肌萎缩侧索硬化患者的中脑超声临床研究

Clinical study of midbrain sonographic images of patients with amyotrophic lateral sclerosis

目的 对肌萎缩侧索硬化（ALS）患者进行经颅超声成像（TCS）检查,探讨其中脑黑质回声的特征性表现.方法 入选2015年1-7月在北京大学第三医院神经内科就诊的107例ALS患者（均符合修正E1 Eseorial诊断确诊或拟诊标准）和40名年龄、性别匹配的健康对照,进行TCS检查,进行半定量分级,分别测量中脑及黑质高回声面积,计算双侧黑质高回声总面积/中脑总面积（S/M）比值.比较ALS组和健康对照组黑质高回声面积和S/M比值间的差异,分析ALS组黑质高回声的影像特点.结果 ALS组TCS阳性患者31例（29.0％）,比例显著高于健康对照组（3/40,7.5％）,差异有统计学意义（x2 =22.708,P＜0.01）.ALS组黑质强回声面积（0.40±0.14） cm2,S/M比值9.5％（6.0％,13.0％）,均显著高于健康对照组[（0.20±0.06） cmu2,5.0％（2.0％,6.0％）;t=12.727、Z=16.545,均P＜0.01].TCS分级与患者有无球部症状相关（r=0.386,P＜0.01）,而与年龄、性别、病程以及ALS功能评分量表评分均无相关性（r=0.043、-0.088、0.018、0.202,P=0.251、0.512、0.894、0.190）.结论 ALS患者中脑超声显示有黑质高回声表现,比例高于对照组.

Objective To observe the characteristics of midbrain hyperechogenicity of amyotrophic lateral sclerosis （ALS） patients by transcranial sonography （TCS）.Methods A total of 107 ALS patients,enrolled from January to July 2015 in Beijing University Third Hospital,with the diagnosis of possible or definite ALS according to revised E1 Escorial criteria were examined by transcrinal B-mode sonography.The area of midbrain and substantia nigra and the area ratio of hyper-substantia nigra/midbrain （S/M） were measured and compared between ALS and 40 age-and gender-matched controls.Results There were 29.0% （31/107） of ALS patients and 7.5% （3/40） of controls who displayed abnormal midbrain hyperechogenic areas for groups comparison （x2 =22.708,P 〈 0.01）.The hyperechogenic substantia nigra area and S/M were （0.40 ± 0.14） cm2 and 9.5% （6.0%,13.0%） in ALS group,whereas （0.20 ± 0.06） cm2 and 5.0% （2%,6.0%） in control group respectively,the difference between the two groups being statistically significant （t =12.727,Z =16.545,both P 〈0.01）.No correlations of hyperechogenic area sizes in ALS patients were found in regard to age,gender,ALS duration or ALS Functional Rating Scale score （r=0.043,-0.088,0.018,0.202;P=0.251,0.512,0.894,0.190）.However,there was significant correlation between TCS severity and ALS subtype （bulbar vs spinal form,r =0.386,P 〈 0.01）.Conclusion Hyperechogenicity of the substantia nigra was found in patients with sporadic ALS with a frequency higher than in controls.