Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia
Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia
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DOI：
10.1093/brain/awr179
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发表时间：
2011-09-01
期刊：
影响因子：
14.5
通讯作者：
Miller, Bruce L.
中科院分区：
文献类型：
--
作者：
Rascovsky, Katya;Hodges, John R.;Miller, Bruce L.
根据最近的文献和集体经验，一个国际联盟制定了诊断行为变异性额颞叶痴呆的修订指南。验证过程回顾性地审查了临床记录，并比较了在多个地点样本的病理证实的额颞叶变性患者的建议和早期标准的敏感性。根据修订后的标准，“可能的”行为变异额颞叶痴呆症需要六个临床区分特征中的三个（去抑制、冷漠/惰性、同情/同理心丧失、持续/强迫行为、口欲亢进和执行障碍神经心理学特征）。“可能的”行为变异型额颞叶痴呆增加了功能障碍和特征性神经影像学，而“具有明确的额颞叶变性”的行为变异型额颞叶痴呆需要组织病理学确认或致病突变。16个脑库提供的病例符合额颞叶变性的组织病理学标准，临床诊断为行为变异性额颞叶痴呆、阿尔茨海默病、路易体痴呆或血管性痴呆。主要原发性进行性失语或锥体外系综合征的病例被排除。在这些尸检确认的病例中，经验丰富的神经科医生或精神科医生在就诊时根据之前和拟议的标准确定了做出诊断所需的临床特征。在137例特征可用于建议和先前建立的标准的病例中，118例（86%）符合“可能”标准，104例（76%）符合“可能”行为变异性额颞叶痴呆的标准。相反，72例（53%）符合先前建立的综合征标准（与“可能”和“很可能”标准相比，P < 0.001）。不符合修订标准的患者年龄明显较大，大多数具有明显记忆障碍的非典型表现。总之，修订后的标准，行为变异性额颞叶痴呆的诊断准确性提高与以前建立的标准相比，在一个样本与已知的额颞叶变性。更高的敏感性，建议的标准可能反映了优化的诊断功能，较少限制性的排除功能和灵活的结构，以适应不同的初始临床表现。未来的研究将需要建立这些修订后的诊断指南的可靠性和特异性。
Based on the recent literature and collective experience, an international consortium developed revised guidelines for the diagnosis of behavioural variant frontotemporal dementia. The validation process retrospectively reviewed clinical records and compared the sensitivity of proposed and earlier criteria in a multi-site sample of patients with pathologically verified frontotemporal lobar degeneration. According to the revised criteria, 'possible' behavioural variant frontotemporal dementia requires three of six clinically discriminating features (disinhibition, apathy/inertia, loss of sympathy/empathy, perseverative/compulsive behaviours, hyperorality and dysexecutive neuropsychological profile). 'Probable' behavioural variant frontotemporal dementia adds functional disability and characteristic neuroimaging, while behavioural variant frontotemporal dementia 'with definite frontotemporal lobar degeneration' requires histopathological confirmation or a pathogenic mutation. Sixteen brain banks contributed cases meeting histopathological criteria for frontotemporal lobar degeneration and a clinical diagnosis of behavioural variant frontotemporal dementia, Alzheimer's disease, dementia with Lewy bodies or vascular dementia at presentation. Cases with predominant primary progressive aphasia or extra-pyramidal syndromes were excluded. In these autopsy-confirmed cases, an experienced neurologist or psychiatrist ascertained clinical features necessary for making a diagnosis according to previous and proposed criteria at presentation. Of 137 cases where features were available for both proposed and previously established criteria, 118 (86%) met 'possible' criteria, and 104 (76%) met criteria for 'probable' behavioural variant frontotemporal dementia. In contrast, 72 cases (53%) met previously established criteria for the syndrome (P < 0.001 for comparison with 'possible' and 'probable' criteria). Patients who failed to meet revised criteria were significantly older and most had atypical presentations with marked memory impairment. In conclusion, the revised criteria for behavioural variant frontotemporal dementia improve diagnostic accuracy compared with previously established criteria in a sample with known frontotemporal lobar degeneration. Greater sensitivity of the proposed criteria may reflect the optimized diagnostic features, less restrictive exclusion features and a flexible structure that accommodates different initial clinical presentations. Future studies will be needed to establish the reliability and specificity of these revised diagnostic guidelines.