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International audience; Background: High consumption of Annona muricata fruit has been previously identified as a risk factor for atypical parkinsonism in the French Caribbean islands.Objective: We tested whether consumption of Annonaceae products could worsen the clinical phenotype of patients with any form of degenerative parkinsonism.Methods: We analyzed neurological data from 180 Caribbean parkinsonian patients and specifically looked for dose effects of lifelong, cumulative Annonaceae consumption on cognitive performance. Using unsupervised clustering, we identified one cluster with mild/moderate symptoms (N = 102) and one with severe symptoms including cognitive impairment (N = 78).Results: We showed that even low cumulative consumption of fruits/juices (>0.2 fruit-years) or any consumption of herbal tea from Annonaceae worsen disease severity and cognitive deficits in degenerative parkinsonism including Parkinson's disease (OR fruits-juices: 3.76 [95% CI: 1.13-15.18]; OR herbal tea: 2.91 [95% CI: 1.34-6.56]).Conclusion: We suggest that more restrictive public health preventive recommendations should be made regarding the consumption of Annonaceae products. © 2022 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.

Understated executive dysfunction (UED) is predictive of cognitive decline and death. We aimed to assess the prevalence of UED, assessed with the clock-drawing test (CDT) and the Frontal Assessment Battery (FAB) in middle-aged adults and to investigate associated characteristics.Cross-sectional analysis of data on 516 community-dwellers aged 50-65, lacking cognitive complaints, who were included prospectively (2010-2017) after a multidimensional geriatric assessment at a "healthy ageing" outpatient clinic. Age- and educational-level-adjusted logistic models were used to assess factors associated with UED.The CDT and FAB were impaired in 27.7% and 14.7% of the participants (median age: 59.7 years). The prevalence [95% confidence interval (CI)] of UED was 36.2% [32.2-40.5%]. After adjustment for age and education, participants with UED were more likely to be obese (odds ratio [95%CI] = 1.89 [1.12-3.19],More than one third of middle-aged adults without cognitive complaints have UED, which was linked to obesity and metabolic syndrome. Cognitive screening tests targeting executive functions might be useful for early detection of UED and the initiation of multidomain interventions improving cognitive performance.

Mild encephalopathy/encephalitis with reversible splenial lesion (MERS) is a transient clinico-radiological syndrome characterized by non-specific encephalopathy and specific magnetic resonance imaging (MRI) pattern. MRI shows an ovoid lesion in the mid-splenium of the corpus callosum (SCC), with signal-intensity anomaly similar to stroke but vanishing within few weeks. Although there are a lot of child MERS cases descriptions, there are just a few adult-onset reported. Our goal is to provide a better clinical and radiological description of this entity. We reported nine adult-onset cases of MERS managed in our stroke unit between 2017 and 2019. The study of our adult series suggests that epilepsy and the context of an infection are very common in MERS. Adult cases show frequent focal neurological deficits and few encephalopathies compared to children. The measurement of very low ADC values in SCC lesion is a new radiological feature of MERS that should be systematically assessed in suspected cases to differentiate this complex syndrome from SCC strokes.

Objectives Using brief samples of speech recordings, we aimed at predicting, through machine learning, the clinical performance in Huntington’s Disease (HD), an inherited Neurodegenerative disease (NDD). Methods We collected and analyzed 126 samples of audio recordings of both forward and backward counting from 103 Huntington’s disease gene carriers [87 manifest and 16 premanifest; mean age 50.6 (SD 11.2), range (27–88) years] from three multicenter prospective studies in France and Belgium (MIG-HD (ClinicalTrials.gov NCT00190450); BIO-HD (ClinicalTrials.gov NCT00190450) and Repair-HD (ClinicalTrials.gov NCT00190450). We pre-registered all of our methods before running any analyses, in order to avoid inflated results. We automatically extracted 60 speech features from blindly annotated samples. We used machine learning models to combine multiple speech features in order to make predictions at individual levels of the clinical markers. We trained machine learning models on 86% of the samples, the remaining 14% constituted the independent test set. We combined speech features with demographics variables (age, sex, CAG repeats, and burden score) to predict cognitive, motor, and functional scores of the Unified Huntington’s disease rating scale. We provided correlation between speech variables and striatal volumes. Results Speech features combined with demographics allowed the prediction of the individual cognitive, motor, and functional scores with a relative error from 12.7 to 20.0% which is better than predictions using demographics and genetic information. Both mean and standard deviation of pause durations during backward recitation and clinical scores correlated with striatal atrophy (Spearman 0.6 and 0.5–0.6, respectively). Interpretation Brief and examiner-free speech recording and analysis may become in the future an efficient method for remote evaluation of the individual condition in HD and likely in other NDD.

