Frontotemporal white matter changes in amyotrophic lateral sclerosis

Frontotemporal white matter changes in amyotrophic lateral sclerosis
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DOI:
10.1007/s00415-005-0646-x
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发表时间:
2005-03-01
影响因子:
6
通讯作者:
Leigh, PN
Leigh, PN
中科院分区:
医学2区
文献类型:
--
作者:
Abrahams, S;Goldstein, LH;Leigh, PN

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认知功能障碍可能发生在一些患有肌萎缩侧索硬化症(ALS)的患者中,这些患者并没有患有痴呆症。最显著和一致的认知缺陷是通过语言流畅性测试发现的。语言流畅性缺陷的ALS患者使用正电子发射断层扫描(PET)显示功能成像异常主要在额颞区。本研究采用基于体素的结构磁共振成像(MRI)扫描的灰色和白色物质密度的自动体积分析,以探讨非痴呆ALS患者语言流畅性缺陷的结构性大脑变化的潜在模式。将两组ALS患者与健康年龄匹配的对照组(n = 12)进行比较,两组ALS患者根据书面语言流畅性测试(ALSi,认知受损,n = 11; ALSu,认知未受损,n = 12)定义为存在或不存在认知障碍。ALSi组与对照组的比较显示,在广泛的运动和非运动区域,包括对应于额颞联合纤维的区域,白色体积显著减少(p < 0.002)。这些患者表现出相应的执行和记忆功能障碍的认知特征。ALSu组和对照组额叶相关纤维区的白色物质减少较少。白色物质的体积也被发现与记忆测试的表现相关。两组患者与对照组相比,灰质体积均无显著减少。额叶和颞叶结构性白色物质异常可能是以前报道的非痴呆ALS患者认知和功能成像异常的基础。研究结果还表明,ALS患者可能存在运动外结构异常,但没有认知变化的证据。研究结果支持这一假设的一个连续的额外的运动大脑和认知的变化,在这种疾病。
Cognitive dysfunction can occur in some patients with amyotrophic lateral sclerosis (ALS) who are not suffering from dementia. The most striking and consistent cognitive deficit has been found using tests of verbal fluency. ALS patients with verbal fluency deficits have shown functional imaging abnormalities predominantly in frontotemporal regions using positron emission tomography (PET). This study used automated volumetric voxel-based analysis of grey and white matter densities of structural magnetic resonance imaging (MRI) scans to explore the underlying pattern of structural cerebral change in nondemented ALS patients with verbal fluency deficits. Two groups of ALS patients, defined by the presence or absence of cognitive impairment on the basis of the Written Verbal Fluency Test (ALSi, cognitively impaired, n = 11; ALSu, cognitively unimpaired n = 12) were compared with healthy age matched controls (n = 12). A comparison of the ALSi group with controls revealed significantly (p < 0.002) reduced white matter volume in extensive motor and non-motor regions, including regions corresponding to frontotemporal association fibres. These patients demonstrated a corresponding cognitive profile of executive and memory dysfunction. Less extensive white matter reductions were revealed in the comparison of the ALSu and control groups in regions corresponding to frontal association fibres. White matter volumes were also found to correlate with performance on memory tests. There were no significant reductions in grey matter volume in the comparison of either patient group with controls. The structural white matter abnormalities in frontal and temporal regions revealed here may underlie the cognitive and functional imaging abnormalities previously reported in non-demented ALS patients. The results also suggest that extra-motor structural abnormalities may be present in ALS patients with no evidence of cognitive change. The findings support the hypothesis of a continuum of extra-motor cerebral and cognitive change in this disorder.