r-n-jРоссийский неврологический журналRussian neurological journal2658-79472686-7192МИА10.30629/2658-7947-2023-28-6-41-50r-n-j-502Research ArticleИССЛЕДОВАНИЯ И КЛИНИЧЕСКИЕ НАБЛЮДЕНИЯCLINICAL RESEARCHES AND CASE REPORTSДифференциальная диагностика хронических приобретенных демиелинизирующих полинейропатийDifferential diagnosis of chronic acquired demyelinating polyneuropathieshttps://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/0000-0002-6338-0392ПирадовМ. А.PiradovM. A.

Москва

Moscow

Научный центр неврологииРоссияResearch Center of NeurologyRussian Federation2023080120242864150Copyright © Гришина Д.А., Супонева Н.А., Пирадов М.А., 20242024Гришина Д.А., Супонева Н.А., Пирадов М.А.Grishina D.A., Suponeva N.A., Piradov M.A.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.r-n-j.com/jour/article/view/502Введение

Введение. Редкость хронических приобретенных полинейропатий (ПНП) с демиелинизирующим характером поражения периферических нервов обусловливает трудности их дифференциальной диагностики.

Цель работы

Цель работы: выявить значимые клинические, нейрофизиологические и сонографические дифференциально-диагностические маркеры при хронической воспалительной демиелинизирующей полирадикулонейропатии

(ХВДП) и не-IgM-парапротеинемических демиелинизирующих полинейропатиях (ПДП).

Материал и методы

Материал и методы: в исследование было включено 80 пациентов: 30 — с ХВДП, 30 — с не-IgM-ПДП, ассоциированной с моноклональной гаммапатией неясного значения (ПДП-МГНЗ), и 20 — с не-IgM-ПДП, ассоциированной с лимфопролиферативным заболеванием (ПДП-ЛПЗ). Включенным в исследование пациентам проведены клиническая оценка неврологических нарушений по шкалам MRC, NIS, ВАШ, INCAT, IRODS, SARA; ЭНМГ- и УЗ-исследования периферических нервов.

Результаты

Результаты. Отмечено преобладание мужчин во всех группах (р > 0,05). По сравнению с пациентами с ХВДП больные с ПДП оказались достоверно старше, у них чаще отмечались нейропатический болевой синдром и трофические нарушения (p < 0,05). У пациентов с ПДП-ЛПЗ в отличие от ХВДП и ПДП-МГНЗ отмечено преобладание дистального паттерна распределения мышечной слабости и большая выраженность сенситивной атаксии (p < 0,05). При ЭНМГ-исследованиях у пациентов с ХВДП по сравнению с больными с ПДП при исследовании моторных волокон нервов рук значимо чаще регистрировались блоки проведения и дисперсия М-волн (p < 0,05); а при исследовании двигательных нервов ног — достоверно реже отмечалась невозбудимость моторных волокон (p < 0,05). УЗ-исследование периферических нервов не продемонстрировало значимых различий между пациентами (p > 0,05).

Заключение

Заключение. Несмотря на наличие тех или иных отличительных черт, анализ клинического фенотипа, нейрофизиологических и сонографических изменений у пациентов с ХВДП и не-IgM-ПДП не показал высокоспецифических различий. Электрофорез белков сыворотки крови с иммунофиксацией позволяет дифференцировать ХВДП и ПДП, а дальнейшее обследование у онкогематолога при парапротеинемии — различить МГНЗ от ЛПЗ.

Introduction

Introduction. The rarity of chronic acquired polyneuropathies (PNP) with the demyelinating nature of peripheral nerve damage causes the difficulties of their differential diagnosis that persist in our country and abroad. Objective: to identify significant clinical, neurophysiological and sonographic differential diagnostic markers in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and non-IgM paraproteinemic demyelinating polyneuropathies (PDP).

Material and methods

Material and methods: 80 patients were included in the study: 30 with CIDP, 30 with non-IgM-PDP associated with monoclonal gammapathy of unclear significance (PDP-MGUS), and 20 with non-IgM-PDP associated with lymphoproliferative disease (PDP-LPD). The patients included in the study underwent clinical evaluation of neurological disorders according to the MRC, NIS, VAS, INCAT, IRODS, SARA scales; ENMG and ultrasound studies of peripheral nerves.

Results

Results. The predominance of men in all groups was noted (p > 0.05). Compared with patients with CIDP, patients with PDP were significantly older, they were more likely to have neuropathic pain syndrome and trophic disorders (p < 0.05). In patients with PDP-LPD, in contrast to CIDP and PDP-MGUS, there was a predominance of the distal pattern of muscle weakness distribution and a greater severity of sensitive ataxia (p < 0.05). During NCV studies in patients with CIDP, compared with patients with PDP, blocks of conduction and dispersion of M-waves were signifi -cantly more often recorded in the study of motor fibers of the nerves of the hands (p < 0.05); and in the study of motor nerves of the legs, non-excitability of motor fibers was significantly less often noted (p < 0.05). Ultrasound examination of peripheral nerves showed no significant differences between patients (p > 0.05).

Conclusion

Conclusion. Clinical phenotype, neurophysiological and sonographic changes in patients with CIDP and PDP do not have highly specific differences. Electrophoresis of serum proteins with immunofixation makes it possible to differentiate CIDP and PDP, and further examination by an oncohematologist with paraproteinemia makes it possible to distinguish MGUS from LPD.

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