[Better assessment, better care: Quetci, a questionnaire for assessing cognitive disorders in nursing practice]. / Mieux évaluer, mieux soigner : le Quetci, un questionnaire d'évaluation des troubles cognitifs IDE.

Cognitive disorders can have significant repercussions on the quality of care and daily life for patients. We have developed a new tool specifically designed for nursing practice to identify these problems in patients with brain tumors. The Cognitive Impairment Assessment Questionnaire for nursing practice is an objective, quick and easy-to-administer tool that is readily accepted by patients.

Using Cognitive Reserve to Create Norms for the TMA-93 (Relational Binding of Images).

BACKGROUND: TMA-93 examines relational binding using images. Biomarker validation has demonstrated that it is discriminative for diagnosing early AD. The effect of cognitive reserve on TMA-93 performance remains unexplored and could improve the interpretative framework for using the test. OBJECTIVE: To study the effect of cognitive reserve on TMA-93 performance and to provide new norms for the test that include its measurement. METHODS: Cognitively unimpaired people aged 55 and over were systematically recruited for this cross-sectional normative study in southern Spain. Age, sex, and scores on the Cognitive Reserve Questionnaire (CRQ; maximum score: 25 points) were collected, and the TMA-93 was administered (maximum score: 30 points). Percentile-based reference data that captured combinations of socio-demographics variables with significant effect on TMA-93 performance were calculated. RESULTS: 902 participants (62.5% female; age: median = 68, IQR = 61-75, range = 55-90) were included. CRQ total scores were globally low (median = 8, IQR = 5-13, range = 0-24). Cognitive reserve, including modifiable items as reading activity and intellectual gaming activity, and age mainly supported the TMA-93 total score variance. Sex seemed to have some influence in the elderly. TMA-93 total scores medians began to drop from 70-75 years old. Higher total score on the CRQ and, possibly, female sex determined a gentler slope. New norms based on these variables showed wide variations in scores for the 5th and 10th percentiles. CONCLUSION: Visual relational binding ability depends on cognitive reserve, including modifiable items. The age-related binding deficit is buffered by higher cognitive reserve and, at older ages, by female sex.

Long-term Neurologic Safety in Patients With B-Cell Lymphoma Treated With Anti-CD19 Chimeric Antigen Receptor T-Cell Therapy.

OBJECTIVES: Anti-CD19 chimeric antigen receptor (CAR) T-cell therapy is a promising treatment in relapsing B-cell lymphoma but is frequently associated with acute neurotoxicity. Neurologic long-term safety has not been thoroughly assessed. METHODS: All patients with consecutive refractory lymphoma admitted in our center for CAR T-cell therapy underwent neurologic examination, extensive neuropsychological assessment, and brain MRI (except 1 patient) and completed self-administrated questionnaires at baseline. The patients who remained disease-free at 2 years were re-evaluated similarly. All neurologic assessments were conducted by senior neurologists. RESULTS: None of the 19 disease-free patients developed new neurologic deficits or MRI changes when compared with baseline. There was no difference in cognitive performances before and 2 years after, even for the 11 patients who had developed acute neurotoxicity after CAR T cells. In self-questionnaire assessments, cognitive complaint was stable, reported by 32% of the patients at 2 years. We observed a reduction in HADS anxiety scores 2 years after treatment when compared with baseline (median score: 7/21 vs 4/21, p = 0.01). DISCUSSION: In conclusion, no significant neurocognitive or neurologic disorders were observed in this cohort of patients, 2 years after treatment with anti-CD19 CAR T cells.

Testing Visual Binding by the TMA-93 in People Aged 75 and Over.

