medalphabetМедицинский алфавитMedical alphabet2078-56312949-2807ООО «Альфмед»10.33667/2078-5631-2024-33-13-18medalphabet-4147Research ArticleСтатьиХроническая воспалительная демиелинизирующая полинейропатия, ассоциированная с подоцитопатией: клинический случай с обзором литературыChronic inflammatory demyelinating polyneuropathy associated with podocytopathy: a clinical case with literature reviewhttps://orcid.org/0000-0001-7891-9883КушнирЯ. Б.KushniYа. B.

Кушнир Яна Богдановна, врач-невролог отделения неврологии № 1

Санкт-Петербург

Kushnir Yana B., neurologist at Dept of Neurology № 1

Saint Petersburg

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

Безводинских Александр Игоревич, клинический ординатор отделения неврологии № 1

Санкт-Петербург

Bezvodinskikh Alexander I., clinical resident at Dept of Neurology № 1

Saint Petersburg

https://orcid.org/0000-0003-3666-9622ВладыкинаА. В.VladykinaA. V.

Владыкина Анастасия Владимировна, клинический ординатор отделения неврологии № 1

Санкт-Петербург

Vladykina Anastasiya V., clinical resident at Dept of Neurology № 1

Saint Petersburg

https://orcid.org/0009-0002-9705-4314ФомичевИ. А.FomichevI. A.

Фомичев Илья Алексеевич, студент лечебного факультета

Санкт-Петербург

Fomichev Ilya A., student at Faculty of Medicine

Saint Petersburg

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

Тотолян Наталья Агафоновна, д.м.н., проф. кафедры неврологии

Санкт-Петербург

Totolyan Natalya A., DM Sci (habil.), prof. at Dept of Neurology

Saint Petersburg

https://orcid.org/0000-0001-6437-232XАмелинА. В.AmelinA. V.

Амелин Александр Витальевич, д.м.н., проф. кафедры неврологии

Санкт-Петербург

Amelin Alexander V., DM Sci (habil.), prof. at Dept of Neurology

Saint Petersburg

ФГБОУ ВО «Первый Санкт-Петербургский государственный медицинский университет имени академика И.П. Павлова» Минздрава РоссииРоссияFirst Saint Petersburg state medical University named after academician I.P. PavlovRussian Federation202424012025033«Неврология и психиатрия» (4)1318Copyright © Кушнир Я.Б., Безводинских А.И., Владыкина А.В., Фомичев И.А., Тотолян Н.А., Амелин А.В., 20252025Кушнир Я.Б., Безводинских А.И., Владыкина А.В., Фомичев И.А., Тотолян Н.А., Амелин А.В.Kushni Y.B., Bezvodinskikh A.I., Vladykina A.V., Fomichev I.A., Totolyan N.A., Amelin A.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.med-alphabet.com/jour/article/view/4147

Хроническая воспалительная демиелинизирующая полинейропатия (ХВДП) – это гетерогенная группа дизиммунных полинейропатий, характеризующаяся прогрессирующим более 2 месяцев или рецидивирующим течением, сэлектрофизиологическими ипатоморфологическими признаками демиелинизации периферических нервов, с ответом на иммунотерапию. Среди редких атипичных ХВДП выделяют вариант, ассоциированный с IgG4-антителами к паранодальному комплексу: к нейрофасцину155, контактину1 и контактин-ассоциированному белку. Наличие антител (АТ) к этим белкам часто связано с тяжелым течением заболевания, плохим ответом на терапию глюкокортикостероидами (ГКС) и внутривенным человеческим иммуноглобулином (ВВИГ). Встатье представлено описание развития у пациента с предшествующей протеинурией тяжелой ХВДП с рецидивирующим течением, с выраженным неврологическим дефицитом, слабостью дыхательных мышц, потребовавшей вспомогательной искусственной вентиляции легких. В процессе обследования у пациента была выявлена болезнь минимальных изменений почек.

Chronic imflammatory demyelinating polyneuropathy (CIPD) is a heterogenous group of disimmune polyneuropathies, characterised by progressive or relapsing-remitting disease course, with electrophysiologically and pathomorphologically detected peripheral nerve demyelination. Immunotherapy is considered reasonable in CIDP. CIDP associated with antibodies against nodo-paranodal proteins, namely, NF155, CNTN1, Contactin associated protein has been described amongst the rare atypical CIDP subtypes. The detection of antibodies against nodo-paranodal proteins might have a predictive value for a more severe disease course with resistance to corticosteroid and/or IVIG treatment. Herein we report a case of severe relapsing-remitting CIDP, characterized by respiratory muscle weakness requiring assisted ventilation, with a previous history of proteinuria, later also diagnosed with Minimal change disease (MCD).

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