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Resume La detection des acides gras est une notion nouvelle importante, pouvant influencer le comportement alimentaire. Les acides gras libres pourraient activer la voie afferente gustative comme toute autre saveur primaire, ce qui a pu etre observe en enregistrant des potentiels evoques gustatifs cerebraux en reponse a des solutions d’acides gras libres. Ces nouvelles donnees pourraient avoir un impact fort dans la comprehension de la physiopathologie de l’obesite. La suralimentation dans l’obesite est en partie due a l’alteration du feedback homeostatique du statut energetique peripherique. La nourriture est un element cle de l’alimentation hedonique basee sur la recompense, qui a des consequences sur la consommation de calories et les capacites sensorielles. Dans ce contexte, la detection des acides gras, alteree selon certains auteurs dans l’obesite, ne serait qu’une consequence de l’exces de poids. Celle-ci serait essentiellement modifiee par le comportement ingestif et la composition corporelle du fait d’une alteration du systeme de recompense pour les aliments palatables, riches en energie (lipides et glucides). La prise en compte d’une alteration du systeme de recompense dans l’obesite ouvre des perspectives nouvelles quant a son diagnostic, a sa prise en charge et a son traitement.

Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disorder resulting from biallelic alterations of the SMN1 gene: deletion, gene conversion or, in rare cases, intragenic variants. The disease severity is mainly influenced by the copy number of SMN2, a nearly identical gene, which produces only low amounts of full-length (FL) mRNA. Here we describe the first example of retrotransposon insertion as a pathogenic SMN1 mutational event. The 50-year-old patient is clinically affected by SMA type III with a diagnostic odyssey spanning nearly 30 years. Despite a mild disease course, he carries a single SMN2 copy. Using Exome Sequencing and Sanger sequencing, we characterized a SVA-F retrotransposon inserted in SMN1 intron 7. Using RT-PCR and RNASeq experiments on lymphoblastoid cell lines, we documented the dramatic decrease of FL transcript production in the patient compared to subjects with the same SMN1 and SMN2 copy number, thus validating the pathogenicity of this SVA insertion. We characterized the mutant FL-SMN1-SVA transcript and showed that it is subject to degradation by nonsense-mediated mRNA decay. The stability of the SMN-SVA protein may explain the mild course of the disease. This observation exemplifies the role of retrotransposons in human genetic disorders.

ObjectiveTo assess the efficiency and relevance of clinical exome sequencing (cES) as a first-tier or second-tier test for the diagnosis of progressive neurological disorders in the daily practice of Neurology and Genetic Departments.MethodsSixty-seven probands with various progressive neurological disorders (cerebellar ataxias, neuromuscular disorders, spastic paraplegias, movement disorders and individuals with complex phenotypes labelled ‘other’) were recruited over a 4-year period regardless of their age, gender, familial history and clinical framework. Individuals could have had prior genetic tests as long as it was not cES. cES was performed in a proband-only (60/67) or trio (7/67) strategy depending on available samples and was analysed with an in-house pipeline including software for CNV and mitochondrial-DNA variant detection.ResultsIn 29/67 individuals, cES identified clearly pathogenic variants leading to a 43% positive yield. When performed as a first-tier test, cES identified pathogenic variants for 53% of individuals (10/19). Difficult cases were solved including double diagnoses within a kindred or identification of a neurodegeneration with brain iron accumulation in a patient with encephalopathy of suspected mitochondrial origin.ConclusionThis study shows that cES is a powerful tool for the daily practice of neurogenetics offering an efficient (43%) and appropriate approach for clinically and genetically complex and heterogeneous disorders.

International audience; PURPOSE: Social cognition disorders after stroke are poorly described. Yet, rehabilitation difficulties are frequent after stroke. This study aimed to evaluate the frequency of social cognition disorders 3 years after a first-ever stroke and to assess the factors associated with this condition. The second aim was to describe all the cognitive domains altered in the same population. METHODS: Patients who suffered from a first-ever ischemic or hemorrhagic stroke underwent clinical and neuropsychological assessment, which included the mini-Social cognition and Emotional Assessment (SEA) for evaluating social cognition. RESULTS: The 43 included patients were 67+/-15 years old, with a median Neurological Institute of Health Stroke Scale (NIHSS) (+/- interquartile range) at 0+/-1, and a median modified Rankin Scale (+/- interquartile range) at 1+/-1. Twenty patients (46.5%) had poor results in the facial emotions subtest; this factor was associated with a low educational grade (P=0.001). Fourteen patients (34.2%) had poor results on the "faux-pas" recognition subtest; this factor was associated with nonverbal episodic memory disorders (P=0.01). Thirty four patients (79.1%) had cognitive impairment, with at least 1 cognitive domain affected. CONCLUSIONS: The study demonstrates the high frequency of social cognition impairment 3 years after the first-ever stroke in young patients. Doctors and nurses should be sensitized to cognitive handicap after stroke because of difficulties for rehabilitation and returning to work.

