Ravits John, Paul Piper, Jorg Cathy
Neurogenomics Laboratory, Benaroya Research Institute at Virginia Mason, Seattle, WA 98101, USA.
Neurology. 2007 May 8;68(19):1571-5. doi: 10.1212/01.wnl.0000260965.20021.47.
To localize and analyze the anatomic distribution of upper motor neuron (UMN) and lower motor neuron (LMN) loss in patients with ALS early in their disease when motor manifestations were still relatively focal using clinical examination signs.
We reviewed records of 100 patients with ALS who were evaluated when the diagnosis was first established or suspected. From the patient history, we ascertained the body region of first symptoms and the time course. From the physical examination, we separately graded severity of UMN and LMN signs in each body region, indexed these to the body region of first symptoms, and sorted and analyzed the data.
Motor manifestations began in one body region in 98% of patients. UMN and LMN signs were both maximal in these same body regions, but they were independent of each other in severity and their outward distribution, which conformed to neuronal anatomy. The outward distribution of both UMN and LMN signs seemed more directed to caudal body regions than to rostral ones.
Motor neuron degeneration in ALS is a focal process at both upper and lower motor neuron levels of the motor system. At each level, it begins corresponding to the same peripheral body region and then advances contiguously and separately to summate over time.
在运动症状仍相对局限的肌萎缩侧索硬化症（ALS）患者疾病早期，利用临床检查体征定位并分析上运动神经元（UMN）和下运动神经元（LMN）损失的解剖分布。
我们回顾了100例首次确诊或疑似ALS患者的记录。从患者病史中，我们确定首发症状的身体部位和病程。通过体格检查，我们分别对每个身体部位的UMN和LMN体征严重程度进行分级，将这些分级与首发症状的身体部位相关联，并对数据进行分类和分析。
98%的患者运动症状始于一个身体部位。UMN和LMN体征在这些相同的身体部位均最为明显，但它们在严重程度和外在分布上相互独立，这与神经元解剖结构相符。UMN和LMN体征的外在分布似乎更多地指向身体的尾端区域而非头端区域。
ALS中的运动神经元变性在运动系统的上运动神经元和下运动神经元水平均为局灶性过程。在每个水平，它始于相应的外周身体部位，然后随着时间的推移持续且分别进展并累加。
