Solders G, Andersson T, Persson A
Department of Clinical Neurophysiology, Huddinge University Hospital, Sweden.
Muscle Nerve. 1991 Nov;14(11):1074-9. doi: 10.1002/mus.880141106.
CNS conduction and autonomic nervous function were investigated in 15 patients with HMSN I. Central motor conduction time (CMCT) was estimated with magnetic brain stimulation and electrical nerve root stimulation. Somatosensory evoked potential (SEP) and visual evoked potential (VEP) were used for assessment of central sensory and visual conduction. Autonomic effector organ functions were assessed with the R-R variation test for parasympathetic function, and the sympathetic skin response test (SSR) for skin sympathetic sudomotor activity. Five of the patients had prolonged CMCT. Central sensory conduction was normal in 3, and slightly prolonged in 1 of the patients, but could not be estimated in 11 due to lack of response from the cervical recording. VEP was abnormal in 2 patients. R-R variations during normal breathing were low in 8 of 15 patients, and low also during deep breathing in 1 of 15. The SSR test was pathological in 5 of 15 patients. Thus, impaired central conduction and/or autonomic dysfunction was not an uncommon finding in patients with HMSN I.
对15例遗传性运动感觉神经病I型（HMSN I）患者的中枢神经系统传导和自主神经功能进行了研究。通过磁脑刺激和神经根电刺激来评估中枢运动传导时间（CMCT）。体感诱发电位（SEP）和视觉诱发电位（VEP）用于评估中枢感觉和视觉传导。通过R-R间期变异性试验评估副交感神经功能的自主效应器器官功能，通过交感皮肤反应试验（SSR）评估皮肤交感汗腺运动活动。5例患者的CMCT延长。3例患者的中枢感觉传导正常，1例患者的中枢感觉传导稍有延长，但11例患者因颈部记录无反应而无法评估。2例患者的VEP异常。15例患者中有8例在正常呼吸时R-R间期变异性降低，15例中有1例在深呼吸时R-R间期变异性也降低。15例患者中有5例的SSR试验呈病理性。因此，中枢传导受损和/或自主神经功能障碍在HMSN I患者中并非罕见的发现。
