Russman Barry S
Department of Neurology, Oregon Health and Science University, Shriners Hospital for Children-Portland, Portland, Oregon, USA.
J Child Neurol. 2007 Aug;22(8):946-51. doi: 10.1177/0883073807305673.
The clinical classification of spinal muscular atrophy, caused by deletion of the survival motor neuron 1 gene (SMN1), is based on age at onset and maximum function achieved. Evidence suggests that maximum function achieved is more closely related to life expectancy than age at onset. Therefore, it is important to wait for a period before assigning a patient to 1 of 5 classes of the disorder. Several diseases result from degeneration of the anterior horn cell but are not caused by SMN1. The classification for these conditions is evolving. This article offers an attempt at organizing one's thinking about this disease group.
由生存运动神经元1基因（SMN1）缺失引起的脊髓性肌萎缩症的临床分类基于发病年龄和所达到的最大功能。有证据表明，所达到的最大功能比发病年龄与预期寿命的关系更为密切。因此，在将患者归入该疾病的5个类别之一之前等待一段时间很重要。几种疾病是由前角细胞变性导致的，但并非由SMN1引起。这些病症的分类正在不断发展。本文试图梳理有关这一疾病组的思路。
