We evaluated the Addenbrooke's cognitive examination (ACE), a simple instrument to differentiate frontotemporal dementia (FTD) from Alzheimer's disease (AD), in our dementia patients clinic population. The Verbal-Language/Orientation-Memory (VLOM) ratio, which compares its language and memory scores, determines whether FTD or AD is more likely. The ACE was translated into French with adaptation maintaining the number of words in the name and address learning and delayed recall test, and with cultural adaptation for the semantic memory. The 85 included subjects had no evidence of two or more organic pathologies, after at least six months of follow-up, and an MMSE score>or=20/30. Patients with cognitive impairment due to alcohol intake were excluded. The diagnosis of a specific dementing illness was based on the consensus of the neurologist and neuropsychologists in the team. Thereafter, another neurologist expert in dementia, blinded to the ACE result and to the diagnosis and treatment, reviewed all cases files and proposed a diagnosis. A diagnostic agreement was reached for 79 cases (92.9%) with 40 (50.6%) dementia: 25 AD (62.5 %), 9 FTD (22.5 %). We estimated that the sensitivity for detecting dementia of an ACE score3.2 was 72%,with a specificity of 69.4%. We conclude that, when used as originally proposed, ACE is very accurate for the detection of dementia, but much less effective in discriminating the most common frontal variant of FTD.

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Human brain activity is intrinsically organized into resting-state networks (RSNs) that transiently activate or deactivate at the sub-second timescale. Few neuroimaging studies have addressed how Alzheimer's disease (AD) affects these fast temporal brain dynamics, and how they relate to the cognitive, structural and metabolic abnormalities characterizing AD. We aimed at closing this gap by investigating both brain structure and function using magnetoencephalography (MEG) and hybrid positron emission tomography-magnetic resonance (PET/MR) in 10 healthy elders, 10 patients with subjective cognitive decline (SCD), 10 patients with amnestic mild cognitive impairment (aMCI) and 10 patients with typical Alzheimer’s disease with dementia (AD). The fast activation/deactivation state dynamics of RSNs were assessed using hidden Markov modeling (HMM) of power envelope fluctuations at rest measured with MEG. Correlations were sought between temporal properties of HMM states and participants' cognitive test scores, whole hippocampal grey matter volume and regional brain glucose metabolism. The posterior default-mode network (DMN) was less often activated and for shorter durations in AD patients than matched healthy elders. No significant difference was found in patients with SCD or aMCI. The time spent by participants in the activated posterior DMN state did not correlate significantly with cognitive scores, nor with the whole hippocampal volume. However, it correlated positively with the regional glucose consumption in the right dorsolateral prefrontal cortex (DLPFC). AD patients present alterations of posterior DMN power activation dynamics at rest that identify an additional electrophysiological correlate of AD-related synaptic and neural dysfunction. The right DLPFC may play a causal role in the activation of the posterior DMN, possibly linked to the occurrence of mind wandering episodes. As such, these data might suggest a neural correlate of the decrease in mind wandering episodes reported in pathological aging.

We evaluated the sensitivity and specificity of our French version of Addenbrooke’s cognitive examination (ACE) to detect dementia in our patient population. One hundred and fifty-eight cases were included in the study. In our patient series, the sensitivity for diagnosing dementia with a Mini-Mental State Examination (MMSE) score of ≤24/30 was 48.5%, the sensitivity of an MMSE score of ≤27/30 was 82.5% with a specificity of 72.1%, the sensitivity of an ACE score of ≤83/100 was 86.6% with a specificity of 70.5% and the sensitivity of an ACE score of ≤88/100 was 97.9% with a specificity of 59%. We conclude that the French version of the ACE is a very accurate test for the detection of dementia, and should be widely used in clinical practice.

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We report a single case study of a brain-damaged patient, ER, who showed a remarkably consistent category-specific deficit for living things. His impairment was observed across tasks (naming, definition, matching, drawing from memory, questionnaires), input modalities (visual, verbal, nonverbal auditory), and output modalities (verbal vs. pointing or visual matching responses) as well as for different types of knowledge. Although visual knowledge of living things was severely affected, his category-specific impairment in nonverbal sound recognition is inconsistent with models of category-specific deficits based on pre-semantic visual descriptions. ER's deficit cannot fully be explained by item typicality, word frequency, visual complexity, homomorphy, age of acquisition, value to perceiver, or modality of transaction. Furthermore, in ER, contextual cues were even slightly detrimental for the recognition of animals. ER's naming and recognition errors were constrained by the categorical structure of the knowledge base: In most cases they respected both the second-and first-order superordinates. In particular, ER's knowledge of shared categorical properties related to biological function was almost spared. This result is compatible with the idea that, for living things, shared functional properties and shared perceptual properties are strongly correlated. Feature-based models assuming perceptual vs. functional semantic components cannot account for ER's deficit, since for living things he was impaired on both kinds of features to a similar extent. ER's behaviour is quite consistent with the notion that conceptual knowledge is organised categorically in the brain, with one or several specialised subsystems for biologically related entities. 301Requests for reprints should be addressed to Régine Kolinsky, Unité de Recherche en Neurosciences Cognitives (UNESCOG), Université Libre de Bruxelles, CP 191, Av. F.D. Roosevelt, 50, B-1050 Brussels, Belgium (Fax: 32-2-650.22.09; Email: rkolins@ulb.ac.be).We wish to thank Tim Shallice for very helpful discussions on a former version of the manuscript, as well as the Editor and two anonymous reviewers for their very constructive comments. We also thank all the people who participated in the present study, including the members of the Research Unit in Cognitive Neuroscience and of the Laboratoire de Psychologie Expérimentale of the Université Libre de Bruxelles, who were prepared to spend several hours filling in questionnaires. This work was supported by A.F.R.F.C.-F.N.R.S. grant. Downloaded by [Archives & Bibliothèques de l'ULB] at 06:20 10 July 2015The observation of specific semantic memory disorders, i.e., that some patients exhibit a selective loss of knowledge of items from certain categories, for example biological (or living) entities, by comparison with nonbiological (or non-living) objects, has generated a large debate about the internal organisation of the semantic system (see recent reviews, e.g., in Caramazza, 1998;Forde & Humphreys, 1999;Humphreys & Forde, 2001;...

Introduction: This paper presents the validation of the French version of the Addenbrooke’s Cognitive Examination Revised (ACE-R). Methods: The variability of the 3 versions of the ACE-R (A, B and C), performed by the same observer, hence mainly 2 or 3 times on 119 patients showing no progression, was first calculated by Cronbach’s alpha coefficient, t test and linear regression. The alpha coefficients of the 3 versions were obtained showing that the ACE-R versions can be considered as one, and an analysis of the interobserver variability was performed by Cohen’s kappa coefficient, t test and linear regression on 12 patients. Eventually, we performed a receiver operating characteristic (ROC) analysis to compare the sensitivities and specificities to detect dementia of the ACE, the ACE-R and Mini Mental State Examination on 319 consecutive patients. Results: The ROC areas of sensitivities and specificities of the ACE and ACE-R were very similar. Two cutoffs were identified at 83/100 and 89/100 with a specificity to normality of 98.6% if the ACE-R score was ≥83 and a sensitivity to dementia of 98.4% if the ACE-R score was ≤89. Conclusion: ACE-R in French is as reliable and valid as the original version to detect dementia.