TY - T1的运动皮层功能决定预后零星的ALS JF -神经学乔-神经病学SP - 513 LP - 520 - 10.1212 / WNL。首页0000000000002912六世- 87 - 5盟Kazumoto涩谷AU - b苏珊娜。公园盟Nimeshan Geevasinga盟Parvathi Menon AU -詹姆斯·豪厄尔斯盟尼尔·g·西蒙AU -威廉Huynh盟Yu-ichi诺托非盟- Jurgen Gotz盟吉莉安j . Kril盟-拉斯m . Ittner韩礼德AU -约翰·霍奇斯盟格伦达盟-史蒂夫Vucic盟马修·c·基尔南Y1 - 2016/08/02 UR - //www.ez-admanager.com/content/87/5/513.abstract N2 -目的:研究皮质功能和生存之间的关系在肌萎缩性脊髓侧索硬化症(ALS)。首页方法:总共有216推荐筛选,参与者与家族性肌萎缩性侧索硬化症或一个冷静的皮层被排除在外。临床措施和表现型从169年零星的ALS患者结合评估皮质功能使用阈值跟踪经颅磁刺激与指标包括短间隔皮层抑制(SICI)。周围神经研究收集,将复合肌肉动作电位振幅。临床预后因素纵向记录,包括ALS功能评定量表——(ALSFRS-R)。结果:169名患者与109名健康的对照相比,降低了SICI (p & lt;0.0001)。在生存分析中,105名患者进展至死,估计平均存活时间37个月。患者的疾病持续时间不到2年(n = 140),那些球发作(p = 0.017),快速肺活量(VC)下降(p & lt;0.0001),快速ALSFRS-R下降(p & lt;0.0001),并降低平均SICI (p = 0.047)预后差。 Multivariate analysis identified rapid VC decline (p < 0.0001), rapid ALSFRS-R decline (p = 0.0060), and reduced averaged SICI (p = 0.011) as factors independently associated with a shorter survival.Conclusions: Cortical dysfunction appears to be a prognostic marker in patients with ALS within 2 years of disease onset, such that patients with reduced averaged SICI, indicative of intracortical hyperexcitability, demonstrated a worse prognosis.APB=abductor pollicis brevis; ALS=amyotrophic lateral sclerosis; ALSFRS-R=Amyotrophic Lateral Sclerosis Functional Rating Scale–Revised; CI=confidence interval; CMAP=compound muscle action potential; CSP=cortical silent period; FVC=forced vital capacity; HR=hazard ratio; ICF=intracortical facilitation; IQR=interquartile range; ISI=interstimulus interval; MEP=motor evoked potential; RMT=resting motor threshold; SICI=short-interval intracortical inhibition; TMS=transcranial magnetic stimulation; TTTMS=threshold tracking transcranial magnetic stimulation; VC=vital capacity ER -