Because of the genetic transmission of Huntington's disease (HD), informal caregivers (ICs, i.e., non-professional caregivers) might experience consecutive and/or concurrent caregiving roles to support several symptomatic relatives with HD over their life. Additionally, some ICs might be HD carriers. However, whether family burden of care is associated with specific factors in HD remains poorly studied.To provide a quantitative view of the IC burden and identify associated factors.This was a cross-sectional assessment of home-dwelling symptomatic HD individuals (from REGISTRY and Bio-HD studies) and their primary adult ICs, including the HD individual's motor, cognitive, behavioral, functional Unified Huntington's Disease Rating Scale score; IC objective burden (quantification of IC time in activities of daily living, instrumental activities of daily living and supervision, using the Resource Utilization in Dementia instrument), IC subjective burden (Zarit Burden Inventory), and ICs' social economic functioning and use of professional home care.We included 80 ICs (mean [SD] age 57 [12.9] years, 60% women) in charge of 80 individuals with early to advanced stage HD (mean age 56 [12.6] years, 51% men). The mean hours of informal care time was high: 7.3 (7.9) h/day (range 0-24); the mean professional home care was 2.8 (2.8) h/day (range 0.1-12.3). This objective burden increased with higher functional loss of the HD individual and with more severe cognitive-behavioral disorders. The mean subjective burden (35.4 [17.8], range 4-73) showed a high level since the earliest stage of HD; it was associated with HD duration (mean 9.2 [4.7] years) and with aggressive symptoms in individuals (44% of cases). The burden was partially related to the multiplex caregiving status (19%). Protective factors lowering the IC burden included the absence of financial hardship (57%), a strong social network (16%) and keeping active on the job market outside home (46%).The objective-subjective burden of ICs related to changing patterns of neuro-psychiatric symptoms and mitigating environmental characteristics around the HD individual-caregiver dyads.

Using brief samples of speech recordings, we aimed at predicting, through machine learning, the clinical performance in Huntington's Disease (HD), an inherited Neurodegenerative disease (NDD).We collected and analyzed 126 samples of audio recordings of both forward and backward counting from 103 Huntington's disease gene carriers [87 manifest and 16 premanifest; mean age 50.6 (SD 11.2), range (27-88) years] from three multicenter prospective studies in France and Belgium (MIG-HD (ClinicalTrials.gov NCT00190450); BIO-HD (ClinicalTrials.gov NCT00190450) and Repair-HD (ClinicalTrials.gov NCT00190450). We pre-registered all of our methods before running any analyses, in order to avoid inflated results. We automatically extracted 60 speech features from blindly annotated samples. We used machine learning models to combine multiple speech features in order to make predictions at individual levels of the clinical markers. We trained machine learning models on 86% of the samples, the remaining 14% constituted the independent test set. We combined speech features with demographics variables (age, sex, CAG repeats, and burden score) to predict cognitive, motor, and functional scores of the Unified Huntington's disease rating scale. We provided correlation between speech variables and striatal volumes.Speech features combined with demographics allowed the prediction of the individual cognitive, motor, and functional scores with a relative error from 12.7 to 20.0% which is better than predictions using demographics and genetic information. Both mean and standard deviation of pause durations during backward recitation and clinical scores correlated with striatal atrophy (Spearman 0.6 and 0.5-0.6, respectively).Brief and examiner-free speech recording and analysis may become in the future an efficient method for remote evaluation of the individual condition in HD and likely in other NDD.

Objectives To estimate the prevalence of understated cognitive impairment by administering the Clock-Drawing Test (CDT) to community-dwelling individuals aged ≥50 years and to investigate the associated clinical phenotype. Design A cross-sectional analysis of baseline data on community-dwelling individuals assessed at an outpatient clinic in the Paris region of France. Setting and Participants Participants aged ≥50 years (n = 488, median age: 62.1 years) prospectively included in the SUCCessful agEing outpatiEnt's Department survey between 2010 and 2014. Methods A multidimensional geriatric assessment, including cognition [7-point CDT, Mini-Mental State Examination (MMSE), the 5-word screening test (5-WT), and the Frontal Assessment Battery (FAB)], gait speed in dual tasks, mood [the Geriatric Depression Scale (GDS)], balance, physical functions (gait speed and handgrip strength), nutrition, bone density, and comorbidities; major cardiovascular risk factors, and Scheltens and Fazekas scores on brain magnetic resonance imaging. Baseline characteristics were analyzed as a function of the CDT score ( Results The prevalence of impairment in the CDT was 23.6%; higher than the values for the MMSE (12.7%), 5-WT (2.3%), and FAB (16.6%). In age-adjusted analyses, a lower educational level (odds ratio [95% confidence interval] = 0.72 [0.58‒0.89]), diabetes (2.57 [1.14‒5.79]), metabolic syndrome (1.93 [1.05‒3.56]), lower gait speed in the cognitive dual task (1.27 [1.05‒1.53]), a poorer Geriatric Depression Scale score (1.86 [1.04‒3.32]), a poorer MMSE score (2.56 [1.35‒4.88]), a poorer FAB score (1.79 [1.01‒3.16]), impaired episodic memory in the 5-WT (4.11 [1.12‒15.02]), and a higher Scheltens score (P = .001) were significantly associated with CDT impairment. Conclusions and Implications Understated cognitive impairment is common among young seniors and is associated with factors known to be linked to a higher risk of cognitive decline and dementia. These findings suggest that the CDT may be of value for identifying high-risk individuals who may then benefit from targeted multidomain prevention actions (diet, exercise, cognitive training, and vascular risk factor management).

