Chinese Medical Sciences Journal ›› 2018, Vol. 33 ›› Issue (2): 100-106.doi: 10.24920/11812

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  1. 1 解放军总医院海南分院 放射科,海南三亚 572013 中国
    2 解放军总医院 放射科,北京 100853 中国
  • 收稿日期:2017-07-17 出版日期:2018-06-30 发布日期:2018-05-28
  • 通讯作者: 马林

Cortical Thinning Pattern of Bulbar- and Spinal-onset Amyotrophic Lateral Sclerosis: a Surface-based Morphometry Study

Chen Zhiye1,Liu Mengqi1,Ma Lin2,*()   

  1. 1 Department of Radiology, Hainan Branch of Chinese PLA General Hospital, Sanya, hainan 572013, China
    2 Department of Radiology, Chinese PLA General Hospital, Beijing 100853, China
  • Received:2017-07-17 Published:2018-06-30 Online:2018-05-28
  • Contact: Ma Lin


目的 不同发病类型的肌萎缩侧索硬化症患者脑部皮层变薄模态仍没有阐明。本研究旨在采用基于表面的形态测量学方法探索延髓发病型及脊髓发病型肌萎缩侧索硬化症患者脑部皮层变薄特征及阳性脑区的皮层厚度与临床变量的相关性。方法 在3.0T磁共振系统上对65名肌萎缩侧索硬化症患者(15名延髓发病型及50名脊髓发病型)行脑结构MRI成像。采用DARTEL方法将脑结构像分割为灰质及白质,并采用基于投射的皮层厚度方法重建中央皮层表面。采用基于表面的形态测量学方法分别比较延髓型及脊髓型肌萎缩侧索硬化症亚组与正常对照组间皮层厚度的差异,并以年龄与性别作为协变量对异常脑区的皮层厚度与临床特征变量进行相关性分析。结果 肌萎缩侧索硬化症患者皮层变薄脑区位于左侧中央前回、左侧中央后回、右侧直回及右侧内侧中央前回。延髓发病型表现为左侧中央前回运动皮层及右侧辅助运动皮层变薄,脊髓发病型表现为左侧后岛叶及右侧直回运动皮层外皮层变薄。在肌萎缩侧索硬化症患者,右侧直回皮层厚度与病程呈负相关(r =-0.311, P = 0.013),右侧中央前回皮层厚度与肌萎缩侧索硬化症功能评分(ALSFRS-R)呈正相关(r = 0.271, P = 0.032)。延髓发病型患者运动皮层厚度与肌萎缩侧索硬化症病程及功能评分不相关,而脊髓型患者运动皮层外皮层厚度与病程呈负相关(左岛叶, r=-0.409, P=0.004; 右直回 r=-0.351, P=0.014)。结论 本研究结果证实延髓发病型肌萎缩侧索硬化症表现为双侧运动皮层变薄,脊髓发病型肌萎缩侧索硬化症表现为运动皮层外皮层变薄。运动皮层变薄可能与疾病致残的内在病理生理变化相关,而运动皮层外皮层变薄可能与病程相关的继发性病理生理改变相关。

关键词: 肌萎缩侧索硬化症, 皮层厚度, 磁共振成像, 基于表面的形态测量学


Objective The precise cortical thinning pattern has not been elucidated for onset subtypes of amyotrophic lateral sclerosis (ALS). The current study aimed to investigate the altered cortical thickness signatures of the bulbar-onset and spinal-onset ALS using surface-based morphometry, and the correlation between the cortical thickness of positive brain regions and clinical variables of the patients.Methods MR structural images were obtained from 65 ALS patients (15 bulbar-onset, 50 spinal-onset) and 65 normal controls (NCs) on a 3.0T MRI system. The structural images were segmented into gray matter and white matter based on DARTEL method and the central cortical surfaces were reconstructed using projection-based thickness method. The surface-based morphometry was performed to identify the alteration of cortical thickness in overall ALS patients, bulbar-onset ALS patients and spinal-onset ALS patients comparing to the NCs. The correlation analysis was applied between the clinical variables and the mean cortical thickness of the abnormal brain regions with age and sex as covariates.Results The cortex thinning of ALS patients was located in the left precentral gyrus, left postcentral gyrus, right gyrus rectus and right medial precentral gyrus. The bulbar-onset ALS (ALS-bulbar) presented motor cortex thinning of left precentral gyrus and right supplementary motor cortex, and the spinal-onset ALS (ALS-spinal) suffered from extra-motor cortex thinning of left posterior insula and right gyrus rectus. In ALS patients, the thickness of right gyrus rectus was negatively correlated to disease duration (r=-0.311, P=0.013), the thickness of right precentral gyrus was positively correlated to the score of ALS functional rating score-revise (ALSFRS-R) (r=0.271, P=0.032). The thickness of motor cortices in ALS-bulbar were not correlated to disease duration and ALSFRS-R score; the thickness of extra-motor cortices in ALS-spinal were negatively correlated to the disease duration (left insula, r=-0.409, P=0.004; right gyrus rectus, r=-0.351, P=0.014).Conclusion The findings suggested that bilateral motor cortex thinning presented in bulbar-onset ALS and extra-motor cortex thinning presented in spinal-onset ALS. The motor cortex thinning may be the intrinsic pathophysiological change that associated to the disease disability, and extra-motor cortex thinning may be secondary pathophysiological change that associated to disease duration.

Key words: amyotrophic lateral sclerosis, cortical thickness, magnetic resonance imaging, surface-based morphometry

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