Protein-energy wasting is a risk factor for mortality and morbidity in hemodialysis patients (HD patients). Food intake could be modified by HD-related changes in the food reward system (i.e., liking and wanting of specific macronutrients). In HD patients on days with and without dialysis, we evaluated (1) the reward system for protein-, fat-, and carbohydrate-rich foods, plasma hormones, and metabolite changes; and (2) the spontaneous ad libitum intake of macronutrients.Twenty-four HD patients evaluated their liking and wanting of macronutrients at 7:30 AM and 11:30 AM on a day with and a day without dialysis. Concentrations of hormones and plasma amino acids were determined. An additional 18 HD patients ate what they wanted from a buffet lunch comprising 8 dishes on a day with and a day without dialysis. Healthy subjects, age-, sex-, and body mass index-matched, served as controls.At 11:30 AM, wanting for protein-rich foods was higher on the day with than on the day without dialysis (P .01), bringing wanting levels close to those of healthy subjects. This increase correlated with changes in the concentrations of plasma amino acids (P .01). HD patients ate more protein from the buffet on the day with than on the day without dialysis (P .01) and more than healthy subjects (P .01).In HD patients, wanting and spontaneous intake of protein-rich foods increase immediately after dialysis. This increase correlated with decreased concentrations of plasma amino acids. Thus, in clinical practice, protein-rich foods should be recommended during and after dialysis in patients with protein-energy wasting.

Small intestinal bacterial overgrowth (SIBO) is a common complication of bariatric surgery. Digestive decontamination treatments with oral antibiotic therapy vary and are not codified. This retrospective study was conducted to analyse the characteristics of bariatric surgery patients who underwent a glucose breath test (GBT) and to analyse the effectiveness of the antibiotic decontamination therapy. A total of 101 operated patients (Roux-en-Y bypass (RYB), omega bypass (ΩB) and sleeve gastrectomy (SG)) who underwent a GBT (75 g/250 mL) were included. Anthropometric data, symptoms of SIBO, type of surgery, use of proton pump inhibitors (PPIs) and antibiotic therapy were analysed. The effectiveness of the antibiotic treatment, defined by improvement of the symptoms, was evaluated during the follow-up. Of the 85 women and 16 men included (48.5 ± 3.6 years old), 63 underwent RYB, 31 underwent ΩB and 7 underwent SG. The GBT was positive in 83% of the patients. A positive test was associated with age (p

Zusammenfassung. Zerebrale Mikroangiopathien umfassen eine Gruppe neuropathologischer Prozesse, die die kleinen Perforationsarterien, Arteriolen und Kapillaren beeinflussen. Neuroimaging-Merkmale der Einengung der kleinen Koronararterienäste (Small Vessel Disease) sind ischämische und hämorrhagische Marker: jüngster kleiner subkortikaler Infarkt, Lakune vermuteten vaskulären Ursprungs, Hyperintensität der weissen Masse, perivaskuläre Räume, zerebrale Mikroblutungen, Hirnatrophie, kortikale oberflächliche Siderose und akute subarachnoidale Blutungen. Diese Läsionen gelten als der Hauptrisikofaktor der vaskulären kognitiven Störungen. Die zerebrale Mikroangiopathie wird typischerweise in zwei Hauptformen kategorisiert: die nicht-amyloide Angiopathie, die auch als «hypertensive Arteriopathie» bezeichnet wird, die oft mit fortgeschrittenem Alter und vaskulären Risikofaktoren und der zerebralen Amyloidangiopathie zusammenhängt. Die zerebrale Amyloidangiopathie ist klinisch charakterisiert durch kognitive Beeinträchtigungen, intrazerebrale Blutungen und durch transiente Episoden fokal-neurologischer Defizite aufgrund von Subarachnoidalblutungen.

Glucose, fructose, and sucrose are important carbohydrates in Western diets with particular sweetness intensity and metabolisms. No study has compared their cerebral detection and their taste perception. Gustatory evoked potentials (GEPs), taste detection thresholds, intensity perception, and pleasantness were compared in response to glucose, fructose, and sucrose solutions at similar sweetness intensities and at identical molar concentrations. Twenty-three healthy subjects were randomly stimulated with 3 solutions of similar sweetness intensity (0.75 M of glucose, 0.47 M of fructose and 0.29 M of sucrose – sit. A), and with an identical molar concentration (0.29 M – sit. B). GEPs were recorded at gustatory cortex areas. Intensity perception and hedonic values of each solution were evaluated as were gustatory thresholds of the solutions. No significant difference was observed concerning the GEP characteristics of the solutions according to their sweetness intensities (sit. A) or their molar concentration (sit. B). In sit. A, the 3 solutions were perceived to have similar intensities and induced similar hedonic sensations. In sit. B, the glucose solution was perceived to be less intense and pleasant than the fructose and the sucrose solutions (P < 0.001) and the fructose solution was perceived to be less intense and pleasant than the sucrose (P < 0.001). Since GEP recordings were similar for glucose, fructose, and sucrose solutions whatever the concentrations, activation of same taste receptor induces similar cortical activation, even when the solutions were perceived differently. Sweet taste perception seems to be encoded by a complex chemical cerebral neuronal network.