Background Cannabis use is increasing worldwide despite the various health effects of this substance. Methods We report 2 cases of acute hippocampal encephalopathy in heavy cannabis users (>10 joints/d). Results In both male patients, acute encephalitis was suspected. Brain magnetic resonance imaging (MRI) diffusion-weighted sequences showed bilateral high signal abnormalities in hippocampal regions. Patients had renal dysfunction, rhabdomyolysis, and inflammatory syndrome. Investigations showed no evidence of infectious or autoimmune encephalitides. Repeated electroencephalograms revealed no epileptic activity. Clinical, biological, and magnetic resonance imaging acute abnormalities improved within weeks. New exposure to cannabis yielded a new episode of encephalopathy. In both patients, severe long-lasting episodic memory impairment associated with hippocampal atrophy were observed several months later. Conclusions Health professionals should be aware of this cannabis-related syndrome given its severe and long-lasting effects.

The symptoms of COVID-19 include fever, fatigue and respiratory illness ranging from cough to acute respiratory distress syndrome (ARDS).1 Neurological complications, such encephalopathy, are more and more reported. They can result from direct viral invasion, hypoxic, toxic or immune-mediated injury. In this report, we describe a patient with posterior reversible encephalopathy syndrome (PRES) associated with SARS-CoV-2 infection. The patient is a 69-year-old woman, with history of coronary artery disease, an endometrial cancer, in remission since 2006 and a right breast cancer without anticancer treatment and in remission since 2018, and no history of hypertension. She presented to the stroke unit, in the end of March 2020, few days before the epidemic peak in France, for three generalised seizures, mutism and delirium, and 7 days’ history of unusual asthenia. Temperature was 37°C, blood pressure at 200/116 mm Hg and oxygen saturation was 95% while she was breathing ambient air. The pulmonary examination was normal. Neurological examination found no focal motor or sensor deficit. Brain MRI, performed 5 hours after the onset of seizures, showed bilateral and symmetric T2 and fluid-attenuated inversion recovery imaging hyperintensities located mainly in the temporal and occipital lobes with increased apparent diffusion coefficient values and leptomeningeal enhancement in the same areas in 3D T1 gadolinium sequence (figure 1). There were no signs of intracranial haemorrhage in susceptibility weighted imaging (images not shown). The vessel’s imaging was normal. The electroencephalogram showed intermittent focal slow delta waves in posterior …

Though accumulating evidence indicates that the striatum is recruited during language processing, the specific function of this subcortical structure in language remains to be elucidated. To answer this question, we used Huntington's disease as a model of striatal lesion. We investigated the morphological deficit of 30 early Huntington's disease patients with a novel linguistic task that can be modeled within an explicit theory of linguistic computation. Behavioral results reflected an impairment in HD patients on the linguistic task. Computational model-based analysis compared the behavioral data to simulated data from two distinct lesion models, a selection deficit model and a grammatical deficit model. This analysis revealed that the impairment derives from an increased randomness in the process of selecting between grammatical alternatives, rather than from a disruption of grammatical knowledge per se. Voxel-based morphometry permitted to correlate this impairment to dorsal striatal degeneration. We thus show that the striatum holds a role in the selection of linguistic alternatives, just as in the selection of motor and cognitive programs.

Background Aging individuals with Down syndrome (DS) are at increased risk of dementia due to trisomy of chromosome 21 on which the amyloid precursor protein gene is located and with increased life expectancy. Yet, little is known about the costs associated with DS dementia and how this compares to Alzheimer's disease (AD). Objective To better understand direct healthcare costs and care consumption in DS dementia, we compared the total cost of care to US Medicare and the drivers of these medical expenditures in individuals with DS with and without dementia, and in those with AD without DS. Methods The effect of dementia in DS on costs and care utilization was estimated with 2015 California Medicare fee-for-service data (parts A and B). Among 3,001,977 Californian Medicare beneficiaries, 353 individuals had DS with dementia (age 45–89 years). We compared their number of chronic comorbidity conditions among 27 and their care and Medicare costs to those of age- and sex-matched individuals with DS without dementia and those with AD without DS. Results Medicare annual cost per beneficiary was a mean of 43.5% and 82.2% higher with DS dementia (mean $35,011) than DS without dementia (mean $24,401) and AD without dementia (mean $19,212), related to greater utilization of inpatient services. DS dementia was associated with increased level of multimorbidity (mean of 3.4 conditions in addition to dementia vs. 2.7 and 2.2 conditions for DS without dementia and AD, respectively), with more emergency room visits (88% vs. 76.5% and 54.4%) and with more primary care physician visits (91.2% vs. 87.3% and 81.3%). Conclusion DS adults with dementia have higher health care costs than DS adults without dementia and adults with AD. Understanding costs and complex health care needs in DS dementia could facilitate management of adult and geriatric care resources for these high-need high-cost individuals.