BACKGROUND: TMA-93 examines relational binding using images. The test has been proven to be discriminative for diagnosing early Alzheimer's disease by biomarkers. Norms for this test are available, but the elderly, at high risk for Alzheimer's disease, have not yet been widely represented. OBJECTIVE: To extend normative data on the TMA-93 for people aged 75 and over. METHODS: An extension of the Spanish TMA-93 normative study was undertaken. Only cognitively unimpaired people aged 75 and over were included. Age, gender, and educational attainment were registered as socio-demographic variables. Using histograms analysis, median comparisons, and linear regression analysis, we selected variables that demonstrated influence on TMA-93 total scores and provided percentile-base reference data according to combinations of those variables. RESULTS: We included 431 new participants, resulting in a total sample of 657 individuals (median age = 78, interquartile range = 76-81, range = 75-93). Percentile-base reference data stratified by a combination of age ranges (75-79, n = 428; and ≥80 years, n = 229), and educational attainment (< first grade, n = 253; first grade, n = 209; > first grade, n = 195) revealed that participants achieved a minimum TMA-93 total score of 26/30 at the 50th-percentile regardless of stratum. At the 10th-percentile, a maximum of 24/30 was achieved in the more educated stratum contrasting with a minimum of 19/30 in the less educated stratum. CONCLUSION: Although mitigated by lower levels of education, performance on the TMA-93 is widely preserved in cognitively unimpaired people aged 75 and over. The test could facilitate the screening of elderly patients with memory complaints.

Comparison of Neuropsychological Assessment by Videoconference and Face to Face.

OBJECTIVE: To compare the administration of neuropsychological tests by teleneuropsychology (TeleNP) and face to face (F-F) in order to determine the feasibility and reliability of TeleNP. METHOD: At the inclusion visit, all participants underwent a traditional F-F neuropsychological assessment as part of their standard care. Four months after inclusion, they were randomized to undergo an additional neuropsychological assessment either by F-F administration or by TeleNP. RESULTS: A total of 150 adults with cognitive complaints, but with no major cognitive or sensorial impairment were included. At 4 months, 69 participants were randomized in the F-F arm and 71 in TeleNP arm (10 lost in the follow-up). The overall satisfaction was high: 87.1% in the TeleNP arm were "very satisfied", and 82.9% indicated no preference between F-F and TeleNP. In agreement with previous data from the literature, neuropsychological assessments gave similar results across both administration conditions for a large majority of tests [Mini-Mental State Examination (MMSE), Free and Cued Selective Reminding Test (FCSRT) French version, Mahieux gestural praxis battery, Frontal Assessment Battery (FAB), time of completion of the Trail making Test (TMT) A and B, number of errors of the TMT B, Rey complex figure test, categorical et phonological verbal fluency tests] and minor differences for others [80-picture naming test (DO-80), FAB, Digit Span forward and backward and number of errors in the TMT A]. CONCLUSIONS: TeleNP is a promising method to be able to test patients as an alternative to F-F condition. Before this procedure can be generalized, it is now necessary to standardize the adaptation of certain tests and to test them in populations with more significant cognitive disorders.

The Diagnostic Value of a Short Memory Test: The TNI-93.

BACKGROUND: The TNI-93 is a quick memory test designed for all patients regardless of their education level. A significant proportion of patients with Alzheimer's disease (AD) are illiterate or poorly educated, and only a few memory tests are adapted for these patients. OBJECTIVE: In this study we aimed at assessing the diagnostic value of the TNI-93 for diagnosis of patients with biologically confirmed amyloid status. METHODS: We included all patients who had an analysis of AD cerebrospinal fluid biomarkers, a neuropsychological assessment including a TNI-93 and an anatomical brain imaging at Avicenne Hospital between January 2009 and November 2019. We compared the TNI-93 scores in patients with amyloid abnormalities (A+) and patients without amyloid abnormalities (A-) according to the AT(N) diagnostic criteria. RESULTS: 108 patients were included (mean age: 66.9±8.5 years old, mean education level: 8.9±5.2 years). Patients from the A + group (N= 80) were significantly more impaired than patients from the A- group (N= 28) on immediate recall (A+: 5.9±2.8; A-: 7.4±2.6; p = 0.001), free recall (A+: 3.5±2.7; A-: 5.9±2.8; p ≤ 0.001), total recall (A+: 5.7±3.5; A-:7.8±2.8; p ≤ 0.001), and on number of intrusions during the recall phase (A+: 1±1.8; A-: 0.1±0.3; p = 0.002). ROC curves revealed that the best scores to discriminate A + from A- patients were immediate recall (Area under curve (AUC): 0.70), number of encoding trials (AUC: 0.73), free recall (AUC: 0.74), and total recall (AUC: 0.74). CONCLUSION: The TNI-93's immediate, free, and total recalls are valuable tools for the 39 diagnosis of AD.

