Relationship between Clinical Parameters and Brain Structure in Sporadic Amyotrophic Lateral Sclerosis Patients According to Onset Type: A Voxel-Based Morphometric Study.
Relationship between Clinical Parameters and Brain Structure in Sporadic Amyotrophic Lateral Sclerosis Patients According to Onset Type: A Voxel-Based Morphometric Study.
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DOI：
10.1371/journal.pone.0168424
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发表时间：
2017
期刊：
影响因子：
3.7
通讯作者：
Kim SH
中科院分区：
文献类型：
--
作者：
Kim HJ;de Leon M;Wang X;Kim HY;Lee YJ;Kim YH;Kim SH
肌萎缩侧索硬化症（ALS）是一种进展迅速，表型异质性的神经退行性疾病，主要影响运动神经元系统。目前基于体素的形态测定（VBM）研究调查了散发性ALS亚型之间的脑萎缩模式是否不同。研究纳入了认知功能正常的散发性ALS患者（n = 62）和年龄匹配的健康对照组（n = 57）。ALS患者根据症状发作时的临床表现分为肢体和延髓发作组（分别为48例和14例）。临床指标为ALS功能评定量表修订版（ALSFRS-R）评分、疾病持续时间和用力肺活量（FVC）。通过VBM比较ALS亚组之间的脑萎缩模式。在肢体型ALS患者中，萎缩主要局限于运动皮质和邻近的前中央和后中央区域。然而，在延髓发作组中，受累区域更广泛，包括这些相同的区域，但也延伸到双侧额颞和左侧上级颞回和缘上回，多元回归分析显示，他们的ALSFRS-R评分与广泛的灰质丢失相关，而FVC与左侧上级颞回皮质下区域的萎缩相关。在肢体型ALS患者中，病程与运动区和邻近区域的萎缩程度有关。散发性ALS亚型显示不同的脑萎缩模式。神经网络相关的肢体和延髓运动功能，在每一个ALS亚型可能是他们的大脑萎缩的不同模式的基础。也就是说，更广泛的皮质和皮质下萎缩与更大的ALSFRS-R严重程度和更短的病程在球发作亚型，并可能解释这些患者的预后不良。
Amyotrophic lateral sclerosis (ALS) is a rapidly progressing, phenotypically heterogeneous neurodegenerative disease affecting mainly the motor neuron system. The present voxel-based morphometry (VBM) study investigated whether patterns of brain atrophy differ among sporadic ALS subtypes. Sporadic ALS patients (n = 62) with normal cognition and age-matched healthy controls (n = 57) were included in the study. ALS patients were divided into limb- and bulbar-onset groups according to clinical manifestations at symptom onset (n = 48 and 14, respectively). Clinical measures were ALS Functional Rating Scale-Revised (ALSFRS-R) score, disease duration, and forced vital capacity (FVC). Patterns of brain atrophy between ALS subgroups were compared by VBM. In limb-onset ALS patients, atrophy was largely confined to the motor cortex and adjacent pre- and postcentral regions. However, in the bulbar-onset group, affected regions were more widespread and included these same areas but also extended to the bilateral frontotemporal and left superior temporal and supramarginal gyri, and multiple regression analysis revealed that their ALSFRS-R scores were associated with extensive loss of gray matter while FVC was related to atrophy in subcortical regions of the left superior temporal gyrus. In limb-onset ALS patients, disease duration was related to the degree of atrophy in the motor and adjacent areas. Sporadic ALS subtypes show different patterns of brain atrophy. Neural networks related to limb and bulbar motor functions in each ALS subtype may underlie their distinct patterns of cerebral atrophy. That is, more extensive cortical and subcortical atrophy is correlated with greater ALSFRS-R severity and shorter disease duration in the bulbar-onset subtype and may explain the poor prognosis of these patients.