according to n. tibialis was significantly more often recorded in patients with typical CIDP compared with the variants (26.5% vs 7.3%, respectively; Pearson’s Chi-Square Test, p=0.0019). The analysis of cumulative indicators (Σ indicators) for n. tibialis also revealed a higher frequency of pathological changes in patients with typical CIDP (61.2% vs 35.8%; Pearson’s Chi-Square Test, p=0.0033). On magnetic resonance imaging (MRI), accumulation of contrast agent by nerve roots and cerebrospinal nerves was detected in 57.2 % (4/7) of patients with motor CIDP variant, which was significantly more often than in other disease variants (Fisher–Freeman–Halton test, p=0.006).

Conclusion

Conclusion. In contemporary practice, laboratory and instrumental diagnostics in CIDP patients plays a critical role in excluding alternative causes of polyneuropathy. The identified laboratory and instrumental features across CIDP variants may be important not only for diagnosis but also for selecting optimal pathogenetic therapy.

хроническая воспалительная демиелинизирующая полинейропатияпарапротеинантитела к ганглиозидамантитела к сульфатидамантинуклеарный факторсвободные каппа и лямбда цепи иммуноглобулиновцереброспинальная жидкостьолигоклональный IgGэлектронейромиографиядистальная латентностьмагнито-резонансная томографияchronic inflammatory demyelinating polyneuropathyparaproteinanti-ganglioside antibodiesanti-sulfatide antibodiesantinuclear factorserum free kappa and lambda light chainscerebrospinal fluidoligoclonal IgG bandselectroneuromyographydistal latencymagnetic resonance imagingReferencesMathey E. K., Park S. B., Hughes R. A. et al. Chronic inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype // Journal of neurology, neurosurgery, and psychiatry. – 2015. – Vol. 86, № 9. – P. 973–985. https://doi.org/10.1136/jnnp-2014-309697.Mathey E. K., Park S. B., Hughes R. A. et al. 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The authors declare that there are no conflicts of interest present.