TMA-93 Validation by Alzheimer's Disease Biomarkers: A Comparison with the Free and Cued Selective Reminding Test on a Biobank Sample.

BACKGROUND: The Memory Associative Test TMA-93 examines visual relational binding, characteristically affected in early-AD stages. OBJECTIVE: We aim to validate the TMA-93 by biomarkers determination and compare its diagnostic characteristics with the Free and Cued Selective Reminding Test (FCSRT). METHODS: Retrospective analysis of a Biobank database. Patients' records initially consulted for memory complaints, scored MMSE≥22, had TMA-93 and FCSRT tested, and AD biomarker determination (Amyloid-PET or CSF), either positive or negative, were selected. As cutoffs, we considered the 10-percentile for TMA-93 (P10/TMA-93), and "total free recall" (TFR) 21/22, total recall (TR) 43/44, and Cued Index < 0.77 for FCSRT from previous Spanish validation and normative studies. Diagnostic utilities were calculated using ROC curves and compared by the DeLong method. We studied if one test improved the other test's prediction, following a forward stepwise logistic regression model. RESULTS: We selected 105 records: 64 "positive" and 41 "negative" biomarkers. TMA-93 total score diagnostic utility (AUC = 0.72; 95%CI:0.62-0.82) was higher than those of the FCSRT: TFR (AUC = 0.70; 95%CI: 0.60-0.80), TR (AUC = 0.63; 95%CI:0.53-0.74), and Cued Index (AUC = 0.62; 95%CI:0.52-0.73). The P10/TMA-93 cutoff showed 86%sensitivity, similar to that of the most sensitive FCSRT cutoff (TFR21/22, 89%) and 29%specificity, lower than that of the most specific FCSRT cutoff (Cued Index < 0.77, 57%). 32.8%of the positive-biomarker group scored above CI/0.77 but below p10TMA-93. The addition of TMA-93 total score to FCSRT variables improved significantly the biomarkers results' prediction. CONCLUSION: TMA-93 demonstrated "reasonable" diagnostic utility, similar to FCSRT, for discriminating AD biomarker groups. TMA-93 total score improved the AD biomarker result prediction when added to FCSRT variables.

Evaluation of mid-term (6-12 months) neurotoxicity in B-cell lymphoma patients treated with CAR T cells: a prospective cohort study.

BACKGROUND: Chimeric antigen receptor-modified T (CAR T) cells are profoundly changing the standard of care in B-cell malignancies. This new therapeutic class induces a significant number of acute neurotoxicity, but data regarding mid- and long-term neurological safety are scarce. We evaluated mid-term neurological safety, with special emphasis on cognitive functions, in a series of adults treated with CAR T cells. METHODS: Patients treated in a single center with CD19-targeted CAR T cells for a relapsing B-cell lymphoma were prospectively followed up by neurologists. Before CAR T-cell infusion, all patients underwent neurological examinations with neuropsychological testing and filled out questionnaires assessing anxiety, depression, and cognitive complaints. Patients surviving without tumor progression were re-evaluated similarly, 6-12 months later. RESULTS: In this prospective cohort of 56 consecutive adult patients treated with CAR T cells, 27 were eligible for mid-term evaluation (median time 7.6 months). Twelve patients developed an acute and reversible neurotoxicity with median duration time of 5.5 days. In all patients, neurological examination on mid-term evaluation was similar to baseline. In self-assessment questionnaires, 63% of patients reported clinically meaningful anxiety, depression, or cognitive difficulties at baseline, a number reduced to 44% at the time of mid-term evaluation. On cognitive assessments, no significant deterioration was found when compared to baseline, in any cognitive functions assessed (verbal and visual memory, executive functions, language, and praxis), even in patients who developed acute neurotoxicity. CONCLUSION: In this cohort of patients treated with CD19-targeted CAR T cells, we found no evidence for neurological or cognitive toxicity, 6-12 months after treatment.