International audience; AIMS: GLP-1 analogues decrease food intake and have great promise for the fight against obesity. Little is known about their effects on food hedonic sensations and taste perception in poor controlled patients with type 2 diabetes (T2D). MATERIALS AND METHODS: Eighteen T2D patients with BMI >/=25kg/m(2) and poor controlled glycemia were studied before and after 3 months of treatment with Liraglutide. Detection thresholds for salty, sweet and bitter tastes, optimal preferences, olfactory liking, wanting and recalled liking for several food items were assessed. Subjects also answered questionnaires to measure their attitudes to food. RESULTS: T2D patients had a significant decrease in bodyweight and HbA1c after treatment with Liraglutide. Liraglutide improved gustative detection threshold of sweet flavors, and decreased wanting for sweet foods and recalled liking for fatty foods. It also led to a decrease in feelings of hunger. CONCLUSIONS: Liraglutide increases sensitivity to sweet tastes and decreases pleasure responses for fatty foods in poor controlled T2D patients, and is of particular interest in the understanding of the mechanisms of weight loss. CLINICAL TRIAL: NCT02674893.

ObjectiveTo genotypically and phenotypically characterize a large pediatric myotonic dystrophy type 1 (DM1) cohort to provide a solid frame of data for future evidence-based health management.MethodsAmong the 2,697 patients with genetically confirmed DM1 included in the French DM-Scope registry, children were enrolled between January 2010 and February 2016 from 24 centers. Comprehensive cross-sectional analysis of most relevant qualitative and quantitative variables was performed.ResultsWe studied 314 children (52% females, with 55% congenital, 31% infantile, 14% juvenile form). The age at inclusion was inversely correlated with the CTG repeat length. The paternal transmission rate was higher than expected, especially in the congenital form (13%). A continuum of highly prevalent neurodevelopmental alterations was observed, including cognitive slowing (83%), attention deficit (64%), written language (64%), and spoken language (63%) disorders. Five percent exhibited autism spectrum disorders. Overall, musculoskeletal impairment was mild. Despite low prevalence, cardiorespiratory impairment could be life-threatening, and frequently occurred early in the first decade (25.9%). Gastrointestinal symptoms (27%) and cataracts (7%) were more frequent than expected, while endocrine or metabolic disorders were scarce.ConclusionsThe pedDM-Scope study details the main genotype and phenotype characteristics of the 3 DM1 pediatric subgroups. It highlights striking profiles that could be useful in health care management (including transition into adulthood) and health policy planning.

Introduction Amputation of the hand is a rare and extremely intense trauma. Replanting and allografting after this type of injury require a major reorganization of the brain. Brain plasticity, though better known in the context of disorders of the central nervous system, is just as indispensable when the extremities are damaged. Materials and methods A 17-year-old patient underwent replantation of the nondominant hand after transmetaphyseal amputation after traumatic injury. After 18 days in hospital and subsequent treatment in a physical rehabilitation center, the patient attended clinical and radiology follow-up sessions over the next 2 years. Results The management of this patient led to an excellent functional outcome in conjunction with successful social and professional reintegration. Electromyography at 18 months confirmed nerve regrowth. Functional magnetic resonance imaging was done at 2 years to evaluate cerebral plasticity. Motor function, largely dependent on the primary motor area, is aided by the addition of secondary and accessory motor areas for both simple and complex movements. A change in sensory information is stimulation in its own right hemisphere and increases solicitation of the contralateral precentral and postcentral gyrus. Conclusions There seems to be a real reversible dynamic plasticity under the balance of inhibitory and excitatory influences exerted on the cortical neurons. Any disruption of this balance requires the brain to adapt to the new circumstances to reestablish the hand as a functioning part of the body.

Communication orale de 32 diapositives dans conférence organisée par la Société de Physiologie et de Biologie Intégrative.; National audience

Introduction et but de l’etude La colonisation bacterienne chronique du grele (CBCG), diagnostiquee par un test respiratoire au glucose, est une complication frequente de la chirurgie bariatrique. Les symptomes de la CBCG sont aspecifiques a type de douleurs abdominales, de ballonnement et de diarrhee. Les traitements de decontamination digestive par antibiotherapies orales sequentielles sont varies et non codifies. Le but de cette etude retrospective etait d’analyser les caracteristiques d’une cohorte de patients operes d’une chirurgie bariatrique ayant realise un test respiratoire au glucose pour une symptomatologie evocatrice de CBCG et d’analyser l’efficacite des antibiotherapies de decontamination digestive utilisees. Materiel et methodes Entre janvier 2018 et avril 2019, 101 patients operes d’une chirurgie bariatrique (by-pass en Roux-en-Y, en omega et sleeve-gastrectomie) ayant realise un test respiratoire au glucose au Centre Hospitalier Universitaire de Dijon (50 g per os, test positif defini par une [H2] basale > 20 ppm ou une elevation par rapport a la basale > 10 ppm dans les 2 premieres heures), ont ete inclus. Les donnees anthropometriques, les symptomes, le type de chirurgie bariatrique, la prise d’inhibiteurs de pompe a proton (IPP), l’antibiotherapie et sa duree ont ete recueillies de maniere retrospective. L’efficacite du traitement antibiotique, definie par une amelioration des symptomes constatee par le patient etait evaluee lors des consultations de suivi ou par telephone. Des tests de Chi2 ont ete realises pour les analyses statistiques avec p Resultats et analyse statistique Sur les 101 patients inclus (85 femmes, 16 hommes, âge 48,5 ± 3,6 ans), 63 avaient beneficie d’un by-pass en Roux-en-Y, 31 d’un by-pass en omega et 7 d’une sleeve. 76 patients presentaient une diarrhee, 52 une douleur abdominale et 20 des ballonnements. Le test respiratoire au glucose etait positif chez 84 patients. Un test positif etait associe significativement a la prise d’IPP (p Conclusion La CBCG doit etre systematiquement recherchee en cas de symptomatologie evocatrice chez des patients aux antecedents de chirurgie bariatrique, quel que soit le type de chirurgie, mais plus particulierement chez les patients les plus âges, de genre feminin et en cas de traitement par IPP. L’efficacite semblait similaire entre les 2 antibiotherapies sequentielles orales de decontamination digestive les plus frequemment prescrites dans notre cohorte (gentamycine/metronidazole et metronidazole).

