颈椎下运动神经元综合征:一个诊断挑战。

IF 3.6 3区 医学 Q1 CLINICAL NEUROLOGY Journal of the Neurological Sciences Pub Date : 2025-01-15 Epub Date: 2024-12-20 DOI:10.1016/j.jns.2024.123357
Andrea Fortuna, Gianni Sorarù
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引用次数: 0

摘要

颈下运动神经元(LMN)综合征,也称为臂膀轻瘫,其特征是上肢肌肉萎缩、无力和反射下降,没有感觉症状。这些综合征可由多种因素引起,包括退行性疾病、免疫介导的疾病、感染、有毒物质暴露、代谢紊乱和血管异常临床表现各不相同,运动神经元受累可能局限于颈椎区域或扩展到其他区域,影响预后。误诊是一个重要的问题,特别是在低运动神经元表现中,错误率接近20%这篇综述提出了一个基于磁共振成像(MRI)的分类系统,症状的发作时间(急性、亚急性或慢性),萎缩的对称性和分布,以及病因(散发性或遗传性)。急性症状可包括脊髓缺血3,而亚急性或慢性形式可表现为对称(如颈椎病)4或不对称(如平山病)5表现。神经生理学评估和颈椎MRI对准确诊断至关重要,因为它们揭示了病变定位的模式和潜在病因的额外线索。系统的诊断方法对于驾驭这些综合征的复杂性至关重要。
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Cervical lower motor neuron syndromes: A diagnostic challenge.

Cervical lower motor neuron (LMN) syndromes, also known as brachial paresis, are characterized by muscle atrophy, weakness, and decreased reflexes in the upper limbs, devoid of sensory symptoms. These syndromes can stem from various factors, including degenerative conditions, immune-mediated diseases, infections, toxic exposures, metabolic disorders, and vascular anomalies.1 Clinical presentations vary, with motor neuron involvement potentially limited to the cervical area or extending to other regions, affecting prognosis. Misdiagnosis is a significant issue, particularly in lower motor neuron presentations, with an error rate nearing 20 %.2 This review proposes a classification system based on magnetic resonance imaging (MRI) findings, the onset timing of symptoms (acute, subacute, or chronic), the symmetry and distribution of atrophy, and the etiology (sporadic or hereditary). Acute conditions may include spinal ischemia,3 whereas subacute or chronic forms can manifest as symmetric (e.g., cervical spondylogenic myelopathy)4 or asymmetric (e.g., Hirayama disease)5 presentations. Neurophysiological assessments and cervical MRI are crucial for accurate diagnosis, as they reveal patterns that provide lesion localization and additional clues to the underlying cause. A systematic diagnostic approach is essential for navigating the complexities of these syndromes.

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来源期刊
Journal of the Neurological Sciences
Journal of the Neurological Sciences 医学-临床神经学
CiteScore
7.60
自引率
2.30%
发文量
313
审稿时长
22 days
期刊介绍: The Journal of the Neurological Sciences provides a medium for the prompt publication of original articles in neurology and neuroscience from around the world. JNS places special emphasis on articles that: 1) provide guidance to clinicians around the world (Best Practices, Global Neurology); 2) report cutting-edge science related to neurology (Basic and Translational Sciences); 3) educate readers about relevant and practical clinical outcomes in neurology (Outcomes Research); and 4) summarize or editorialize the current state of the literature (Reviews, Commentaries, and Editorials). JNS accepts most types of manuscripts for consideration including original research papers, short communications, reviews, book reviews, letters to the Editor, opinions and editorials. Topics considered will be from neurology-related fields that are of interest to practicing physicians around the world. Examples include neuromuscular diseases, demyelination, atrophies, dementia, neoplasms, infections, epilepsies, disturbances of consciousness, stroke and cerebral circulation, growth and development, plasticity and intermediary metabolism.
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