Rascovsky K, Hodges JR, Knopman D, et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia

Department of Neurology, Perelman School of Medicine, University of Pennsylvania, 3400 Spruce Street, 3 West Gates, Philadelphia, PA 19104, USA.
Brain (Impact Factor: 9.2). 08/2011; 134(Pt 9):2456-77. DOI: 10.1093/brain/awr179
Source: PubMed


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.

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Available from: Patricia Lillo, Oct 04, 2015
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    • "Vascular lesions were considered when the score was 5 or 6 out of 6 (i.e., a lesion greater than 11 mm or confluent lesions). An etiologic diagnosis of the dementia for each patient was made at the end of the follow-up of 5.0 ± 2.9 years (mean ± SD), using Dubois' criteria for AD [6], McKeith's criteria for Lewy body disease (LBD) [7], and Rascovsky's criteria for frontotemporal dementia [8]. Diagnosis of hippocampal sclerosis was made using clinical arguments and Barkhof's MRI scale of the hippocampus with a score of 4/4 [9]. "
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    • "Seventy participants were recruited at the Alzheimer Institute of the Pitié-Salpêtrière Hospital in Paris (France), resulting in 30 AD patients, 20 bvFTD patients, and 20 healthy controls. Patients fulfilled consensual diagnostic criteria for bvFTD (Rascovsky et al., 2011) or AD (Albert et al., 2011; Dubois et al., 2007). All patients were followed at least 24 month to increase the clinical confidence of the diagnosis. "
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    ABSTRACT: The clinical differential diagnosis of Alzheimer's disease (AD) and behavioral-variant frontotemporal dementia (bvFTD) can no longer rely only on episodic memory impairment or executive dysfunctions, as highlighted by recent findings showing that both diseases could present with similar impairments. Objective cognitive tests assessing specific symptoms, such as impulsivity in bvFTD, are thus crucially needed. The aim of this study was to evaluate the differences in impulsivity between bvFTD and AD using a delay-discounting paradigm. An ecological delay-discounting test was administrated to 70 participants including 30 ADs, 20 bvFTD and 20 controls. AD patients were divided according to the severity of the disease into mild or moderate group. The delay-discounting score, reflecting the total percentage of impulsive choice across the entire task, was analyzed for each group. This score showed that bvFTD patients were significantly more impulsive than controls and AD patients at mild or moderate stage. AD patients, regardless of disease stage, did not differ from controls. ROC analyses revealed high and significant area under the curve (AUC, 95% confidence interval) for this score to differentiate bvFTD from AD (0.704) or controls (0.904), or both group (AD + controls; AUC = 0.791). The total delay-discounting score provided by our task showed that it could accurately differentiate bvFTD patients from AD and controls. These results support the relevancy of using tests inspired by experimental psychoeconomics and taping into reward processing to increase the distinction between both diseases. (PsycINFO Database Record (c) 2015 APA, all rights reserved).
    Neuropsychology 04/2015; DOI:10.1037/neu0000197 · 3.27 Impact Factor
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    • "Examining face and object memory in frontotemporal dementia. Neuropsychologia (2015), this information, patients were classified according to current consensus diagnostic criteria for bvFTD or SD (Gorno-Tempini et al., 2011; Rascovsky et al., 2011). In line with previous studies, individuals with SD were classified by the clinical team as either left-or right-lateralised based on their coronal MRI brain scan of the temporal lobes and their clinical presentation (e.g., Kamminga et al., in press). "
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    ABSTRACT: The ability to perceive, learn and recognise faces is a complex ability, which is key to successful social interactions. This ability is proposed to be coordinated by neural regions in the occipital and temporal lobes, specialised for face perception and memory. While previous studies have suggested that memory for faces is compromised in some dementia syndromes, it remains unclear whether this simply reflects more generalised memory deficits. Here, we examined basic face perception (Identity-Matching), face recognition (Cambridge Face Memory Task) and object recognition (Cambridge Car Memory Task) in 11 semantic dementia (SD) patients (8 left-lateralised, 3 right-lateralised) and 13 behavioural-variant frontotemporal dementia (bvFTD) patients, compared with 11 controls. On the Identity-Matching task, bvFTD were impaired compared to controls, with a similar trend observed in the SD group. Importantly, both bvFTD and SD also demonstrated impaired face recognition. In contrast, only bvFTD showed impaired object recognition, with SD performing within normal limits on this task. Voxel-based morphometry analyses revealed that Identity-Matching and face recognition were associated with partly dissociable regions including the fusiform cortex and anterior temporal lobe. Object-memory was associated with thalamic integrity in the bvFTD group only. These results reveal that face perception and face memory deficits are common in bvFTD and SD, and have been previously underestimated. These deficits are due to neurodegeneration of key regions within the'core' and'extended' face processing system, providing convergent evidence of the neural regions supporting face perception. From a clinical perspective, impaired ability to recognise faces is common in bvFTD and SD and therefore strategies to improve face perception and memory may be beneficial for these patients. Copyright © 2015. Published by Elsevier Ltd.
    Neuropsychologia 03/2015; 71. DOI:10.1016/j.neuropsychologia.2015.03.020 · 3.30 Impact Factor
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