Open access article 15 p. (manuscript ID: nutrients-695395); International audience; Aspartame and Stevia are widely substituted for sugar. Little is known about cerebral activation in response to low-caloric sweeteners in comparison with high-caloric sugar, whereas these molecules lead to different metabolic effects. We aimed to compare gustatory evoked potentials (GEPs) obtained in response to sucrose solution in young, healthy subjects, with GEPs obtained in response to aspartame and Stevia. Twenty healthy volunteers were randomly stimulated with three solutions of similar intensities of sweetness: Sucrose 10 g/100 mL of water, aspartame 0.05 g/100 mL, and Stevia 0.03 g/100 mL. GEPs were recorded with EEG (Electroencephalogram) electrodes. Hedonic values of each solution were evaluated using the visual analog scale (VAS). The main result was that P1 latencies of GEPs were significantly shorter when subjects were stimulated by the sucrose solution than when they were stimulated by either the aspartame or the Stevia one. P1 latencies were also significantly shorter when subjects were stimulated by the aspartame solution than the Stevia one. No significant correlation was noted between GEP parameters and hedonic values marked by VAS. Although sucrose, aspartame, and Stevia lead to the same taste perception, cerebral activation by these three sweet solutions are different according to GEPs recording. Besides differences of taste receptors and cerebral areas activated by these substances, neural plasticity, and change in synaptic connections related to sweet innate preference and sweet conditioning, could be the best hypothesis to explain the differences in cerebral gustatory processing after sucrose and sweeteners activation.

International audience; Pain in amyotrophic lateral sclerosis (ALS) is paradoxical in this disease of the upper and lower motor neurons. As such, it remains an underestimated and neglected clinical problem because it is poorly identified by physicians, its mechanisms are numerous and its treatments are generally not effective. Pain may be primary in the form of cramps, spasticity and neuropathy, or secondary as nociceptive pain, and may arise before the first motor symptoms. It may also lead to depression and, in all cases, affect patients' daily activities and quality of life. Given the high frequency of pain in ALS, the use of analgesic or sedative drugs is necessary and should reduce the course of the disease. Nevertheless, it is important to understand the pathophysiological mechanisms of pain in ALS, and to train physicians how to detect ALS pain early on and provide dedicated treatments. In France, the implementation of ALS centers is a positive response to the public-health problem resulting from this disorder.

Etude de cas; Introduction: Moyamoya syndrome is a rare progressive cerebrovascular occlusive disease for which several associated conditions have been described. Case Report: We report the case of a 76-year-old woman with a history of May-Hegglin anomaly who presented with an isolated unusual diffuse headache. Initial laboratory investigations showed only thrombocytopenia (platelet count 95000/mu L). Unenhanced brain computed tomography scan revealed a small subarachnoid hemorrhage in the left frontal lobe. Computed tomography angiography showed occlusion of the terminal portion of the left internal carotid artery (ICA) and narrowing of the terminal portion of the right ICA with abnormal collateral vessels. Conventional angiography confirmed the tight stenosis of the extremity of the right ICA, and the occlusion of the left ICA, associated with an extensive collateral network consistent with moyamoya syndrome. No aneurysm was identified. Conclusions: The rarity of these 2 diseases makes a fortuitous association very unlikely. We thus concluded that this observation could be the first reported case of May-Hegglin anomaly as a cause of moyamoya syndrome.