Reliability and Feasibility of the Memory Associative Test TMA-93.

BACKGROUND: Memory tests focused on binding may be more sensitive to diagnose Alzheimer's disease (AD) at an early phase. TMA-93 examines relational binding by images. OBJECTIVE: Evaluate the reliability (internal consistency and inter-rater and test-retest reliability) and feasibility of the TMA-93 in a clinic setting with low-educated individuals and limited face-to-face time per patient. METHODS: The study was undertaken in a neurology outpatient clinic of a hospital in Southern Spain. The internal consistency of the TMA-93 was estimated in 35 patients with amnestic mild cognitive impairment (aMCI) and 40 healthy controls (HCs). The inter-rater reliability (by two raters) and feasibility (by recording the percentage of participants who completed the test, and by timing the administration time) were evaluated in HCs (n = 16), aMCI patients (n = 18), and mild dementia patients (n = 15). The test-retest reliability for the TMA-93 total score was studied in 51 HCs tested by the same examiner 2-4 months apart. The internal consistency was estimated by Cronbach's alpha. The inter-rater and test-retest reliability was quantified by the intraclass correlation coefficient (ICC). The administration time was compared by diagnosis. RESULTS: The internal consistency was "optimal" (Cronbach's alpha = 0.936). The test-retest reliability was "good" [ICC = 0.802 (CI 95%  = 0.653-0.887)]. The inter-rater reliability was "optimal" [ICC = 0.999, (CI 95%  = 0.999-1)]. All participants completed the test. The administration time ranged from less than 3 min in HCs to 6 min in aMCI patients, and 7 min in mild dementia patients. CONCLUSION: Good feasibility and reliability support using the TMA-93 for examining visual relational binding, particularly in the context of low-educational attainment and limited time per patient.

Norms for Testing Visual Binding Using the Memory Associative Test (TMA-93) in Older Educationally-Diverse Adults.

BACKGROUND: TMA-93 examines binding by images, a potential advantage for less-educated individuals. OBJECTIVE: To obtain norms from older Spanish adults for TMA-93. METHODS: A cross-sectional normative study was undertaken in a general neurology outpatient clinic of a university hospital in the Southern Spanish region of Andalusia. Partners of patients who attended the clinic were systematically recruited when eligible: aged 50 and over, no memory complaints, and a total score equal or above percentile 10 on Phototest. Age, gender, and educational attainment were considered as sociodemographic variables. TMA-93 was administered and the total score was registered. RESULTS: The final sample contained 1,131 participants (mean age = 65.7, SD = 9.2), including 305 individuals (27%) who did not completed primary studies. The total score on TMA-93 showed a non-normal, left asymmetric, and leptokurtic distribution (median = 29, interquartile range = 27-30, range = 16-30) mitigated by lower education and older age. Stratified analysis by age and education showed wide variations of the scores for the 5-percentile. CONCLUSION: TMA-93 runs with a ceiling effect in non-cognitively impaired older Spanish adults. The score for the 5-percentile depends on age and education. The test is feasible for low-educated individuals.

How to assess and manage cognitive impairment induced by treatments of non-central nervous system cancer.

