Continuum of frontal lobe impairment in amyotrophic lateral sclerosis
Continuum of frontal lobe impairment in amyotrophic lateral sclerosis
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DOI：
10.1001/archneur.64.4.530
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发表时间：
2007-04-01
影响因子：
--
通讯作者：
Lomen-Hoerth, Catherine
中科院分区：
文献类型：
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作者：
Murphy, Jennifer M.;Henry, Roland G.;Lomen-Hoerth, Catherine
目的：确定肌萎缩侧索硬化症 (ALS) 患者认知和行为异常的性质和患病率。设计：临床特征调查。地点：大学医学中心内的多学科诊所。患者：连续招募 30 名新 ALS 患者作为志愿者样本。在受邀者中，有 23 名参与者（20 名散发性 ALS 和 3 名家族性 ALS）报名。参与者年龄从 27 岁到 80 岁（平均年龄 56.5 岁）；受教育程度为12至21年（平均受教育程度为大学3.5年）； 17 名参与者 (74%) 为男性。主要结果指标：神经心理学测试、神经行为访谈和结构化磁共振成像。结果：患者被分为额颞叶变性亚型 (n = 5)、疑似阿尔茨海默病 (n = 1)、认知障碍亚型 (n = 2)、行为障碍 (n = 4) 以及认知和行为正常 (n =) 11）。五项神经心理学测试、两项行为异常和右半球灰质减少将患者分为正常组和异常组。结论：在该样本中，相当大比例的 ALS 患者具有一系列额颞叶损伤的行为和认知变化。右半球萎缩可能是 ALS 患者认知障碍的生物标志物。
Objective: To identify the nature and prevalence of cognitive and behavioral abnormalities in patients with amyotrophic lateral sclerosis (ALS).Design: Survey of clinical characteristics.Setting: Multidisciplinary clinic within a university medical center.Patients: A volunteer sample of 30 new patients with ALS were recruited consecutively. Of those invited, 23 participants (20 with sporadic ALS and 3 with familial ALS) enrolled.Participants ranged in age from 27 to 80 years (mean age, 56.5 years); the education level ranged from 12 to 21 years (mean education level, 3.5 years of college); and 17 participants (74%) were male.Main Outcome Measures: Neuropsychological tests, neurobehavioral interviews, and structured magnetic resonance imaging.Results: Patients were classified into subtypes of frontotemporal lobar degeneration (n = 5), suspected Alzheimer disease (n = 1), and subthreshold variants of cognitive impairment (n = 2), behavioral impairment (n = 4), and cognitively and behaviorally normal (n = 11). Five neuropsychological tests, 2 behavioral abnormalities, and right hemisphere gray matter reductions differentiated patients into normal and abnormal groups.Conclusions: In this sample, a sizable proportion of patients with ALS possess a range of behavioral and cognitive changes that lie on a spectrum of frontotemporal impairment. Right hemisphere atrophy may be a biomarker for cognitive impairment in patients with ALS.