Objective Psychotic phenomena can occur in non-clinical subjects. The goals of this study were to assess the prevalence of delusions, hallucinations and minor ‘psychotic’ phenomena (visual illusions, feeling of presence and passage hallucinations) and to describe the characteristics of the latter in a non-clinical older population. Methods Three hundred and thirteen individuals aged 60 years and older, without cognitive deficits (according to mini-mental state examination scores) or patent psychotic disease, answered a structured questionnaire focusing on delusions, hallucinations and minor phenomena that they had experienced in the previous month. The study sample was stratified by age and gender according to French demographic characteristics. Results Twenty per cent of participants reported one or more psychotic phenomena. These subjects did not differ from those without psychotic symptoms as regards their age, mini-mental state examination scores or education. Minor phenomena were the most common (13%). Hallucinations, in any sensory modality, occurred in 9% of participants. No verbal auditory hallucinations or delusions were reported. The prevalence of minor phenomena increased with age and was associated with the use of psychoactive drugs. Conclusion By extending the spectrum of psychotic symptoms to minor phenomena, we found that psychotic symptoms were common in a non-clinical older population. Whether the increasing prevalence of minor phenomena with age is due to prodromal neurodegenerative disease or to other factors remains to be investigated. Copyright © 2016 John Wiley & Sons, Ltd.

Understanding the neurobiological basis of complex human behaviors is a key aim for social neuroscience. Examining clinical populations with relatively circumscribed brain damage and related behavioral deficits can provide insights into brain regions which are necessary for abilities such as face processing, emotion recognition, theory of mind, and empathy. In this review, we reflect on the emerging body of evidence which combines experimental behavioral studies and neuroimaging analysis techniques in clinical groups, with a focus on four progressive neurodegenerative disorders: behavioral-variant frontotemporal dementia, semantic dementia, Huntington’s disease, and Alzheimer’s disease. These clinical syndromes are characterized by divergent patterns of neurodegeneration and variable degrees of impairment in social cognition and social behavior. Here, we review the paradigms which have been employed and the current patterns of findings in these syndromes and discuss how this line of research informs our understanding of the “social brain.” In addition, we consider how our conception of these clinical phenotypes has changed, as aspects of social cognition have been incorporated into diagnostic and prognostic frameworks. Finally, we propose potential avenues for future research in these syndromes to address outstanding social neuroscience questions.

Theories of embodied cognition suggest that perceiving an emotion involves somatovisceral and motoric re-experiencing. Here we suggest taking such an embodied stance when looking at emotion processing deficits in patients with Huntington's Disease (HD), a neurodegenerative motor disorder. The literature on these patients' emotion recognition deficit has recently been enriched by some reports of impaired emotion expression. The goal of the study was to find out if expression deficits might be linked to a more motoric level of impairment. We used electromyography (EMG) to compare voluntary emotion expression from words to emotion imitation from static face images, and spontaneous emotion mimicry in 28 HD patients and 24 matched controls. For the latter two imitation conditions, an underlying emotion understanding is not imperative (even though performance might be helped by it). EMG measures were compared to emotion recognition and to the capacity to identify and describe emotions using alexithymia questionnaires. Alexithymia questionnaires tap into the more somato-visceral or interoceptive aspects of emotion perception. Furthermore, we correlated patients' expression and recognition scores to cerebral grey matter volume using voxel-based morphometry (VBM). EMG results replicated impaired voluntary emotion expression in HD. Critically, voluntary imitation and spontaneous mimicry were equally impaired and correlated with impaired recognition. By contrast, alexithymia scores were normal, suggesting that emotion representations on the level of internal experience might be spared. Recognition correlated with brain volume in the caudate as well as in areas previously associated with shared action representations, namely somatosensory, posterior parietal, posterior superior temporal sulcus (pSTS) and subcentral sulcus. Together, these findings indicate that in these patients emotion deficits might be tied to the "motoric level" of emotion expression. Such a double-sided recognition and expression impairment may have important consequences, interrupting empathy in nonverbal communication both ways (understanding and being understood), independently of intact internal experience of emotion.

Introduction L’utilisation recreative du cannabis est parfois responsable de troubles neurovasculaires ou neuropsychiatriques. Nous decrivons deux cas inedits d’encephalopathie toxique hippocampique aigue et persistante, par consommation chronique de cannabis. Observation Deux hommes, âges de 38 et 32 ans, gros consommateurs de cannabis (10 a 15 joints/j) et avec un antecedent d’epilepsie generalisee recente sous monotherapie sont hospitalises en soins intensifs pour encephalopathie febrile avec trouble de la vigilance, rhabdomyolyse et insuffisance renale aigue. Le bilan elimine les etiologies infectieuses, auto-immunes ou paraneoplasiques. Les tests toxicologiques sont exclusivement positifs au cannabis. L’EEG ne retrouve pas d’activite epileptique. L’IRM montre des hypersignaux hippocampiques bilateraux, en diffusion avec restriction de l’ADC, T2 et FLAIR, sans autre anomalie structurelle. Un des patients a recu un traitement probabiliste par immunoglobulines intraveineuses. La fonction renale et les CPK se normalisent en quelques jours et l’IRM en quelques semaines. Pourtant, le bilan neuropsychologique realise apres normalisation de la vigilance montre une atteinte severe, isolee de la memoire episodique, avec trouble du stockage et de la consolidation, persistant apres plus de trois mois de l’episode aigu. Chez le premier patient, la reprise du cannabis apres 6 mois s’est accompagnee d’un nouvel episode d’encephalopathie avec troubles de la fonction renale et rhabdomyolyse. Discussion Le Δ-9-tetrahydrocannabinol et le cannabidiol agissent sur les recepteurs cannabinoides CB1, fortement exprimes dans les hippocampes. Le cannabis naturel ou de synthese est connu pour etre responsable de troubles cognitifs, epileptiques et renaux. Cependant, l’encephalopathie toxique hippocampique non reversible associee a une insuffisance renale aigue et a une rhabdomyolyse chez le gros consommateur de cannabis n’a jamais ete decrite. Conclusion La description de ce nouveau syndrome d’encephalopathie toxique hippocampique liee au cannabis doit alerter sur les dangers de cette drogue a l’heure de la generalisation mondiale de sa consommation.