BACKGROUND Because of the aging population and a rise in the number of stroke survivors, the prevalence of post-stroke cognitive impairment (PSCI) is increasing. OBJECTIVE To identify the factors associated with 3-month PSCI. METHODS All consecutive stroke patients without pre-stroke dementia, mild cognitive disorders, or severe aphasia hospitalized in the Neurology Department of Dijon, University Hospital, France (November 2010 - February 2012) were included in this prospective cohort study. Demographics, vascular risk factors, and stroke data were collected. A first cognitive evaluation was performed during the hospitalization using the Mini-Mental State Exam (MMSE) and the Montreal Cognitive Assessment (MOCA). Patients assessable at 3 months were categorized as cognitively impaired if the MMSE score was ≤26/30 and MOCA

We aimed to evaluate the influence of prestroke cognitive impairment (PSCI) on outcomes in stroke patients treated with IV recombinant tissue plasminogen activator (rtPA).OPHELIE-COG was a prospective observational multicenter study conducted in French and Japanese patients treated with IV rtPA for cerebral ischemia. The preexisting cognitive status was evaluated by the short version of the Informant Questionnaire on Cognitive Decline in the Elderly. PSCI was defined as a mean score3. The primary endpoint was a favorable outcome (modified Rankin Scale [mRS] score 0-1) after 3 months. Secondary endpoints were symptomatic intracerebral hemorrhage (sICH), mRS scores 0-2, and mortality at 3 months. We performed a pooled analysis with Biostroke and Strokdem.Of 205 patients, 62 (30.2%) met criteria for PSCI. They were 11 years older (p0.001). Although they had more sICH and were less frequently independent after 3 months, they did not differ for any endpoint after adjustment for age, baseline NIH Stroke Scale score, and onset-to-needle time: sICH (odds ratio [OR] 2.78; 95% confidence interval [CI] 0.65-11.86), mRS 0-1 (OR 0.82; 95% CI 0.41-1.65), mRS 0-2 (OR 0.62; 95% CI 0.28-1.37), death (OR 0.40; 95% CI 0.08-2.03). The pooled analysis found no association of PSCI with any endpoint.Ischemic stroke patients with PSCI should receive rtPA if they are eligible. This conclusion cannot be extended to severe cognitive impairment or severe strokes.This study provides Class IV evidence that in patients with PSCI presenting with acute ischemic stroke, IV rtPA improves outcomes.

Background Giant cell arteritis (GCA) is the most common vasculitis in people ≥50 years and can be associated with stroke. We aimed to evaluate the epidemiology and characteristics of stroke in patients with GCA. Methods All patients with a biopsy-proven diagnosis of GCA were identified among residents of the city of Dijon, France (152 000 inhabitants), between 2001 and 2012 using a prospective database. Among these, patients who suffered from stroke were retrieved by crossing data from the population-based Dijon Stroke Registry. Demographics and clinical features were recorded. We considered that the stroke was GCA-related if the stroke revealed GCA or occurred between the onset of symptoms and 4 weeks after the start of treatment. Results Among the 57 biopsy-proven patients with GCA (incidence rate 10.9/100 000/year in individuals ≥50 years), 4 (7.0%) experienced a GCA-related stroke. Three were men and all had ≥2 vascular risk factors and were ≥80 years. The stroke was vertebrobasilar for 3/4 patients and undetermined for the remaining one. The incidence rate of GCA-related stroke in patients ≥50 years was 0.76/100 000/year (95% CI 0 to 2.47), 1.36/100 000/year in men (95% CI 0 to 3.63) and 0.33/100 000/year (95% CI 0 to 1.45) in women. Conclusions This population-based study demonstrated that GCA-related stroke essentially affects the vertebrobasilar territory and mainly occurs in old men with associated vascular risk factors. Although rare, GCA symptoms must be searched for in elderly patients with stroke, and optimal vascular prevention must be conducted carefully in patients with GCA with a high vascular risk before initiating GCA treatment.

Background: Acute stress may trigger vascular events. We aimed to investigate whether important football competitions involving the French football team increased the occurrence of stroke. Methods: We retrospectively retrieved data of fatal and nonfatal stroke during 4 World Football Cups (1986, 1998, 2002, and 2006) and 4 European Championships (1992, 1996, 2000, and 2004), based on data from the population-based Stroke Registry of Dijon, France. One period of exposure was analyzed: the period of competition extended to 15 days before and after the competitions. The number of strokes was compared between exposed and unexposed corresponding periods of preceding and following years using Poisson regression. Results: A total of 175 strokes were observed during the exposed periods compared with 192 and 217 strokes in the unexposed preceding and following periods. Multivariate regression analyses showed an overall 30% significant decrease in stroke numbers between periods of competition and unexposed periods of following year (risk ratio (RR) 5 1.3; 95% confidence interval [CI] 5 1.0-1.6; P 5.029) but not with that of preceding year (RR 5 1.1; 95% CI 5 .9-1.3; P 5.367). This was mostly explained by a 40% decrease in stroke numbers during European Championships, compared with the unexposed following periods (RR 5 1.4; 95% CI 5 1.0-1.9; P 5.044) in stratified analyses by football competitions. Conclusions: Watching European football competitions had a positive impact in the city of Dijon with a decrease of stroke numbers. European championship is possibly associated with higher television audience and long-lasting euphoria although other factors may be involved. Further studies using nationwide data are recommended to vali

