Frontal lobe dysfunction in amyotrophic lateral sclerosis - A PET study

Frontal lobe dysfunction in amyotrophic lateral sclerosis - A PET study
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DOI:
10.1093/brain/119.6.2105
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发表时间:
1996-12-01
期刊:
影响因子:
14.5
通讯作者:
Leigh, PN
Leigh, PN
中科院分区:
医学1区
文献类型:
--
作者:
Abrahams, S;Goldstein, LH;Leigh, PN

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PET测量区域脑血流(rCBF)用于探讨肌萎缩侧索硬化症(ALS)的额叶功能障碍。采用执行额叶功能(言语流畅性)激活范式,对比单词生成和单词重复过程中的rCBF。根据存在或不存在认知障碍(ALSi)定义的两组ALS患者(受损,n = 6; ALSu,未受损,n = 6)与年龄匹配的健康对照组(n = 6)进行比较。患者的选择是基于先前口头流畅性书面测试的表现。对患者的额外神经心理学评估显示,只有ALSi组存在执行和记忆功能障碍,在内在生成测试中存在明显缺陷。ALSi患者在皮层和皮层下区域表现出显著(P < 0.001)的激活受损,包括背外侧前额叶皮质(DLPFC, 46区和9区)、外侧运动前皮质(8区和6区)、内侧前额叶和运动前皮质(8区和9区)、双侧岛叶皮质和丘脑前核复核。虽然三组在扫描范式上表现出相同的单词生成性能,但ALSu组表现出相对未受损的激活模式。这些结果支持在一些非痴呆性ALS患者中存在运动外神经元的参与,特别是沿丘脑-额叶关联通路。此外,本研究提示一些伴有认知障碍的ALS患者存在DLPFC功能障碍。
PET measurements of regional cerebral blood flow (rCBF) were used to explore frontal lobe dysfunction in amyotrophic lateral sclerosis (ALS). An activation paradigm of executive frontal lobe function (verbal fluency), which contrasted rCBF during word generation and word repetition, was used. Two groups of ALS patients, defined by the presence or absence of cognitive impairment (ALSi) (impaired, n = 6; ALSu, unimpaired, n = 6) were compared with healthy age-matched controls (n = 6). Patient selection was based on prior performance on a written test of verbal fluency. Additional neuropsychological assessment of the patients revealed evidence of executive and memory dysfunction in the ALSi group only, with marked deficits in tests of intrinsic generation. The ALSi patients displayed significantly (P < 0.001) impaired activation in cortical and subcortical regions including the dorsolateral prefrontal cortex (DLPFC; areas 46 and 9), lateral premotor cortex (areas 8 and 6), medial prefrontal and premotor cortices (areas 8 and 9), insular cortex bilaterally and the anterior thalamic nuclear complex. Although the three groups showed matched word generation performance on the scanning paradigm, the ALSu group displayed a relatively unimpaired pattern of activation. These results support the presence of extra-motor neuronal involvement, particularly along a thalamo-frontal association pathway, in some non-demented ALS patients. In addition, this study suggests dysfunction of DLPFC in some ALS patients with associated cognitive impairments.