Kalmijn S, Oey P L, Wokke J H, Wieneke G H
Department of Clinical Neurophysiology, Rudolf Magnus Institute of Neurosciences, University Medical Centre, Utrecht, The Netherlands.
Electromyogr Clin Neurophysiol. 1999 Sep;39(6):349-53.
Noninvasive tests of four autonomic organ systems (vasomotor control, baroreceptor reflexes, sudomotor function and pupillary reflexes) were performed on nine patients with hereditary motor and sensory neuropathy (HMSN) type I and three patients with Lambert-Eaton myasthenic syndrome (LEMS). The results were compared with those of 33 control subjects. Autonomic dysfunction was considered present when at least two of the four organ system tests were abnormal. The three patients with LEMS had abnormal results in two or more different systems, whereas only one of the nine patients with HMSN type I had two abnormal test results. This study demonstrates that autonomic dysfunction is not a common finding in patients with HMSN type I and its presence should alert us to find the cause of this autonomic disorder.
对9例I型遗传性运动和感觉神经病（HMSN）患者及3例兰伯特-伊顿肌无力综合征（LEMS）患者进行了四个自主神经系统（血管运动控制、压力感受器反射、汗腺功能和瞳孔反射）的无创检测。将结果与33名对照受试者的结果进行比较。当四个器官系统检测中至少两项异常时，则认为存在自主神经功能障碍。3例LEMS患者在两个或更多不同系统中结果异常，而9例I型HMSN患者中只有1例有两项检测结果异常。本研究表明，自主神经功能障碍在I型HMSN患者中并非常见表现，其出现应提醒我们寻找这种自主神经障碍的病因。
