肌电图呈广泛神经源性损害的平山病与肌萎缩侧索硬化患者电生理特点比较  

Electrophysiological differences between Hirayama disease patients with diffuse neurogenic damages on electromyography and patients with amyotrophic lateral sclerosis

作　　者：沈东超[1] 方佳[1] 丁青云[1] 崔博[1] 李大伟[1] 邰宏飞[1] 管宇宙[1] 崔丽英 

机构地区：[1]中国医学科学院北京协和医院神经科,100730 [2]中国医学科学院神经科学中心

出　　处：《中华神经科杂志》2016年第8期587-591,共5页Chinese Journal of Neurology

摘　　要：目的 比较肌电图呈广泛神经源性损害的平山病患者与肌萎缩侧索硬化（amyotrophic lateral sclerosis，ALS）患者的电生理特点。方法 对2006年4月至2016年1月于北京协和医院神经科经临床和肌电图检查诊断为平山病的83例患者资料进行回顾，寻找肌电图呈广泛神经源性损害的患者，为每例患者选择2例性别、病程相匹配的青年型ALS患者作为对照，对两组患者的电生理特点进行比较。结果 83例平山病患者中共有8例（9.6%）肌电图呈广泛神经源性损害，与ALS对照组[（38.6±4.2）岁]相比，平山病组患者年龄更小[（17.5±2.4）岁，t＝12.92，P〈0.01]，双侧受累者更少（1/8与12/16，P＝0.008），正中神经运动传导复合肌肉动作电位（CMAP）波幅降低者更少（1/8与15/16，P〈0.01），尺神经与正中神经的CMAP比值更低[0.66（0.29，0.79）与1.83（1.20, 3.82），Z＝124.0，P〈0.01]，上肢肌肉的自发电位更少（小指展肌Z＝60.5，P＝0.003；伸指总肌Z＝36.0，P＝0.019），胸锁乳突肌运动单位电位时限较相同性别、年龄组正常值增加的百分比更少[33%（31%，50%）与52%（43%，58%）, Z＝103, P＝0.016]。结论 平山病患者肌电图存在广泛神经源性损害的发病机制尚不明确，上述电生理特点有助于其与ALS相鉴别。Objective To compare the electrophysiological features between Hirayama disease （HD） patients with diffuse neurogenie damages on electromyography （EMG） and patients with amyotrophic lateral sclerosis （ALS） . Methods The data of 83 patients who were diagnosed with HD based on eleetromyographical findings collected from April 2006 to January 2016 were reviewed to find patients with diffused neurogenie damages on EMG. Two ALS patients who were sex- and disease duration-matched were chosen as controls for every HD patient. Results Eight of 83 HD patients （9. 6% ） were found to have diffuse neurogenic damages on EMG. Compared with the ALS controls （ （38. 6 ±4. 2） years old）, the HD group were younger （ （ 17.5 ± 2. 4 ） years old, t = 12. 92, P 〈 0. 01 ）, had fewer cases with involvement on both sides （1/8 vs 12/16, P = 0. 008 ）, and fewer cases with decreased amplitude of the median nerve compound muscle action potential （CMAP; 1/8 vs 15/16, P 〈 0. 01 ）. The ulnar/median CMAP ratio was lower （0. 66 （0. 29,0. 79） vs 1.83 （ 1.20, 3.82）, Z = 124.0, P 〈 0.01 ）, the spontaneous potential of the upper limbs was less （abductor digiti minimi Z = 60. 5, P = 0. 003 ;extensor digitorum communis Z = 36. 0, P =0. 019）, and the duration of motor unit potential in the stemoeleidomastoid muscle had less increase （33% （31% ,50% ） vs 52% （43% ,58%）, Z = 103, P = 0. 016） in HD patients compared with ALS. Conclusion The pathogenic mechanism of HD with diffuse neurogenic damages on EMG remains unclear, but above-mentioned electrophysiological differences might be helpful to the differential diagnosis of the two diseases.

关 键 词：平山病 肌萎缩侧索硬化 肌电图 

分 类 号：R746.4[医药卫生—神经病学与精神病学]