Munsat T L, Andres P L, Finison L, Conlon T, Thibodeau L
Department of Neurology, New England Medical Center, Boston, MA 02111.
Neurology. 1988 Mar;38(3):409-13. doi: 10.1212/wnl.38.3.409.
Using a quantitative, reliable, sensitive and valid measurement technique, we analyzed the rate and pattern of motor deterioration in 50 strictly defined ALS patients for up to 67 months. We observed that the rate of motoneuron loss was linear and symmetric. Bulbar function deteriorated more slowly than respiratory, arm, and leg function. The loss of leg strength was slower than in the arm. No correlation was observed between age at onset and rate of deterioration, or with different regions of onset. Arm strength changed more slowly in women, but other functions showed no male-female differences. These data should prove useful in the design of clinical trials and in generating testable hypotheses of the etiology of this disease.
我们使用一种定量、可靠、灵敏且有效的测量技术，对50例严格界定的肌萎缩侧索硬化症（ALS）患者长达67个月的运动功能恶化速率和模式进行了分析。我们观察到运动神经元丧失的速率呈线性且对称。延髓功能的恶化比呼吸、上肢和下肢功能更为缓慢。下肢力量的丧失比上肢更慢。未观察到发病年龄与恶化速率之间或与不同发病部位之间存在相关性。女性上肢力量变化更为缓慢，但其他功能未显示出性别差异。这些数据在临床试验设计以及生成关于该疾病病因的可检验假设方面应会很有用。
