Dataset Information

The Awaji criteria increases the diagnostic sensitivity of the revised El Escorial criteria for amyotrophic lateral sclerosis diagnosis in a Chinese population.

ABSTRACT:

Objectives

The accurate and early diagnosis of amyotrophic lateral sclerosis (ALS) is important for extending the life expectancy of patients. However, previous studies that have assessed the diagnostic sensitivities of the Awaji criteria (AC) and the revised El Escorial criteria (rEEC) in patients with ALS have been inconsistent, most of them were consensual regarding the advantage of Awaji over conventional criteria. Our study sought to compare the roles of AC and rEEC in the diagnosis of ALS.

Methods

Data from a total of 294 consecutive patients with ALS were collected between January 2014 and August 2015 in the Peking Union Medical College Hospital. The clinical and electrophysiological records of 247 patients were eventually analyzed. The primary outcome measures were the sensitivities of the AC and rEEC for the diagnosis of ALS.

Results

The sensitivity of probable or definite ALS as diagnosed with the AC (78%) was greater than that of the rEEC (36%, P <0.001). Following the application of the AC, 103 of the 147 patients categorized as probable ALS-laboratory supported from the rEEC were upgraded to probable or definite ALS, and 44 were downgraded to possible ALS.

Conclusions

Our data demonstrated that the AC exhibited greater diagnostic sensitivity than the rEEC in a Chinese ALS population. The use of the AC should be considered in clinical practice.

SUBMITTER: Li DW

PROVIDER: S-EPMC5332033 | biostudies-literature | 2017

REPOSITORIES: biostudies-literature

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Publications

The Awaji criteria increases the diagnostic sensitivity of the revised El Escorial criteria for amyotrophic lateral sclerosis diagnosis in a Chinese population.

Li Da-Wei DW Liu Mingsheng M Cui Bo B Fang Jia J Guan Yu-Zhou YZ Ding Qingyun Q Li Xiaoguang X Cui Liying L

PloS one 20170301 3

<h4>Objectives</h4>The accurate and early diagnosis of amyotrophic lateral sclerosis (ALS) is important for extending the life expectancy of patients. However, previous studies that have assessed the diagnostic sensitivities of the Awaji criteria (AC) and the revised El Escorial criteria (rEEC) in patients with ALS have been inconsistent, most of them were consensual regarding the advantage of Awaji over conventional criteria. Our study sought to compare the roles of AC and rEEC in the diagnosis ...[more]

PMID: 28249004

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Project description: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.

Dataset Information

The Awaji criteria increases the diagnostic sensitivity of the revised El Escorial criteria for amyotrophic lateral sclerosis diagnosis in a Chinese population.

Objectives

Methods

Results

Conclusions

Publications

The Awaji criteria increases the diagnostic sensitivity of the revised El Escorial criteria for amyotrophic lateral sclerosis diagnosis in a Chinese population.

Similar Datasets

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