Lindeman E, Leffers P, Spaans F, Drukker J, Reulen J
Department of Rehabilitation, University Hospital Maastricht, The Netherlands.
Scand J Rehabil Med. 1995 Mar;27(1):59-64.
In order to obtain more information about the deterioration of motor function in patients with myotonic dystrophy and in patients with hereditary motor and sensory neuropathy, changes in strength and functionality were determined at one year intervals, over a follow-up period of 1 to 5 years. Twenty-five myotonic dystrophy patients and 16 hereditary motor and sensory neuropathy patients participated. Strength measurements were restricted to knee extension and flexion torques measured isokinetically on a dynamometer at two velocities (120 and 60 degrees/s). Functionality was measured as scaled time scores for rising from sitting, rising from supine, walking and stair climbing. Myotonic dystrophy patients showed a reduction of knee extension torques at 60 degrees/sec (mean decrease per year 5.7 Nm, or 9%) as well as a decrease in functionality. Hereditary motor and sensory neuropathy patients showed no statistically significant changes in torques or functionality. The correlation between isokinetically measured knee extension torques and functional scores was statistically significant in myotonic dystrophy but not in hereditary motor and sensory neuropathy. No correlation was found between changes in torques and changes in functional scores.
为了获取有关强直性肌营养不良患者和遗传性运动感觉神经病患者运动功能恶化的更多信息，在1至5年的随访期内，每隔一年测定一次力量和功能的变化。25例强直性肌营养不良患者和16例遗传性运动感觉神经病患者参与了研究。力量测量仅限于在测力计上以两种速度（120度/秒和60度/秒）等速测量的膝关节伸展和屈曲扭矩。功能通过从坐姿起身、从仰卧位起身、行走和爬楼梯的时间得分进行衡量。强直性肌营养不良患者在60度/秒时膝关节伸展扭矩降低（每年平均降低5.7牛米，即9%），功能也有所下降。遗传性运动感觉神经病患者的扭矩或功能没有统计学上的显著变化。在强直性肌营养不良中，等速测量的膝关节伸展扭矩与功能评分之间的相关性具有统计学意义，但在遗传性运动感觉神经病中则不然。未发现扭矩变化与功能评分变化之间存在相关性。
