神经生理指数在评估肌萎缩侧索硬化疾病进展速度中的价值

The value of neurophysiological index in evaluating the progression of amyotrophic lateral sclerosis

目的 通过神经传导研究计算神经生理指数（NI）并评估其在肌萎缩侧索硬化（ALS）疾病进展速度中的价值.方法 纳入2010年1月至2016年8月东南大学附属中大医院门诊随诊或病房收治的ALS患者48例,就诊时符合肯定ALS的36例,临床很可能ALS的12例（随访后均证实为肯定ALS）;球部起病ALS 8例,上肢起病ALS 40例.同时纳入年龄、性别相匹配的健康受试者54例.由专门神经内科医师行ALS功能量表修订版（ALSFRS-R）评估,所有受试者均在我院肌电图室行电生理检查,疾病进展速度用疾病进展率（ΔFS）表示：ΔFS=48-初次就诊ALSFRS-R评分/起病至就诊时间.分别计算并比较复合肌肉动作电位（CMAP）、F波出现率、运动末端潜伏期（DML）及NI与ΔFS之间的相关性.结果 ALS患者ALSFRS-R评分为（14.56±10.10）分,症状持续时间为（14.56±10.10）个月,ΔFS为（1.54±1.18）分/月.ALS组正中神经NI为0.60±0.76,对照组正中神经NI为2.56±0.78,两组差异有统计学意义（t=-12.5,P〈0.01）;ALS组尺神经NI为0.96±0.10,对照组尺神经NI为0.96±0.10,两组差异有统计学意义（t=-0.31,P=0.003）;正中神经NI和尺神经NI与ΔFS均呈负相关（r=-0.63,P=0.000;r=-0.506,P=0.007）;曲线拟合估计发现正中神经NI与ΔFS呈指数关系（R2=0.668,P〈0.01）.结论 NI是一种客观的神经电生理指标,可用于ALS进展速度的评估,判断预后.

Objective To evaluate the value of neurophysiological index （ NI） in evaluating the rate of progression of amyotrophic lateral sclerosis （ ALS ） . Methods Forty-eight patients with ALS were enrolled in Zhongda Hospital of Southeast University from January 2010 to August 2016, of whom 36 patients met the ALS definite diagnostic criteria , 12 patients met the ALS probable diagnostic criteria （ following study confirmed those patients met the ALS definite diagnostic criteria ） , including 8 bulbar-onset and 40 upper extremity onset forms of the disease .Fifty-four age-and sex-matched healthy subjects served as controls.After evaluated by the ALS Functional Rating Scale-revised （ ALSFRS-R） , all subjects underwent electrophysiological examination in the Electromyography Lab of the hospital .The rate of disease progression （ΔFS） =48-total ALSFRS-R score at initial visit/symptom duration （months）.The relevance between the complex muscle action potential （CMAP）, F frequency, distal motor latency （DML）, NI and the ΔFS was investigated respectively.Results In ALS group, the ALSFRS-R score was 14.56 ±10.10, the duration from onset to diagnosis was （14.56 ±10.10） months, and theΔFS was 1.54 ±1.18 per month.The median nerve NI in ALS group was 0.60 ±0.76, in control group was 2.56 ±0.78, with statistically significant difference between two groups （t=-12.5, P〈0.01）.The ulnar nerve NI in ALS group was 0.70 ±0.55, in control group was 0.96 ±0.10, also with statistically significant difference between two groups （t=-0.31, P=0.003）.The median nerve NI and ulnar nerve NI both were negatively correlated withΔFS （r=-0.63, P=0.000; r=-0.506, P=0.007）.The ΔFS was exponentially based on median nerve NI （R2 =0.668, P〈0.01）.Conclusion NI is an objective electrophysiological index , which could be used to evaluate the rate of disease progression in ALS , and to evaluate the prognosis of the disease .