肌萎缩侧索硬化症患者延髓区域 MRI 皮质厚度特点及对生存的影响. (Chinese)

Objective To investigate cortical thickness changes in the face-head region of the primary motor cortex (PMC) and its effect on survival in amyotrophy lateral sclerosis (ALS) patients. Methods A retrospective analysis was performed on 105 ALS patients who underwent head MRI scan at the same time. The A4hf (face-head) region of PMC was used as the region of interest (ROI). According to clinical symptoms, patients were divided into two groups: bulbar involvement and non-bulbar involvement. The differences of clinical features and cortical thickness in ROI were analyzed. According to the symptoms of bulbar palsy, physical examination of nervous system and EMG of tongue muscle, the patients with bulbar palsy were divided into lower motor neuron (LMN), upper motor neuron (UMN) and LMN+UMN groups. The differences of bulbar subgroup score and ROI of cortical thickness were analyzed. Age at onset, body mass index, delayed time of diagnosis, bulbar subgroup score, and ROI cortical thickness were included in survival analysis. Results (1) The ROI cortical thickness was significantly lower in bulbar involvement group than non-bulbar involvement group (-0.198±0.87 vs. 0.235±0.95, P=0.017). (2) There were no significant differences in the bulbar subgroup scores or cortical thickness of ROI between LMN, UMN and LMN+UMN groups (P＞0.05). (3) Survival analysis showed age of onset (HR=3.296, 95% CI:1.63-6.664, P=0.001), delayed time of diagnosis (HR=0.361, 95% CI:0.184-0.705, P=0.003), bulbar subgroup score (HR 0.389, 95% CI:0.174-0.868, P=0.021), and ZRE＿ROI cortical thickness (HR=2.309, 95% CI:1.046-5.096, P=0.038) were independent influencing factors of ALS survival. Conclusion Cortical thickness in A4hf (face-head) region can more objectively reflect UMN signs of region bulbar. In addition to age of onset and delayed time of diagnosis, bulbar subgroup score and cortical thickness of face-head region are also independent influencing factors, and cortical thinning in face-head region is a protective factor for survival of ALS patients. [ABSTRACT FROM AUTHOR]

目的 探讨肌萎缩侧索硬化症（amyotrophy lateral sclerosis, ALS）患者初级运动皮层（primary motor cortex, PMC）的face-head区域皮质厚度变化特点及对生存期的影响。方法 对同时行头颅MRI扫描的105例ALS患者回顾性分析, PMC区A4hf（face-head）区域作为兴趣区域（region of interest, ROI）,根据患者临床症状分为延髓受累和未受累两组, 分析临床特点和ROI区域的皮质厚度在两组间的差异；根据患者延髓麻痹症状、神经系统查体和舌肌肌电图, 将延髓受累ALS分为下运动神经元（lower motor neuron, LMN）、上运动神经元（upper motor neuron, UMN）和LMN+UMN共3组, 分析延髓亚组评分和ROI皮质厚度在3组间的差异；将起病年龄、体质量指数、诊断延迟时间、延髓亚组评分和ROI皮质厚度纳入生存分析。结果 （1）延髓受累组ROI皮质厚度明显小于延髓未受累组（-0.198±0.87 vs. 0.235±0.95, P=0.017）;（2）延髓亚组评分和ROI皮质厚度在LMN、UMN和LMN+UMN三组间无统计学差异（P＞0.05）;（3）生存分析示起病年龄（HR=3.296, 95%CI:1.63～6.664,P=0.001）、诊断延迟时间（HR=0.361, 95%CI:0.184～0.705, P=0.003）、延髓亚组评分（HR=0.389, 95%CI:0.174～0.868,P=0.021）及ZRE＿ROI皮质厚度（HR=2.309, 95%CI:1.046～5.096, P=0.038）均是ALS患者生存期的独立影响因素。结论 大脑运动前回A4hf（face-head）区域的皮质厚度可更为客观反映延髓UMN体征。影响ALS患者生存期的因素除了起病年龄和诊断延迟时间, 延髓亚组评分和face-head区域的皮质厚度也是其独立影响因素, 且face-head区域皮质变薄是ALS患者生存期的保护因素。 [ABSTRACT FROM AUTHOR]

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