Finger cold-induced vasodilatation, sympathetic skin response, and R-R interval variation in patients with progressive in spinal muscular atrophy
Finger cold-induced vasodilatation, sympathetic skin response, and R-R interval variation in patients with progressive in spinal muscular atrophy
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DOI：
10.1177/08830738050200110301
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发表时间：
2005-11-01
影响因子：
1.9
通讯作者：
Kohno, Y
中科院分区：
文献类型：
--
作者：
Arai, H;Tanabe, Y;Kohno, Y
为了阐明脊髓性肌萎缩的自主神经功能，我们评估了10例脊髓性肌萎缩患者手指冷诱导的血管舒张、交感皮肤反应和R-R间隔变化：1型7例，2型2例，3型1例。将手指冷致血管舒张、交感皮肤反应及R-R间期变化与健康儿童进行比较。10例脊髓性肌萎缩患者中有6例手指冷致血管舒张异常；这在健康儿童中是正常的。平均交感皮肤反应潜伏期和振幅与健康儿童无显著差异。在所有6例脊髓性肌萎缩患者中，交感皮肤对声音刺激的反应幅度都不存在或很低。脊髓性肌萎缩症患者与健康儿童的平均R-R间隔变化无显著差异。结果表明，一些脊髓性肌萎缩患者存在自主神经功能障碍，特别是交感神经过度活跃，类似于肌萎缩侧索硬化症的功能障碍。
To elucidate autonomic function in spinal muscular atrophy, we evaluated finger cold-induced vasodilatation, sympathetic skin response, and R-R interval variation in 10 patients with spinal muscular atrophy: 7 of type 1, 2 of type 2, and 1 of type 3. Results of finger cold-induced vasodilatation, sympathetic skin response, and R-R interval variation were compared with those of healthy children. Finger cold-induced vasodilatation was abnormal in 6 of 10 patients with spinal muscular atrophy; it was normal in the healthy children. The mean sympathetic skin response latency and amplitude did not differ significantly from those of the healthy children. Amplitudes of sympathetic skin response to sound stimulation were absent or low in all six patients with spinal muscular atrophy. No significant difference was found in the mean R-R interval variation of patients with spinal muscular atrophy and healthy children. Results show that some patients with spinal muscular atrophy have autonomic dysfunction, especially sympathetic nerve hyperactivity, that resembles dysfunction observed in amyotrophic lateral sclerosis.