A number of neurotoxicity associated with oncological treatments has been reported in non-central nervous system cancers. An expert group presents the state of the art and a guide to help the choice of appropriated tools to assess patient cognition in studies on oncology and neurobehavior in animal models. In addition, current cognitive rehabilitation programs currently under evaluation are also discussed. Cognitive assessments in oncology depend on the research question, study design, cognitive domains, patients' characteristics, psychometric properties of the tests, and whether the tests are supervised or not by a neuropsychologist. Batteries of electronic tests can be proposed, but several of them are characterized by weak psychometric developments. In order to improve the comprehension on the impact of cancer treatments on cognition, new animal models are in development, and would in the future include non-human primate models. By bringing together the skills and practices of oncologists, neurologists, neuropsychologists, neuroscientists, we propose a series of specific tools and tests that accompany the cognitive management of non-CNS cancer patients.

Music as a mnemonic strategy to mitigate verbal episodic memory in Alzheimer's disease: Does musical valence matter?

Introduction: Music is increasingly used to improve cognition in clinical settings. However, it remains unclear whether its use as a mnemonic strategy is effective in Alzheimer's disease (AD). The present study aimed at determining whether a musical mnemonic might mitigate patients' learning of new verbal information and at exploring the effect of factors such as retention delay and emotional valence of the musical excerpt used. Method: 13 patients with AD and 26 healthy comparisons (HC) with a low musical expertise were included. They learned texts about everyday life themes that were either set to familiar instrumental music, which was positively- or negatively-valenced, or spoken only. Immediate and delayed recalls (after 10 min and 24 hours) were measured. Results: Main results showed that (i) HC demonstrated better verbal episodic memory performance than participants with AD; (ii) participants with AD encoded texts paired with positively-valenced music better than texts paired with negatively-valenced music; (iii) participants with AD recalled sung texts better than spoken texts (after 10 min and 24 hours), regardless of musical valence while HC displayed better recall for texts paired with positively-valenced music. Conclusions: Musical mnemonics may help people with AD learn verbal information that relates to their daily life, regardless the musical expertise of the patients. This result gives promising clinical insights showing that music processing is robust to brain damage in AD. Possible hypotheses explaining the effectiveness of musical mnemonics in AD regardless the musical valence are discussed (e.g., different processing between musical and spoken conditions; disappearance of the positivity bias and implications with respect to the underlying socio-emotional selectivity theory).

TMA-93 for Diagnosing Amnestic Mild Cognitive Impairment: A Comparison With the Free and Cued Selective Reminding Test.

BACKGROUND: TMA-93 examines binding by images, an advantage for the less educated individuals. AIM: To compare the discriminative validity of TMA-93 against the picture version of Free and Cued Selective Reminding Test (FCSRT) to distinguish patients with amnestic mild cognitive impairment (aMCI) from normal controls (NCs) without excluding less educated individuals. DESIGN: Phase I diagnostic evaluation study. PARTICIPANTS: A total of 30 patients with aMCI and 30 NCs matched for sociodemographics variables. STATISTICAL ANALYSIS: The diagnostic accuracy for each test was calculated by conducting receiver operating characteristic curve analysis. Hanley and McNeil method was used to compare diagnostic accuracy of different tests on the same sample. RESULTS: Up to 41.7% of the sample had less than a first grade of education. Both tests showed excellent diagnostic accuracy. The comparisons did not show significant differences. CONCLUSIONS: TMA-93 is so accurate as FCSRT to differentiate aMCI from controls including less educated individuals. The test could be considered as a choice in this sociodemographic context.

How to Assess Executive Functions in a Low-Educated and Multicultural Population Using a Switching Verbal Fluency Test (the TFA-93) in Neurodegenerative Diseases?

Few neuropsychological tests are available to assess executive dysfunction in low-educated and multicultural populations. To address this issue, the TFA-93, a switching verbal fluency test to assess cognitive flexibility, was administered to 70 healthy controls, 57 patients with a clinical diagnosis of Alzheimer's disease, and 21 with a clinical diagnosis of a neurodegenerative disease associated with frontal disorders. Most of the participants were low-educated and nonnative French speakers. The TFA-93 comprises 2 categorical fluency tasks (animals and fruits) and a fluency task in which participants have to switch between animals and fruits. Correct responses and errors were collected, and a flexibility index expressed the switching cost. Results showed that correct responses were lower, and the switching cost was greater in both patient groups. In low-educated and multicultural populations, the TFA-93 seems to be a good alternative to assess flexibility compared to the standard neuropsychological tools based on academic abilities.

