肌萎缩侧索硬化症上下运动神经元症状的发作和扩散模式。
Onset and spreading patterns of upper and lower motor neuron symptoms in amyotrophic lateral sclerosis.
机构信息
Department of Neurology, Hannover Medical School, Carl Neuberg Strasse 1, 30625 Hannover, Germany.
出版信息
Muscle Nerve. 2011 May;43(5):636-42. doi: 10.1002/mus.21936.
INTRODUCTION
The potential linkage between upper (UMN) and lower motor neuron (LMN) involvement in amyotrophic lateral sclerosis (ALS) has not yet been fully elucidated. There is ongoing discussion as to whether ALS is primarily a disease of UMNs or LMNs.
METHODS
We performed a retrospective analysis of 189 ALS patients from our ALS outpatient database to investigate the different spreading patterns of UMN and LMN affection in disease progression in relation to the onset region.
RESULTS
The body region with the highest UMN involvement at onset in general also had the highest frequency of LMN signs and vice versa. This is in line with the hypothesis of a focal onset of disease, which then spreads to adjacent areas. However, there was a great variation between ALS phenotypes.
DISCUSSION
These observations support the hypothesis of focal damage of a localized group of motor neurons, which then spreads to adjacent motor neurons.
介绍
肌萎缩侧索硬化症（ALS）中，上运动神经元（UMN）和下运动神经元（LMN）的潜在关联尚未完全阐明。目前仍在讨论 ALS 究竟主要是 UMN 疾病还是 LMN 疾病。
方法
我们对来自 ALS 门诊数据库的 189 名 ALS 患者进行了回顾性分析，以研究 UMN 和 LMN 受累在疾病进展中与起始区域相关的不同传播模式。
结果
一般来说，发病时 UMN 受累最严重的身体区域，同时也最常出现 LMN 体征，反之亦然。这与疾病的局灶性起始假说一致，然后向相邻区域扩散。然而，不同的 ALS 表型之间存在很大差异。
讨论
这些观察结果支持了局部运动神经元群局灶性损伤，然后向相邻运动神经元扩散的假说。
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