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[副肿瘤性眼阵挛-肌阵挛综合征]

[Paraneoplastic opsoclonus-myoclonus syndrome].

作者信息

Fedoseeva I F, Goncharenko A V, Poponnikova T V, Glebova I V, Pinevich O S, Goncharenko V A

机构信息

Kemerovo State Medical University, Kemerovo, Russia.

Belyaev Kuzbass Regional Clinical Hospital, Kemerovo, Russia.

出版信息

Zh Nevrol Psikhiatr Im S S Korsakova. 2024;124(12):165-170. doi: 10.17116/jnevro2024124121165.

DOI:10.17116/jnevro2024124121165
PMID:39731387
Abstract

Opsoclonus-myoclonus syndrome (OMS) is a rare neurological disorder characterized by a combination of main symptoms: opsoclonus, myoclonus, ataxia, psychoemotional and behavioral disturbances. OMS can develop in children as a result of immunopathological processes against the background of infectious or oncological pathology and lead to persistent neurological deficit. A case of ten-year observation of paraneoplastic OMS associated with neuroblastoma in a child is presented. Within 6 months, the clinical picture of OMS was not full and manifested by recurrent cerebellar ataxia and psychoemotional disorders. The appearance of opsoclonus against the background of increased disturbances in statics and coordination made it possible to diagnose OMS and suggest its paraneoplastic genesis. The peculiarity of the case is the combination of opsoclonus with the development of a symptomatic epileptiform ictus. Surgical treatment of neuroblastoma, immunosuppressive therapy in combination with nootropic and symptomatic anticonvulsant therapy have shown effectiveness and led to stabilization of the condition, regression of cerebellar symptoms and restoration of the rate of mental development. The long course of OMS with the gradual formation of a complete symptom complex complicates timely diagnosis of the underlying disease. Cases of cerebellar ataxia, myoclonus and abnormal eye movements, regardless of the severity and sequence of development of the clinical picture, require an interdisciplinary diagnostic approach and consideration of oncological pathology in a differential diagnostic aspect.

摘要

眼阵挛-肌阵挛综合征(OMS)是一种罕见的神经系统疾病,其主要症状包括眼阵挛、肌阵挛、共济失调、心理情绪和行为障碍。OMS可在儿童期因感染性或肿瘤性病理背景下的免疫病理过程而发生,并导致持续性神经功能缺损。本文介绍了一例对一名患有与神经母细胞瘤相关的副肿瘤性OMS的儿童进行十年观察的病例。在6个月内,OMS的临床表现并不完全,表现为反复出现的小脑性共济失调和心理情绪障碍。在静态和协调性障碍加重的背景下出现眼阵挛,使得能够诊断OMS并提示其副肿瘤性病因。该病例的特点是眼阵挛与症状性癫痫样发作的发生相结合。神经母细胞瘤的手术治疗、免疫抑制治疗与促智药和对症抗惊厥治疗相结合已显示出有效性,并导致病情稳定、小脑症状消退以及智力发育速度恢复。OMS病程较长且症状复合体逐渐形成,这使得潜在疾病的及时诊断变得复杂。无论临床表现的严重程度和发展顺序如何,小脑性共济失调、肌阵挛和异常眼动的病例都需要采用多学科诊断方法,并在鉴别诊断方面考虑肿瘤性病理。

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