The TMA-93: A New Memory Test for Alzheimer's Disease in Illiterate and Less Educated People.

The present study aimed at validating the Memory Associative Test of the district of Seine-Saint-Denis (TMA)-93, a new test of episodic memory. The TMA-93 was proposed to mostly less educated and multicultural elderly population composed of 376 healthy controls (HC) and 94 patients with Alzheimer's disease (AD). The construct validity was checked by studying correlations with a widely used memory test (the Free and Cued Selective Reminding Test [FCSRT]) in the subsample of literate patients. Results showed that (i) all the TMA-93 scores of the patients with AD were lower than those of the HC, (ii) the TMA-93 total score identified patients with AD with a high sensitivity (88%) and very high specificity (97%), and (iii) the TMA-93 total score was strongly correlated with both free recall and total recall scores of the FCSRT. Taken together, results showed that the TMA-93 is a reliable tool to assess episodic memory in a multicultural, less educated, or illiterate population, with good construct validity for AD diagnostic accuracy.

The sense of self is supported by several independent cognitive processes in Alzheimer's disease and self-reported age tracks cognitive impairment.

Do patients with Alzheimer's disease loose themselves? The impact of dementia on the sense of self has been extensively studied over the past 15 years. However, most studies investigate only one marker of the self - such as mirror self-recognition or the use of the pronoun "I" - and do not track how this marker evolves in the course of the illness in comparison to other markers. This situation has resulted in fragmented findings rather than converging evidence for a coherent model of the self in dementia. In our two studies, we use a questionnaire to investigate four markers of the self simultaneously (self-knowledge, mirror self-recognition, the bodily distinction between self and other, and self-reported age) in the same 60 patients spread across three stages of Alzheimer's disease. This method allows us to determine whether these markers are impaired independently over the progression of the illness. Our results suggest that the sense of self relies on a complex structure supported by several independent cognitive processes that are impacted differently by the progression of dementia. In particular, despite the early deterioration of self-knowledge, patients at advanced stages of the disease seem to maintain a sense of self, rooted into mirror self-recognition and the bodily self. Furthermore, self-reported age predicts the level of cognitive impairment. We suggest that a better understanding of the stage at which each marker of the self breaks down can help clinicians support their patients better by targeting the preserved dimensions of their identity at any given point in the progression of their condition.

TNI-93: A New Memory Test for Dementia Detection in Illiterate and Low-Educated Patients.

OBJECTIVE: To validate the TNI-93 test in illiterate and low-educated subjects by setting cutoff scores to discriminate non-demented and demented subjects in a clinical setting (CESILL) and verifying the adequacy of these cutoff scores in a population-based study (AMI cohort). METHOD: We used two study samples. First, a clinical setting (CESILL) comprising normal elderly participants and demented patients, mostly multicultural, low educated, or illiterate, was used to compute the cutoff scores of TNI-93 for the detection of dementia. Second, the AMI cohort, a population-based cohort of retired farmers living in a rural setting, was used as a replication study, to assess the detection properties of the cutoff scores in a different population composed mostly of low-educated older people. RESULTS: When combining the two scores, that is, free recall <6 or total recall <9, TNI-93 can detect dementia with a high sensitivity (87%) and specificity (96%), in the CESILL setting. These cutoff scores were roughly similar in the AMI cohort with high sensitivity (80% sensitivity) and specificity (81% specificity). In both study samples, the level of education had no effect on performance. CONCLUSIONS: The TNI-93 appears to be a good test to detect dementia. The absence of a significant effect of education level on the performances makes the TNI-93 a tool of choice in the screening of dementia in illiterate/low-educated subjects.