The Unified Huntington's Disease Rating Scale (UHDRS) adequately measures decline in patients at early and moderate stages of Huntington's disease (HD). In patients with advanced HD, floor effects hamper the evaluation, thus calling for an adjusted scale. We designed the UHDRS-For Advanced Patients (UHDRS-FAP) to improve longitudinal assessment of patients at the advanced disease stage. Sixty-nine patients with a Total Functional Capacity score ≤ 5 were recruited in France and the Netherlands. Among them, 45 patients were followed longitudinally (mean ± standard deviation, 1.6 ± 1.2 years) with the UHDRS-FAP; 30 patients also were assessed with the UHDRS. In cross-sectional analyses, the psychometric properties and inter-rater reliability of the scale were evaluated. Longitudinal analyses were used to evaluate the sensitivity to decline of the UHDRS-FAP compared with the UHDRS. Internal consistency was higher for motor (0.84) and cognitive (0.91) scores than for somatic (0.70) and behavioral (0.49) scores. Inter-rater reliability was ≥ 0.88 for all scores. The somatic score, which was specific to the UHDRS-FAP, declined over time along with motor and cognitive performance on both scales. Although performance with the two scales was correlated, the UHDRS-FAP appeared to be more sensitive to change and was the only scale that detected decline in patients with a Total Functional Capacity score ≤ 1. Neither scale detected a significant decline in behavioral scores. The results indicate that the UHDRS-FAP is reliable and more sensitive to change than the original UHDRS for cognitive and motor domains. It offers items that are relevant for daily care. Behavioral scores tended to decline, but this may reflect the decline in patients' communicative abilities. © 2013 International Parkinson and Movement Disorder Society

Importance As the life expectancy of people with Down syndrome (DS) has markedly increased over the past decades, older adults with DS may be experiencing a higher incidence of aging conditions. In addition to longevity, the amyloid precursor protein gene located on chromosome 21 places individuals with DS at a high risk for developing Alzheimer disease. Yet, few studies have determined prevalence of dementia and comorbidities among older people with DS. Objective To determine the prevalence of dementia and aging-related comorbidities in older adult individuals with DS. Design, Setting, and Participants Cross-sectional analysis of 2015 California Medicare claims data. We examined 1 year of cross-sectional Medicare claims data that included 100% of Californian Medicare beneficiaries enrolled in both Medicare Part A and B in 2015. Of these 3 001 977 Californian Medicare beneficiaries 45 years or older, 878 individuals were identified as having a diagnosis of DS. Data were analyzed between April 2017 and February 2018. Main Outcomes and Measures The frequency of DS dementia was assessed across different age categories. The number and frequency of 27 comorbidities were compared among individuals with DS with and without dementia and by age and sex groups. Results A total of 353 DS individuals (40%) were identified as having dementia diagnoses (mean, 58.7 years; 173 women [49%]) and 525 without dementia diagnoses (mean, 55.9 years; 250 women [48%]). The frequency of DS dementia among those 65 years or older rose to 49%. The mean number of comorbidities per individual increased with age in general. Comorbid conditions were more numerous among those with dementia compared with those with DS without dementia (mean, 3.4 vs 2.5, respectively), especially among those younger than 65 years. In particular, 4 treatable conditions, hypothyroidism, epilepsy, anemia, and weight loss, were much more frequent in DS dementia. Conclusions and Relevance Older Medicare beneficiaries in California with DS, especially those with dementia, have a high level of multimorbidity including several treatable conditions. While DS follow-up has long been confined to the pediatric sphere, we found that longevity in individuals with DS will necessitate complex adult and geriatric care. More evidenced-based and standardized follow-up could support better long-term comorbidity management and dementia care among aging adults with DS.

Patients with Huntington's disease (HD), a neurodegenerative disorder that causes major motor impairments, also show cognitive and emotional deficits. While their deficit in recognising emotions has been explored in depth, little is known about their ability to express emotions and understand their feelings. If these faculties were impaired, patients might not only mis-read emotion expressions in others but their own emotions might be mis-interpreted by others as well, or thirdly, they might have difficulties understanding and describing their feelings. We compared the performance of recognition and expression of facial emotions in 13 HD patients with mild motor impairments but without significant bucco-facial abnormalities, and 13 controls matched for age and education. Emotion recognition was investigated in a forced-choice recognition test (FCR), and emotion expression by filming participants while they mimed the six basic emotional facial expressions (anger, disgust, fear, surprise, sadness and joy) to the experimenter. The films were then segmented into 60 stimuli per participant and four external raters performed a FCR on this material. Further, we tested understanding of feelings in self (alexithymia) and others (empathy) using questionnaires. Both recognition and expression were impaired across different emotions in HD compared to controls and recognition and expression scores were correlated. By contrast, alexithymia and empathy scores were very similar in HD and controls. This might suggest that emotion deficits in HD might be tied to the expression itself. Because similar emotion recognition-expression deficits are also found in Parkinson's Disease and vascular lesions of the striatum, our results further confirm the importance of the striatum for emotion recognition and expression, while access to the meaning of feelings relies on a different brain network, and is spared in HD.

