平山病的临床特征和神经电生理诊断

Clinical features and neurophysiological diagnosis of Hirayama disease

目的探讨平山病的临床和神经电生理特点,提高临床医师对该病的诊断水平。方法收集2010年1月至2012年4月我科收治的12例平山病患者的临床及神经电生理资料,对其特征进行分析。结果①患者均为男性,起病年龄为18.1岁,均有头、颈反复屈曲史。单侧起病9例,其中3例双侧不对称性受累。11例伴有冷麻痹,所有患者均有手指伸展时震颤和前臂斜坡样萎缩。②感觉神经传导均正常,均未见运动神经传导阻滞,运动神经传导异常主要为尺神经混合肌肉动作电位(CMAP)波幅减低。③交感皮肤反应异常率60%,主要为双上肢波潜伏时延长。④针电极肌电图异常率以下颈髓支配肌肉最高。结论该病有其特定的发病年龄及特殊的临床症状和体征,神经传导检查有助于除外尺神经及臂丛神经损害,针电极肌电图对确诊本病非常重要。

Objective To explore the clinical and neurophysiological features of Hirayama disease and to improve the diagnostic level of this disease for clinical doctors.Methods The clinical and neurophysiological data of 12 cases between January 2010 and April 2012 were collected and analyzed.Results ① All of the 12 cases were male and the mean age at onset was 18.1 years.All patients had a history of sustained or repeated neck flexion;9 cases showed unilateral symptoms and 3 cases showed bilateral but asymmetric involvements;11 cases showed cold paresis of the fingers.Tremor upon mild finger extension and forearm oblique atrophy were noted in every patient.② Sensory nerve conduction was normal,and there were no nerve conduction blocks present in any patient.Reduced amplitude of the compound muscle action potential of the ulnar nerve was the most prominent finding in nerve conduction studies.③ The abnormal finding of sympathetic skin response was a prolonged latency in both upper limbs（40%） of all the tested patients.④ The abnormality of needle EMG was more prominent in the muscles innervated by the lower cervical segments.Conclusion Hirayama disease has a specific age of onset and special clinical symptoms and signs.Nerve conduction study is helpful in ruling out the ulnar nerve and brachial plexus neuropathy.Needle EMG sampling is very important for diagnosis.