Sporadic Creutzfeldt-Jakob disease (sCJD) generally manifests itself by cognitive or rapidly progressive motor symptoms. An atypical onset or an unusual evolution may delay the diagnosis. Among patients with a confirmed diagnosis of sCJD following a post-mortem neuropathological examination at the Neuropathology Centre of Lille, France, those who had presented with atypical cognitive disorders at onset were included in the study. Four patients were included. The first patient (64-years-old) presented early language disorders, later accompanied by apathy and behavioral disorders. The prolonged course suggested a diagnosis of progressive primary aphasia. The second patient (68-years-old) presented with aphasia, apraxia, and ataxia of the right upper limb with parkinsonian syndrome, suggesting corticobasal degeneration. In the two last patients (58- and 61-years-old), the onset was marked by an anxiety-depression syndrome, falls, visual hallucinations, extra-pyramidal syndrome, and fluctuating cognitive decline. The diagnosis raised was probable Lewy body dementia. The 14.3.3 protein was found in two of the four cases. The clinical elements found may initially suggest focal atrophy or Lewy body dementia. A very rapid clinical deterioration generally suggests sCJD, but in the last case, the evolution was particularly slow. The diagnosis of sCJD must be considered in cases of rapid-onset dementia, even if all of the clinical criteria are not present. The detection of the 14.3.3 protein and multifold increase in total-Tau with normal or slightly increased phosphorylated-Tau in the CSF are additional arguments to reinforce the diagnosis. The post-mortem neuropathological examination is important to confirm the diagnosis.

We aimed to investigate the impact of smoking status on clinical severity in patients with ischemic stroke event (IS). Patients were prospectively identified among residents of the city of Dijon, France (ca. 151,000 inhabitants), using a population-based registry, between 2006 and 2011. Demographic and clinical data were recorded. The initial clinical severity was quantified by the means of the National Institutes of Health Stroke Scale (NIHSS). Multivariable ordinal logistic regression was used to assess the effect of smoking status on severity. Among the 1,056 recorded patients with IS, data about smoking status were available for 973 (92.1%), of whom 658 (67.3%) were non-smokers, 187 (19.2%) were current smokers, and 128 (13.2%) were former smokers. Compared with non-smoking, former smoking was associated with less severe IS (OR 0.55; 95% CI 0.38-0.82, p = 0.003), whereas this association was not found for current smokers (OR 0.97; 95% CI 0.69-1.36, p = 0.856). Further work is needed to understand the underlying mechanisms of this finding.

Recent data have suggested that stroke incidence in young people may be rising. In this population-based study, we aimed to determine whether the incidence of stroke in people aged55 years old had changed over the last three decades.All cases of first-ever stroke (ischaemic stroke, spontaneous intracerebral haemorrhage, and undetermined stroke) occurring in Dijon, France, from 1985 to 2011 were prospectively collected from a population-based registry. Incidence rates were calculated and temporal trends were analysed by age groups and stroke subtypes using a Poisson regression to estimate incidence rate ratios (IRR). Risk factors and premorbid treatments were analysed.Over the 27-year study period, 4506 patients were recorded (53% women, mean age 74.6±14.4, 10.1% aged55 years). An increase in overall stroke incidence was noted, as was a rise in ischaemic stroke in individuals aged55 years (IRR 1.308; 95% CI 0.982 to 1.741, p=0.066 for period 1994-2002 vs period 1985-1993, and IRR 1.697; 95% CI 1.340 to 2.150, p0.001 for period 2003-2011 vs period 1994-2002), which was consistent for men and women. In these young patients, smoking was the most frequent risk factor (43%).Multiple factors may account for the increased incidence of ischaemic stroke in people aged55 years including changes in vascular risk factors, better awareness of the disease and treatment options in the population and among practitioners leading to more frequent referrals for specialised care, and improvements in stroke diagnosis. Stroke prevention must be encouraged even in young adults.

Introduction: Inclusion body myopathy with Paget's disease of the bone and frontotemporal dementia (IBMPFD) is a rare late-onset autosomal dominant disorder due to a mutation of the valosin-containing protein (VCP) gene. Case Report: We report the case of a patient who developed progressive weakness of the limbs in his fifties, until he was confined to a wheelchair. At that time, he developed acute behavioural changes including irritability, severe anxiety and major depression, which led to him being hospitalised in a psychiatric hospital. He also suffered from aphasia and executive function impairment, which helped us to diagnose a behavioural form of frontotemporal dementia (FTD). The diagnosis of IBMPFD due to a mutation in the VCP gene was confirmed by a genetic study of the VCP gene (R155H mutation). Discussion: The clinical diagnosis of IBMPFD is suggested by the presence of at least one of three major manifestations as follows: inclusion body myopathy (mean onset at 42 years of age), Paget's disease of the bone and FTD (mean onset at 55 years of age). It is mostly the behavioural form of FTD (behavioural changes, executive dysfunction and aphasia). One interesting finding in our report is the predominance of the psychiatric symptoms at the beginning of the behavioural changes, which led to the diagnosis of FTD. The diagnosis of IBMPFD was confirmed by the genetic study: the R155H mutation found on exon 5 domain CDC48 is the most frequent of the 18 known mutations in the VCP gene.