International audience; Pointing is a communicative gesture that allows individuals to share information about surrounding objects with other humans. Patients with heterotopagnosia are specifically impaired in pointing to other humans' body parts but not in pointing to themselves or to objects. Here, we describe a female patient with heterotopagnosia who was more accurate in pointing to men's body parts than to women's body parts. We replicated this gender effect in healthy participants with faster reaction times for pointing to men's body parts than to women's body parts. We discuss the role of gender stereotypes in explaining why it is more difficult to point to women than to men.

A 67-year-old man was referred for fluctuating neuropsychiatric symptoms, featuring depression, delirious episodes, recurrent visual hallucinations and catatonic syndrome associated with cognitive decline. No parkinsonism was found clinically even under neuroleptic treatment. (18)F-FDG PET/CT showed hypometabolism in the posterior associative cortex including the occipital cortex, suggesting Lewy body dementia, but (123)I-FP-CIT SPECT was normal and cardiac (123)I-MIBG imaging showed no signs of sympathetic denervation. Alzheimer's disease was excluded by a normal (18)F-florbetaben PET/CT. This report suggests a rare case of α-synucleinopathy without brainstem involvement, referred to as "cerebral type" of Lewy body disease.

Heterotopagnosia is the acquired inability of brain-lesioned patients to point at someone else's body parts when prompted. The cognitive basis of this disorder is unclear. It might result from a biological function deficit critical for communication in human beings; alternatively, it could result from the disruption of a body representation. Here, we report three patients with heterotopagnosia following a recent left parieto-occipital stroke and a previous insular lesion. The patients were tested on their ability to name, point out and grasp several targets including body parts (own, real others' and figurative others'). Language, visuo-spatial deficits or any confounding neuropsychological disorders were controlled for. We found that the patients erroneously pointed to their own body parts when asked to point at someone else's. Strikingly, their ability to grasp someone else's body parts was largely unimpaired. The dissociation between their grasping and communicative pointing abilities supports the hypothesis that heterotopagnosia is a disorder of communicative function conveyed by pointing but not by grasping. In addition, pointing performance in our patients varied according to the target: the more similar the target was to a real person, the worse the patients' pointing performance. We suggest that communicative pointing might require a specific representation of the addressee's body and point of view, a heterocentric representation. In the patients described here this phenomenon resulted from a combined insulo-parietal lesion, which may explain why, in contrast to other patients described previously, the heterotopagnosia was long-lasting.

Psychotic phenomena can occur in non-clinical subjects. The goals of this study were to assess the prevalence of delusions, hallucinations and minor 'psychotic' phenomena (visual illusions, feeling of presence and passage hallucinations) and to describe the characteristics of the latter in a non-clinical older population.Three hundred and thirteen individuals aged 60 years and older, without cognitive deficits (according to mini-mental state examination scores) or patent psychotic disease, answered a structured questionnaire focusing on delusions, hallucinations and minor phenomena that they had experienced in the previous month. The study sample was stratified by age and gender according to French demographic characteristics.Twenty per cent of participants reported one or more psychotic phenomena. These subjects did not differ from those without psychotic symptoms as regards their age, mini-mental state examination scores or education. Minor phenomena were the most common (13%). Hallucinations, in any sensory modality, occurred in 9% of participants. No verbal auditory hallucinations or delusions were reported. The prevalence of minor phenomena increased with age and was associated with the use of psychoactive drugs.By extending the spectrum of psychotic symptoms to minor phenomena, we found that psychotic symptoms were common in a non-clinical older population. Whether the increasing prevalence of minor phenomena with age is due to prodromal neurodegenerative disease or to other factors remains to be investigated. Copyright © 2016 John WileySons, Ltd.

