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首页> 外文期刊>Archives of neurology. >Neurobehavioral features in frontotemporal dementia with amyotrophic lateral sclerosis.
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Neurobehavioral features in frontotemporal dementia with amyotrophic lateral sclerosis.

机译：神经行为功能额颞叶痴呆与肌萎缩性脊髓侧索硬化症。

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OBJECTIVE: To compare the clinical features at presentation in patients with frontotemporal dementia (FTD) who develop amyotrophic lateral sclerosis (ALS) with those of patients with behavioral variant FTD (bvFTD) who do not develop ALS. DESIGN: Archival data analysis on 61 deceased patients with FTD. We reviewed the clinical features at presentation (behavioral changes, psychotic symptoms, language, and executive and memory problems) and survival. SETTING: Early Onset Dementia Clinic, Cambridge, England. Patients From a total of 156 patients with a clinical diagnosis of behavioral FTD, we selected 61 deceased patients with comprehensive medical records, including 43 with bvFTD and 18 with FTD/ALS. MAIN OUTCOME MEASURES: Clinical features and survival. RESULTS: There was a significant association between the presence of delusions (50%; odds ratio, 4.4; 95% confidence interval, 1.3-14.5) and diagnosis of FTD/ALS (n = 18), whereas the behavioral features were identical in both groups. The interval between the onset of behavioral changes and diagnosis of ALS was less than 2 years in 12 (67%) of the patients with FTD/ALS. The median survival from symptom onset was significantly shorter for the FTD/ALS group (2.4 years; 95% confidence interval, 1.8-3.0 years) than for the bvFTD group (6.6 years; 5.6-7.6 years). CONCLUSIONS: Delusions are particularly common in patients who develop FTD/ALS. The occurrence of delusions in the context of behavioral FTD should lead to an early search for ALS features.
机译：目的:比较的临床特征表示患者额颞叶肌萎缩性脊髓侧索痴呆(FTD)发展硬化(ALS)的患者行为变异FTD (bvFTD)不发展肌萎缩性侧索硬化症。已故FTD患者。临床特征表现(行为变化,精神病症状,语言,和行政和内存问题)和生存。设置:早发性痴呆诊所,剑桥,英格兰。我们的临床诊断行为FTD,选择61死去的患者全面医疗记录,包括43 bvFTD和18FTD /肌萎缩性侧索硬化症。特性和生存。重要的存在之间的联系妄想(50%;区间,1.3 - -14.5)和诊断的FTD /肌萎缩性侧索硬化症(n =18),而行为特征两组相同。的行为变化和诊断ALS是小于2年12 (67%)FTD / ALS患者。症状出现明显的短FTD / ALS组(2.4年;区间,1.8 - -3.0年)比bvFTD组(6.6年;在病人妄想尤其常见开发FTD /肌萎缩性侧索硬化症。的环境行为FTD应该导致一个早期的搜索ALS的特性。

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