Background and purpose Suffering a stroke during the weekend is associated with a poorer prognosis. The impact of implementing a dedicated stroke care network in Dijon, France, in 2003 on 30-day mortality in strokes/transient ischaemic attacks (TIA) occurring during weekends/bank holidays was evaluated. Methods All cases of stroke and TIA from 1985 to 2010 were identified from a population-based registry, using multiple overlapping sources of information. Demographics and clinical data were recorded. Cox regression models were used to evaluate associations between day of onset (weekdays versus weekends/bank holidays) and 30-day all-cause mortality. Data were stratified according to time periods [before (1985–2003) and after (2004–2010) implementation of the stroke network] and stroke subtypes (ischaemic stroke and intracerebral hemorrhage). Results Of the 5864 recorded patients, 1465 (25%) had their event during weekends/bank holidays. Patients with stroke/TIA during weekdays were comparable with those with stroke/TIA during weekends/bank holidays for baseline characteristics. Excess mortality was observed in patients with stroke/TIA during weekends/bank holidays during 1985–2003 (18.2% vs. 14.0%, P

Summary Background We aimed to demonstrate that a combined stroke and telefibrinolysis network can reduce the rates of death and recurrent stroke events. In these two types of organisation, nurses play a pivotal role in all phases of stroke patient care. In our study, nurses are responsible for the coordination of care throughout the care continuum. In this paper, we describe the stroke and telestroke network created in 2004 in Burgundy, France, its positive impact on death and recurrent stroke rates, and how telefibrinolysis could improve these rates. Methods We evaluated the role of the clinical nurse in a multidisciplinary stroke network, called G5, established in 2003 between the Stroke Unit of Dijon University Hospital and four general regional hospitals, whose innovative mission was telephone follow-up of stroke patients discharged home. In 2012 a telefibrinolysis network was integrated into the G5 stroke network. Results The preliminary results concerning the role of the clinical nurse in a telefibrinolysis network demonstrate that the same efficacy is achieved without causing undesirable events such as increased cerebral haemorrhage. The stroke network and telestroke demonstrated that the role of the clinical nurse was beneficial in reducing the rate of recurrence by 16% in the first year. Conclusion Telefibrinolysis is a reliable procedure enabling a greater number of patients to be fibrinolysed. By monitoring patients discharged home, via a call-centre, clinical nurses play a strategic role in telestroke.

Background: We aimed to determine the accuracy of the iScore for predicting mortality and early disability following ischemic stroke in a French population-based study. Methods: All patients with acute ischemic stroke were identified among residents of the city of Dijon, France, between 2006 and 2011, using a population-based stroke registry. The 30-day iScore and 1-year iScore were calculated. A logistic regression model was used to assess the performance of the iScore for predicting both 30-day and 1-year mortality, and poor functional outcome at discharge (modified Rankin Scale score of 3-6). The discrimination and calibration of the model were assessed using the c-statistic and the Hosmer-Lemeshow goodness-of-fit test, respectively. Results: Among the 1,199 ischemic stroke patients recorded, 107 (8.9%) were excluded because at least one item of data was missing. For the remaining 1,092 patients, the c-statistic was 0.85 (95% CI: 0.82-0.89) for 30-day and 0.84 (0.81-0.87) for 1-year mortality, and calibration was good (p = 0.82 and p = 0.96, respectively, for the Hosmer-Lemeshow test). Similar results were found for disability (c-statistic 0.81, 95% CI: 0.79-0.84, and p = 0.45 for the Hosmer-Lemeshow test). Conclusion: This is the first population-based study to demonstrate the accuracy of the iScore for predicting mortality and early disability in ischemic stroke patients.

Background and purpose Previous antiplatelet therapy (APT) in cardiovascular prevention is common in patients with first-ever stroke. We aimed to evaluate the prognostic value of APT on early outcome in stroke patients. Methods All first-ever strokes from 1985 to 2011 were identified from the population-based Stroke Registry of Dijon, France. Demographic features, risk factors, prestroke treatments and clinical information were recorded. Multivariate analyses were performed to evaluate the associations between pre-admission APT and both severe handicap at discharge, and mortality at 1 month and 1 year. Results Among the 4275 patients, 870 (20.4%) were previously treated with APT. Severe handicap at discharge was noted in 233 (26.8%) APT users and in 974 (28.7%) non-users. Prestroke APT use was associated with lower odds of severe handicap at discharge [adjusted odds ratio (OR): 0.79; 95% confidence interval (CI): 063–1.00; P = 0.046], non-significant better survival at 1 month [adjusted hazard ratio (HR): 0.87; 95% CI: 0.70–1.09; P = 0.222] and no effect on 1-year mortality (HR: 0.94; 95% CI 0.80–1.10; P = 0.429). In stratum-specific analyses, APT was associated with a lower risk of 1-month mortality in patients with cardioembolic ischaemic stroke (HR: 0.65; 95% CI: 0.43–0.98; P = 0.040). Conclusions APT before stroke was associated with less severe handicap at discharge, with no significant protective effect for mortality at 1 month except in patients with cardioembolic stroke. No protective effect of APT was observed for mortality at 1 year. Further studies are needed to understand the mechanisms underlying the distinct effects of prior APT observed across the ischaemic stroke subtypes.