Music enhances verbal episodic memory in Alzheimer's disease.

INTRODUCTION: Although previous studies suggest that music may facilitate verbal learning in a healthy population, such a mnemonic effect has seldom been investigated in Alzheimer's disease (AD). Moreover, memorization of texts was generally compared when either sung or spoken. In the present study, it was examined whether the benefit observed on verbal learning was specific to music or whether an associative context binding items together led to similar benefits, regardless of the nature of the association. METHOD: Twelve patients with mild AD and 15 healthy controls learned texts presented with either a musical (sung) or a nonmusical association (spoken associated to a silent movie sequence) or without association (spoken alone). Immediate and delayed (after a 5-min delay) recall was measured. RESULTS: Main results showed that (a) sung texts were better remembered than spoken texts, both immediately and after a retention delay, for both groups; (b) the musical benefit was robust, being observed in most AD patients; (c) the nonmusical association may also facilitate verbal learning but to a lesser extent. CONCLUSIONS: A musical association during the encoding stage facilitates learning and retention in AD. Furthermore, this advantage seemed quite specific to music. The results are discussed with respect to the clinical applications in AD; theoretical implications are highlighted to explain the power of music as a mnemonic technique.

[Artificial intelligence meeting neuropsychology. Semantic memory in normal and pathological aging]. / L'intelligence artificielle à la rencontre de la neuropsychologie: mémoire sémantique, vieillissement normal et pathologique.

Artificial intelligence (IA) is the subject of much research, but also many fantasies. It aims to reproduce human intelligence in its learning capacity, knowledge storage and computation. In 2014, the Defense Advanced Research Projects Agency (DARPA) started the restoring active memory (RAM) program that attempt to develop implantable technology to bridge gaps in the injured brain and restore normal memory function to people with memory loss caused by injury or disease. In another IA's field, computational ontologies (a formal and shared conceptualization) try to model knowledge in order to represent a structured and unambiguous meaning of the concepts of a target domain. The aim of these structures is to ensure a consensual understanding of their meaning and a univariant use (the same concept is used by all to categorize the same individuals). The first representations of knowledge in the AI's domain are largely based on model tests of semantic memory. This one, as a component of long-term memory is the memory of words, ideas, concepts. It is the only declarative memory system that resists so remarkably to the effects of age. In contrast, non-specific cognitive changes may decrease the performance of elderly in various events and instead report difficulties of access to semantic representations that affect the semantics stock itself. Some dementias, like semantic dementia and Alzheimer's disease, are linked to alteration of semantic memory. We propose in this paper, using the computational ontologies model, a formal and relatively thin modeling, in the service of neuropsychology: 1) for the practitioner with decision support systems, 2) for the patient as cognitive prosthesis outsourced, and 3) for the researcher to study semantic memory.

Posterior cortical atrophy as an extreme phenotype of GRN mutations.

IMPORTANCE: Posterior cortical atrophy (PCA) is characterized by progressive visuoperceptual and visuospatial deficits and commonly considered to be an atypical variant of Alzheimer disease. Mutations of the GRN gene are responsible for a large phenotypic spectrum, but, to our knowledge, the association of PCA with GRN mutations has never been described. OBSERVATIONS: We studied a patient presenting with insidious impairment of basic visuoperceptual skills and apperceptive visual agnosia with predominant posterior atrophy corresponding to a visual/ventral variant of PCA. A heterozygous p.Arg110* (c.328C>T) GRN mutation was identified in this patient. CONCLUSIONS AND RELEVANCE: This study extends the clinical spectrum of GRN mutations that may be responsible for a PCA phenotype. The GRN phenotypes overlap other degenerative dementias and highlight the limits of actual nosologic boundaries in dementias. The GRN gene should be analyzed in patients with PCA, particularly when the damage progresses to anterior cerebral regions and a family history of dementia is present.