International audience; We report a patient with an unusual initial metabolic presentation of imported human rabies who became symptomatic within 2 weeks of returning from Mali to France. This is the single case of imported human rabies identified in France within the past 11 years and the first report of viral RNA in bronchial secretions. CASE REPORT A 57-year-old male without past medical history presented to an emergency room in March 2014 because of fever and generalized pain. He had been a resident of France for the previous 15 years and had returned 2 weeks earlier following a 6-month stay in Mali. Approximately 1 month before his return to France, he underwent a right foot injury from a tree branch which eventually healed with local care. On admission, he was conscious and cooperative but appeared anxious; he had fever (38.2°C) and abundant sweating and complained of generalized pain, mostly in the lower limbs. A clean, noninflammatory wound scar was noted on the right foot. He had marked tachypnea (rate, 40 inspirations/minute) and periodic deeper inspirations. Chest examination results were unremark-able. Arterial blood gas measurements while the patient was breathing room air were as follows: pH 7.79; partial pressure of carbon dioxide (PaCO 2), 11 mm Hg; PaO 2 , 136 mm Hg; bicar-bonates, 16 mmol/liter; and lactate, 3.7 mmol/liter. The capillary blood glucose was measured at 12 mmol/liter, and a urine dipstick revealed glycosuria (3) and ketonuria (3). Serum creatinine was at 94 mol/liter and sodium at 136 mmol/liter. Thick and thin blood smears were negative for malaria. Chest X-ray results were normal. The patient was transferred to our intensive care unit (ICU) with a presumptive diagnosis of uncontrolled diabetes and unexplained severe respiratory alkalosis. He received intravenous fluids, continuous insulin infusion, and tetanus prevention with vaccination and serotherapy. Hyperventilation, ketonuria, and metabolic abnormalities resolved within 2 days, with normaliza-tion of pH and PaCO 2. Glycated hemoglobin was 6.5%, and the patient required no further insulin administration during his ICU stay. Two days after ICU admission, he developed bouts of hyper-activity, disorientation, and delirium with thoughts of impending death associated with persecution ideas, alternating with periods of drowsiness and returns to normal behavior when he seemed aware of his disorder and criticized it. Hypersalivation was remarkable , and the patient occasionally spat on ICU personnel. Motor weakness, deep tendon reflexes, and limb sensory perceptions were normal. The results of a computed tomography scan and magnetic resonance imaging (MRI) of the brain were unre-markable. Electroencephalogram results showed no epileptic activity. The cerebrospinal fluid (CSF) test result was normal, with a negative PCR test for herpes simplex virus 1 (HSV-1) and HSV-2. Serological tests for human immunodeficiency virus type 1 (HIV-1) and HIV-2 and HIV-1 p24 antigen were negative. Thyroid stimulating hormone and ammonia levels were in the normal range. Syphilis serologic tests, including Venereal Disease Research Laboratory (VDRL) and Treponema Pallidum Hem Agglutination (TPHA) tests, were negative. On day 8 of ICU admission, the patient developed a rapidly extensive flaccid and areflectic tetraparesis without involvement of cranial nerves. His condition deteriorated, with altered consciousness and hypercapnic acidosis, and the patient required mechanical ventilation on day 9. Results of a repeated CSF analysis were normal. Results of a second cerebral and cervical MRI test were normal. Electroneuromyography showed an acute motor ax-onal neuropathy of both upper and lower limbs without sensory impairment. The results of a search for antineuron antibodies were negative, and the urinary porphobilinogen level was normal. The patient received methylprednisolone (1 g/day from day 8 to day 10) and intravenous immunoglobulin (2 g/kg of body weight from day 8 to day 12) for suspected paraneoplastic limbic enceph-alitis or Guillain-Barré syndrome. A search for rabies was performed on a skin biopsy specimen and salivary swabs obtained on day 13. A profound coma with periodic inspiratory spasms persisted , and the patient died on day 19 after ICU admission.

As Zika virus spreads, the associated clinical syndromes need to be defined. In this report, an 81-year-old man is found to have Zika virus–associated meningoencephalitis.

International audience; Patients with Huntington's disease (HD) are often described as unaware of their motor symptoms, their behavioral disorders or their cognitive deficits, including memory. Nevertheless, because patients with Parkinson's disease (PD) remain aware of their memory deficits despite striatal dysfunction, we hypothesize that early stage HD patients in whom degeneration predominates in the striatum can accurately judge their own memory disorders whereas more advanced patients cannot. In order to test our hypothesis, we compared subjective questionnaires of memory deficits (in HD patients and in their proxies) and objective measures of memory dysfunction in patients. Forty-six patients with manifest HD attending the outpatient department of the French National Reference Center for HD and thirty-three proxies were enrolled. We found that HD patients at an early stage of the disease (Stage 1) were more accurate than their proxies at evaluating their own memory deficits, independently from their depression level. The proxies were more influenced by patients' functional decline rather than by patients' memory deficits. Patients with moderate disease (Stage 2) misestimated their memory deficits compared to their proxies, whose judgment was nonetheless influenced by the severity of both functional decline and depression. Contrasting subjective memory ratings from the patients and their objective memory performance, we demonstrate that although HD patients are often reported to be unaware of their neurological, cognitive and behavioral symptoms, it is not the case for memory deficits at an early stage. Loss of awareness of memory deficits in HD is associated with the severity of the disease in terms of CAG repeats, functional decline, motor dysfunction and cognitive impairment, including memory deficits and executive dysfunction.

PURPOSE: Huntington's disease is a rare condition. Patients are commonly treated with antipsychotics and tetrabenazine. The evidence of their effect on disease progression is limited and no comparative study between these drugs has been conducted. We therefore compared the effectiveness of antipsychotics on disease progression. METHODS: 956 patients from the Huntington French Speaking Group were followed for up to 8 years between 2002 and 2010. The effectiveness of treatments was assessed using Unified Huntington's Disease Rating Scale (UHDRS) scores and then compared using a mixed model adjusted on a multiple propensity score. RESULTS: 63% of patients were treated with antipsychotics during the survey period. The most commonly prescribed medications were dibenzodiazepines (38%), risperidone (13%), tetrabenazine (12%) and benzamides (12%). There was no difference between treatments on the motor and behavioural declines observed, after taking the patient profiles at the start of the drug prescription into account. In contrast, the functional decline was lower in the dibenzodiazepine group than the other antipsychotic groups (Total Functional Capacity: 0.41 ± 0.17 units per year vs. risperidone and 0.54 ± 0.19 vs. tetrabenazine, both p

Second person social cognition cannot be restricted to dyadic interactions between two persons (the “I” and the “you”). Many instances of social communication are triadic, and involve a third person (the “him/her/it”), which is the object of the interaction. We discuss neuropsychological and brain imaging data showing that triadic interactions involve dedicated brain networks distinct from those of dyadic interactions.