Background Dementia is a frequent condition in stroke patients. Aims To investigate the effect of dementia on access to diagnostic procedures in ischaemic stroke patients. Methods All cases of ischaemic stroke from 2006 to 2010 were identified from the population-based Stroke Registry of Dijon, France. Patients' characteristics were recorded, as was the use of brain computed tomography scans, brain magnetic resonance imaging, electrocardiogram, echocardiography, and Doppler ultrasonography of the cervical arteries. Dementia was diagnosed according to Diagnostic and Statistical Manual of Mental Disorders-IV criteria. Logistic regression models were used to evaluate the associations between dementia and the use of the diagnostic procedures. Results Of the 907 patients recorded, 104 were excluded because of death and inability to test cognition. Among the remaining 803 patients, 149 (18·5%) had dementia. Almost all of the patients underwent a brain computed tomography scan and an electrocardiogram during their stay. In contrast, the use of both Doppler ultrasonography of the cervical arteries (79·2% versus 90·2%, P < 0·001), echocardiography (32·9% versus 43·6%, P = 0·02), and brain magnetic resonance imaging (21·5% versus 34·4%, P < 0·001) were significantly lower in stroke patients with dementia than in those without. In multivariate logistic regression, dementia was associated with a lower use of both Doppler ultrasonography (odds ratio = 0·49; 95% confidence interval: 0·29–0·81, P = 0·005), echocardiography (odds ratio = 0·57; 95% confidence interval: 0·37–0·89, P = 0·012), brain magnetic resonance imaging (odds ratio = 0·55; 95% confidence interval: 0·34–0·89, P = 0·015), and a comprehensive assessment (odds ratio = 0·62; 95% confidence interval: 0·40–0·96, P = 0·033). Conclusion Demented patients were less likely to undergo diagnostic procedures after ischaemic stroke. Further studies are needed to determine whether this lower utilization could account for the reported excess in recurrent events in these patients.

International audience; Background: 25-Hydroxyvitamin D (25(OH)D) deficiency is a frequent condition in patients who suffer a stroke, and several studies suggested that it may be associated with a poorer prognosis. The aim of this study was to investigate specifically the association between 25(OH)D levels and functional outcome at 3 months in ischemic stroke patients treated with intravenous thrombolysis. Methods: Consecutive ischemic stroke patients who received intravenous thrombolysis were enrolled between 2010 and 2013. Baseline characteristics were collected, and serum concentrations of 25(OH)D were measured within the first 24 hours after admission and were analyzed according to the quartiles of their distribution (= 25 nmol/L). Multivariable ordinal logistic regression was used to evaluate the association between 25(OH)D and 3-month functional outcome assessed by the modified Rankin score. Results: Three hundred fifty-two patients were included (mean age 68.6 +/- 15.8, 50.7% women, mean 25(OH)D level 45 +/- 25 nmol/L). The characteristics of the patients only differed with regard to higher premorbid functional impairment in patients with low 25(OH)D. In univariate analysis, the risk of functional impairment in patients with low 25(OH)D levels was greater than that in patients with higher 25(OH) D levels (odds ratio [OR] 2.10, 95% confidence interval [CI]: 1.35-3.27, P = .001). This association was still observed after adjustment for confounding variables (OR 1.70, 95% CI: 1.06-2.71, P = .027). Conclusion: A low serum 25(OH)D level is associated with worse functional outcome in patients with acute ischemic stroke treated with intravenous thrombolysis. Further investigations are required to understand the underlying mechanisms of this association.

Background: Psychotropic drugs are frequently prescribed in nursing homes (NH). Nonetheless, we hoped that institutionalization decreases the number of psychotropic drug classes prescribed, because NH residents may have more psychosocial interventions than patients living at home. Objective: The aim was to compare the type and number of psychotropic drugs prescribed in elderly NH residents with dementia with those in community-living patients. Methods: This cross-sectional study included elderly patients (at least 75 years old) with dementia recorded in the National Alzheimer’s data Bank (“Banque Nationale Alzheimer”) during the year 2012 and who were taking at least one psychotropic drug. Psychotropic drugs were classified as follows: antidepressant, anxiolytic, hypnotic, and antipsychotic drugs. Patients were classified into three categories of dementia severity according to the MMSE score. Results: Among the 50,932 patients with dementia recorded in the BNA, 40.1% had at least one psychotropic drug prescribed. Most of the patients who were treated by at least one psychotropic drug class had antidepressant therapy (69.0%), whatever their residence type, and 16.1% were treated with antipsychotics. Among the study population, 51.9% of the NH residents and 67.4% of the patients living at home had only one psychotropic drug class prescribed. Living in a NH was significantly associated with the more frequent prescription of anxiolytic, hypnotic, and antipsychotic drugs, and with a greater number of psychotropic drug classes prescribed, whatever the severity of the dementia. Conclusion: We underlined the more frequent prescription of psychotropic drugs in NH residents regardless of MMSE scores.