EMG Laboratory, Department of Neurology, University of Athens, Aeghinition Hospital, Athens, Greece.
Acta Neurol Belg. 2009 Dec;109(4):294-7.
The aim of this study was to investigate small myelinated (Adelta) and unmyelinated (C) fiber function in patients with CMT1A and CMTX polyneuropathy. 17 CMT1A and 10 Cx32 polyneuropathy patients were investigated with warm and cold threshold to evaluate small myelinated (Adelta) an unmyelinated (C) somatic fiber function and with sympathetic skin responses (SSR) to evaluate postganglionic sympathetic fiber function. Median age and disease duration did not differ between the two groups. Charcot-Marie-Tooth neuropathy score was higher in CMTX patients. Mean MCV differed significantly between the two groups in both Median and Ulnar nerve. In CMT1A patients warm threshold was abnormal in 72% and cold threshold in 53%. On the contrary, in Cx32 patients group warm and cold threshold was abnormal in 10 and 20% respectively. SSR was also abnormal in only a small number of both CMT1A and Cx32 patients (24% and 10% respectively).
Small fiber function is frequently impaired in CMT1A polyneuropathy patients.
本研究旨在探讨 CMT1A 和 Cx32 型多发性神经病患者的小髓鞘（Adelta）和无髓鞘（C）纤维功能。
17 例 CMT1A 和 10 例 Cx32 型多发性神经病患者接受冷热阈值检查以评估小髓鞘（Adelta）和无髓鞘（C）躯体纤维功能，接受交感皮肤反应（SSR）检查以评估节后交感纤维功能。
两组患者的中位年龄和疾病持续时间无差异。CMTX 患者的 Charcot-Marie-Tooth 神经病评分较高。CMT1A 患者的正中神经和尺神经的平均 MCV 明显不同。在 CMT1A 患者中，72%存在异常热阈值，53%存在异常冷阈值。相反，在 Cx32 患者中，冷阈值和热阈值分别有 10%和 20%存在异常。仅有少数 CMT1A 和 Cx32 患者的 SSR 也异常（分别为 24%和 10%）。
CMT1A 型多发性神经病患者的小纤维功能经常受损。
