r-n-jРоссийский неврологический журналRussian neurological journal2658-79472686-7192МИА10.30629/2658-7947-2024-29-2-4-16r-n-j-550Research ArticleОБЗОРЫREVIEWSПарапротеинемические полиневропатииParaproteinemic neuropathieshttps://orcid.org/0000-0001-5937-9463ЗиновьеваО. Е.ZinovyevaO. E.

Москва

Moscow

zinovyevaolga@yandex.ruhttps://orcid.org/0009-0003-5897-7594НовиковД. К.NovikovD. K.

Москва

Moscow

danil61503@gmail.comhttps://orcid.org/0009-0006-2538-070XОчил-зодаН. З.Ochil-zodaN. Z.

Москва

Moscow

ms.ochilzoda@mail.ruhttps://orcid.org/0000-0003-4260-0226РамеевВ. В.RameevV. V.

Москва

Moscow

vvrameev@mail.ruКафедра нервных болезней и нейрохирургии института клинической медицины, ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России (Сеченовский Университет)РоссияThe Department of Nervous Diseases and Neurosurgery of the Institute of Clinical Medicine of the I.M. Sechenov First Moscow State Medical University of the Ministry of Health of Russia (Sechenov University)Russian FederationКафедра внутренних, профессиональных болезней и ревматологии института клинической медицины, ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России (Сеченовский Университет)РоссияThe Department of Internal, Occupational Diseases and Rheumatology of the Institute of Clinical Medicine of the I.M. Sechenov First Moscow State Medical University of the Ministry of Health of Russia (Sechenov University)Russian Federation202424052024292416Copyright © Зиновьева О.Е., Новиков Д.К., Очил-зода Н.З., Рамеев В.В., 20242024Зиновьева О.Е., Новиков Д.К., Очил-зода Н.З., Рамеев В.В.Zinovyeva O.E., Novikov D.K., Ochil-zoda N.Z., Rameev V.V.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.r-n-j.com/jour/article/view/550

Парапротеинемические полиневропатии (ППН) встречаются нечасто и обусловлены заболеваниями, сопровождающимися образованием и накоплением патологического белка парапротеина, который представляет собой иммуноглобулин или его свободную легкую цепь. К таким заболеваниям относятся моноклональная гаммапатия неопределенного значения, множественная миелома, макроглобулинемия Вальденстрема, амилоидоз легких цепей, POEMS-синдром и др. Диагностика ППН нередко вызывает затруднения, поскольку ее клиническая картина может быть неспецифичной, стертой или атипичной, а для подтверждения наличия парапротеина необходимо использовать специализированные лабораторные методы, в том числе иммунохимический анализ крови и мочи. Также лекарственные препараты, используемые для лечения парапротеинемических заболеваний, сами могут вызвать симптомы полиневропатии, поэтому важно своевременно заподозрить первые симптомы заболевания, определить механизм повреждения нервных волокон, провести дифференциальную диагностику и начать патогенетическую терапию, направленную на подавление синтеза патологического белка.

Paraproteinemic polyneuropathies (PPN) occur generally infrequently and arise from diseases, associated with formation and accumulation of a pathological protein, commonly known as paraprotein, which is typically an immunoglobulin or its free light chain. Such diseases include the following: monoclonal gammopathy of undetermined significance, multiple myeloma, Waldenström’s macroglobulinemia, AL amyloidosis, POEMS syndrome, etc. Diagnosis of PPN is challenging due to nonspecific, indistinct or atypical clinical manifestation, and it is necessary to apply specialized laboratory methods, including immunochemical analysis of blood and urine. Moreover, medications can cause symptoms of polyneuropathy themselves. Thus, it is important to suspect the first symptoms of the disease in time, determine the mechanism of damage to nerve fibers, carry out differential diagnosis and initiate therapy targeting the suppression of pathological protein synthesis.

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