A prospective study of cognitive impairment in ALS
A prospective study of cognitive impairment in ALS
复制标题
ALS 认知障碍的前瞻性研究
作者：
M. Strong;G. M. Grace;J. Orange;H. A. Leeper;Ravi S. Menon;C. Aere
目的：前瞻性地描述 ALS 患者的认知特征。方法：临床明确的 ALS 患者（11 名男性，2 名女性），年龄 39.9 至 74.0 岁（平均年龄，54.2 ± 9.6 岁；平均病程，21.1 ± 10.5 个月）接受神经心理学、语言和言语测试，然后进行 MR 1H 波谱 (4 T)。五名配偶对照受试者完成了相同的方案。 8 名 ALS 患者参加了为期 6 个月的随访研究。结果：与对照受试者相比，ALS 患者在词语生成、识别记忆（面孔）和无运动视觉感知方面表现出轻度障碍。延髓发作的患者在许多指标（工作记忆、问题解决/认知灵活性、视觉感知以及单词和面孔的识别记忆）方面表现出更大的损伤，并且随着时间的推移，认知损伤似乎更加严重。 ALS 患者在对抗命名测试中表现出贫血，在遵循命令或重复方面没有明显问题。言语运动表现得分和清晰度得分没有显着差异。未观察到用力肺活量、用力呼气量或峰值呼气流速显着下降。尽管在初始测试 (T1) 时正常，但 MR 1H 光谱显示，在两个测试间隔期间，非主要中央前运动带中的 N-乙酰天冬氨酸/肌酸 (NAA/Cr) 比率有所降低。相比之下，在延髓发病患者中，在 T1 时从前扣带回获得的 NAA/Cr 比值已经降低 (p < 0.001)，而在同一区域的肢体发病个体中没有观察到缺陷。结论：患有认知障碍和前扣带回神经元缺失的延髓发作 ALS 患者随后出现更严重的神经心理功能障碍，而语言和言语能力仍然相对保留。值得注意的是，没有延髓体征并不能预示认知能力不会下降。
Objective: To characterize prospectively the cognitive profile in ALS. Methods: Clinically definite ALS patients (11 men, 2 women), age 39.9 to 74.0 years (mean age, 54.2 ± 9.6 years; mean disease duration, 21.1 ± 10.5 months) underwent neuropsychologic, language, and speech testing followed by MR 1H spectroscopy (4 T). Five spousal control subjects completed an identical protocol. Eight ALS patients participated in follow-up studies at a 6-month interval. Results: Relative to control subjects, ALS patients showed mild impairment in word generation, recognition memory (faces), and motor-free visual perception. Bulbar-onset patients showed greater impairment in a number of measures (working memory, problem solving/cognitive flexibility, visual perception, and recognition memory for words and faces), and cognitive impairment appeared more progressive over time. ALS patients demonstrated anomia on a confrontation naming test, with no significant problems following commands or repeating. Speech motor performance scores and intelligibility scores were not significantly different. No significant declines in forced vital capacity, forced expiratory volume, or peak expiratory flow rates were observed. Although normal at initial testing (T1), MR 1H spectroscopy demonstrated a reduction of the N-acetylaspartate/creatine (NAA/Cr) ratio in the nondominant precentral motor strip across the two testing intervals. In contrast, the NAA/Cr ratio obtained from the anterior cingulate gyrus at T1 was already reduced in bulbar-onset patients (p < 0.001), whereas no deficits were observed in limb-onset individuals in the same region. Conclusions: Bulbar-onset ALS patients with cognitive impairments and neuronal loss in the anterior cingulate gyrus subsequently developed more profound neuropsychological dysfunction whereas both language and speech capabilities remained relatively preserved. Of note, the absence of bulbar signs did not predict an absence of cognitive decline.
影响因子：
9.9
作者：
R. Faber
通讯作者：
R. Faber
关岛肌萎缩侧索硬化症/帕金森病-痴呆症复合体的神经原纤维变性。
DOI：
--
发表时间：
1995
期刊：
The American journal of pathology.
影响因子：
--
作者：
Buee-Scherrer,V;Buee,L;Hof,PR;Leveugle,B;Gilles,C;Loerzel,AJ;Perl,DP;Delacourte,A
通讯作者：
Delacourte,A
DOI：
10.1044/jshr.3406.1269
发表时间：
1991-12-01
期刊：
JOURNAL OF SPEECH AND HEARING RESEARCH
影响因子：
--
作者：
KENT, RD;SUFIT, RL;BROOKS, BR
通讯作者：
BROOKS, BR