International audience; Background A feeling of presence (FP), that is, the vivid sensation that somebody (distinct from oneself) is present nearby, is commonly reported by patients with Parkinson's disease (PD), but its phenomenology has not been described precisely. The objective of this study was to provide a detailed description of FP in PD and to discuss its possible mechanisms. Patients and methods The authors studied 52 non-demented PD patients reporting FP in the preceding month (38 consecutive outpatients and 14 inpatients). FP characteristics were recorded with a structured questionnaire. The outpatients with FP were compared with 78 consecutive outpatients without FP. Results About half the patients said they recognised the 'identity' of the presence. More than 75% of patients said the FP were not distressing, were short-lasting, were felt beside and/or behind the patient, and occurred while indoors; most patients checked for a real presence, but their insight was generally preserved. In 31% of cases, the patients had an unformed visual hallucination simultaneously with the FP. A higher daily levodopa-equivalent dose and the presence of visual illusions or hallucinations were independently associated with FP. Discussion Although FP is not a sensory perception, projection of the sensation into the extrapersonal space, along with the frequent co-occurrence of elementary visual hallucinations and the strong association with visual hallucinations or illusions, supports its hallucinatory nature. FP may be viewed as a 'social' hallucination, involving an area or network specifically activated when a living being is present, independently of any perceptual clue.

Humans usually point at objects to communicate with other persons, although they generally avoid pointing at the other's body. Moreover, patients with heterotopagnosia after left parietal damage cannot point at another person's body parts, although they can point at objects and at their own body parts and although they can grasp the others' body parts. Strikingly, their performance gradually improves for figurative human body targets. Altogether, this suggests that the body of another real person holds a specific status in communicative pointing. Here, we test in healthy individuals whether performance for communicative pointing is influenced by the communicative capacity of the target. In Experiment 1, pointing at another real person's body parts was compared to pointing at objects, and in Experiment 2, the person was replaced by a manikin. While reaction times for pointing at objects were shorter compared to pointing at other person's body parts, they were similar for objects and manikin body parts. By adapting Experiment 1 to PET-scan imaging (Experiment 3), we showed that, compared to pointing at objects, the brain network for pointing at other person's body parts involves the left posterior intraparietal sulcus, lesion of which could cause heterotopagnosia. Taken together, our results indicate that the specificity of pointing at another person's body goes beyond the visuo-spatial features of the human body and might rather rely on its communicative capacity.

Communicative pointing is a human specific gesture which allows sharing information about a visual item with another person. It sets up a three-way relationship between a subject who points, an addressee and an object. Yet psychophysical and neuroimaging studies have focused on non-communicative pointing, which implies a two-way relationship between a subject and an object without the involvement of an addressee, and makes such gesture comparable to touching or grasping. Thus, experimental data on the communicating function of pointing remain scarce. Here, we examine whether the communicative value of pointing modifies both its behavioral and neural correlates by comparing pointing with or without communication. We found that when healthy participants pointed repeatedly at the same object, the communicative interaction with an addressee induced a spatial reshaping of both the pointing trajectories and the endpoint variability. Our finding supports the hypothesis that a change in reference frame occurs when pointing conveys a communicative intention. In addition, measurement of regional cerebral blood flow using H(2)O(15) PET-scan showed that pointing when communicating with an addressee activated the right posterior superior temporal sulcus and the right medial prefrontal cortex, in contrast to pointing without communication. Such a right hemisphere network suggests that the communicative value of pointing is related to processes involved in taking another person's perspective. This study brings to light the need for future studies on communicative pointing and its neural correlates by unraveling the three-way relationship between subject, object and an addressee.

International audience; New criteria for Parkinson's disease-associated psychosis (PDAP) were recently proposed by a NINDS-NIMH working group. We assessed 116 consecutive unselected outpatients with PD for the existence of psychotic symptoms during the previous month, using a structured questionnaire covering the whole spectrum of PDAP symptoms. Hallucinations occurred in 42% of the patients (visual: 16%; nonvisual: 35%), delusions in 4%, and minor symptoms in 45% (sense of presence, visual illusions, or passage hallucinations). The prevalence of PDAP was 43% when the usual definition was used (hallucinations and/or delusions) and 60% when the NINDS-NIHM criteria were used. Correlations between PDAP and patient characteristics varied with the definition of PDAP. These findings suggest that the epidemiology of PDAP should be re-evaluated with the new criteria. Minor symptoms and nonvisual hallucinations are an important part of the PDAP spectrum, which has commonly been restricted to visual hallucinations and delusions.