[Corneal confocal microscopy detects small-fiber neuropathy in patients with amyotrophic lateral sclerosis].

Objective: To investigate small fiber neuropathy in patients with amyotrophic lateral sclerosis (ALS) by corneal confocal microscopy. Methods: A total of 57 ALS patients were consecutively enrolled in Department of Neurology between June 2015 and February 2016, including 37 men and 20 women with mean age 24-80 (52±11) years. There were 30 controls including 21 men and 9 women with mean age 23-76 (55±13) years. All subjects underwent corneal confocal microscopy (CCM), contact heat evoked potential (CHEP) and skin sympathetic reflection (SSR) to quantify small nerve fiber pathology. Four parameters, such as nerve fiber length (NFL), nerve branch density (NBD), nerve fiber density (NFD) and nerve fiber tortuosity (NFT) were assessed by corneal confocal microscopy. All statistical calculations were conducted using SPSS version 12.0. Results: Compared with control group, corneal nerve fiber length (NFL),nerve fiber density (NFD) were significantly decreased [(12.2±4.4)mm/mm2 vs.(15.1±4.5) mm/mm2,P=0.028;(50.8±24.0)/mm2 vs. (68.3±16.4)/mm2,P=0.002],and nerve fiber tortuosity (NFT) were significantly increased [(2.6±1.0)level vs.(1.0±0.5)level, P<0.01)] in SFN group, while nerve branch density (NBD) were comparable (P=0.700).The course of disease is correlated with NFT (r=0.25,P=0.030). Conclusions: CCM is a new sensitive noninvasive clinical technique that detects early small fiber nerve damage in patients with ALS.目的： 通过角膜共焦显微镜（CCM）评价肌萎缩侧索硬化症患者小纤维病变情况及其诊断价值。 方法： 肌萎缩侧索硬化症患者57例，为2015年6月至2016年2月北京大学第三医院神经内科门诊和住院患者，男性37例、女性20例，年龄24~80（52±11）岁。健康对照组30名，男性21名、女性9名，年龄23~76（55±13）岁。采用CCM量化分析角膜神经纤维的角膜神经纤维长度（NFL），神经分支密度（NBD），神经纤维密度（NFD），神经纤维弯曲度（NFT），并同时进行接触性热痛诱发电位（CHEP），皮肤交感反射（SSR）等检查。计量资料用x¯±s或M（Q1，Q3）表示；计量资料符合正态分布者组间比较用t检验，偏态分布计量资料可用秩和检验等非参数检验，计数资料分析用χ²检验。多因素计量资料相关分析用简单相关分析。所有统计分析采用SPSS 12.0软件处理。 结果： 肌萎缩侧索硬化症组角膜NFL、NFD均显著低于健康对照组［（12.2±4.4）mm/mm2比（15.1±4.5）mm/mm2，P=0.028；（50.8±24.0）个/mm2比（68.3±16.4）个/mm2，P=0.002］，NFT高于对照组［（2.6±1.0）级比（1.0±0.5）级，P<0.01］，差异均有统计学意义。病程与NFT相关（r=0.25，P=0.030）。 结论： 肌萎缩侧索硬化症患者存在明确小纤维病变。CCM对于小纤维神经病的早期诊断有重要价值。.