Marcello N, Baratti M, Ortaggio F, Vescovini E, Zanoni P, Tugnoli V, De Grandis D
Department of Neurology, Arcispedale S. Maria Nuova, Reggio Emilia, Italy.
Electromyogr Clin Neurophysiol. 1995 Nov;35(7):387-95.
To verify an eventual disfunction of the palmar sympathetic skin activity in Duchenne Muscular Dystrophy, we studied the sympathetic skin response (SSR) with endogenous, Deep Breathing (DB-SSR), exogenous Startle (SE-SSR) and Electric type stimuli (EE-SSR) in a group of patients and a control group. At the same time as the SSR, the heart rate (HR) and the basic R-R interval variation (RRIV) as well as the EMG diaphragmatic activity were checked. In this way, significant differences in the latency (p = 0.02) and amplitude (p = 0.009) values of the EE-SSR and in the amplitude values of the SE-SSR (p = 0.001) and of the DB-SSR (p = 0.02) were noted in the DMD group compared to the control group. In the group of patients the rise in latency of the EE-SSR correlates with the age (p = 0.0001) and with the reduction of its amplitude (p = 0.03). This last parameter correlates with the stage of the illness (p = 0.02). In the control group the mean amplitude of the EE-SSR is approximately 30% greater than that of startle and 50% of deep breathing. Although with amplitudes homogeneously reduced, these ratios are also conserved in the DMD group. Moreover the HR is greater (p = 0.0001) whilst the RRIV is reduced (p = 0.02) in the group of the patients. However these parameters do not correlated either to the cardiac involvement or to the stage of the illness. The presence of the SSR for all the modalities of stimulation indicates the substantial integrity of the afferent and the efferent paths of the ANS. The alterations in latency and amplitude of the SSR in the patients could be the consequence of a lack of dystrophin at the level of the sudoriparous myopithelium in analogy to what has been demonstrated in the mdx mouse.
为了验证杜氏肌营养不良症患者手掌交感神经皮肤活动是否存在功能障碍，我们在一组患者和一个对照组中，采用内源性深呼吸（DB - SSR）、外源性惊吓（SE - SSR）和电刺激（EE - SSR）研究了交感神经皮肤反应（SSR）。在检测SSR的同时，还检查了心率（HR）、基本R - R间期变化（RRIV）以及膈肌肌电图活动。结果发现，与对照组相比，杜氏肌营养不良症（DMD）组的EE - SSR潜伏期（p = 0.02）和波幅（p = 0.009）值、SE - SSR波幅值（p = 0.001）以及DB - SSR波幅值（p = 0.02）存在显著差异。在患者组中，EE - SSR潜伏期的延长与年龄相关（p = 0.0001），且与波幅降低相关（p = 0.03）。最后这个参数与疾病阶段相关（p = 0.02）。在对照组中，EE - SSR的平均波幅比惊吓反应的波幅大约大30%，比深呼吸反应的波幅大50%。尽管DMD组的波幅均一性降低，但这些比例仍然保持。此外，患者组的心率更高（p = 0.0001），而RRIV降低（p = 0.02）。然而，这些参数与心脏受累情况或疾病阶段均无相关性。所有刺激方式下SSR的存在表明自主神经系统传入和传出路径基本完整。患者SSR潜伏期和波幅的改变可能是由于汗腺肌上皮水平缺乏抗肌萎缩蛋白，这与mdx小鼠的情况类似。
