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首页> 外文期刊>Current opinion in neurology >Pediatric central nervous system inflammatory demyelination: acute disseminated encephalomyelitis, clinically isolated syndromes, neuromyelitis optica, and multiple sclerosis.
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Pediatric central nervous system inflammatory demyelination: acute disseminated encephalomyelitis, clinically isolated syndromes, neuromyelitis optica, and multiple sclerosis.

机译：小儿中枢神经系统炎症性脱髓鞘：急性弥漫性脑脊髓炎，临床孤立综合征，视神经脊髓炎和多发性硬化症。

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PURPOSE OF REVIEW: We review the recent consensus definitions for acute disseminated encephalomyelitis,clinically isolated syndromes, neuromyelitis optica, and multiple sclerosis (MS) in children. We also discuss the importance of clinically defined consistency, the need for biomarker-based patient delineation, the likelihood of subsequent MS diagnosis following acute demyelination, and current therapeutic options. RECENT FINDINGS: Studies of children after a first episode of demyelination have identified disease onset in adolescence, intrathecal oligoclonal bands and optic neuritis as associated with a higher MS risk, whereas prepubertal onset, presence of polyfocal features with encephalopathy, and transverse myelitis have been associated with a lower risk of subsequent MS. The relapsing-remitting form of MS accounts for over 96% of all MS in children. Neuromyelitis optica appears to be a distinct clinical and biological entity for which neuromyelitis optica IgG provides a high degree of specificity. Neuroimaging plays a key role in the diagnosis of acute demyelination, and serial imaging can provide evidence of lesion dissemination in time that can confirm a diagnosis of MS even in the absence of clinical relapse. SUMMARY: Although clinical definitions, increased awareness, and MRI have contributed to the increasing identification of acute demyelination and MS in children, challenges remain in predicting MS risk. Identification of reliable biomarkers or application of more advanced neuroimaging techniques would serve as invaluable tools to distinguish monophasic demyelination from the first attack of MS.
机译：审查的目的：我们审查有关儿童急性弥漫性脑脊髓炎，临床孤立综合征，视神经脊髓炎和多发性硬化症（MS）的最新共识定义。我们还讨论了临床定义一致性的重要性，基于生物标记物的患者描述的需求，急性脱髓鞘后后续MS诊断的可能性以及当前的治疗选择。最新发现：对脱髓鞘首次发作的儿童进行的研究已经确定，青春期疾病发作，鞘内寡克隆带和视神经炎与MS风险较高相关，而青春期前发作，多灶性特征性脑病和横贯性脊髓炎均与相关继发MS的风险较低。 MS的复发-缓解形式占儿童所有MS的96％以上。视神经脊髓炎似乎是一种独特的临床和生物学实体，视神经脊髓炎IgG提供了高度的特异性。神经影像学在急性脱髓鞘的诊断中起着关键作用，连续影像学可以及时提供病变扩散的证据，即使在没有临床复发的情况下也可以确诊MS。简介：尽管临床定义，意识的增强和MRI促进了儿童急性脱髓鞘和MS识别的增加，但在预测MS风险方面仍然存在挑战。可靠的生物标记物的鉴定或更先进的神经成像技术的应用将作为区分单相脱髓鞘与MS首次发作的宝贵工具。

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