Subarachnoid Hemorrhage Revealing Moyamoya Syndrome in a Patient With May-Hegglin Anomaly.

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/µL). 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.

Relevance of Post-Stroke Circulating BDNF Levels as a Prognostic Biomarker of Stroke Outcome. Impact of rt-PA Treatment.

The recombinant form of tissue plasminogen activator (rt-PA) is the only curative treatment for ischemic stroke. Recently, t-PA has been linked to the metabolism of brain-derived neurotrophic factor (BDNF), a major neurotrophin involved in post-stroke neuroplasticity. Thus, the objective of our study was to investigate the impact of rt-PA treatment on post-stroke circulating BDNF levels in humans and in animals. Serum BDNF levels and t-PA/plasmin activity were measured at hospital admission and at up to 90 days in stroke patients receiving (n = 24) or not (n = 14) rt-PA perfusion. We investigated the relationships between serum BDNF with concurrent t-PA/plasmin activity, neurological outcomes and cardiovascular scores at admission. In parallel, serum BDNF levels and t-PA/plasmin activity were assessed before and after (1, 4 and 24h) the induction of ischemic stroke in rats. Our study revealed higher serum BDNF levels and better neurological outcome in rt-PA-treated than non-treated patients. However, serum BDNF levels did not predict stroke outcome when the whole cohort of stroke patients was analyzed. By contrast, serum BDNF levels when measured at admission and at day 90 correlated with cardiovascular scores, and those at day 1 correlated with serum t-PA/plasmin activity in the whole cohort of patients whereas no association could be found in the rt-PA-treated group. In rats devoid of cardiovascular risk, no difference in post-stroke serum BDNF levels was detected between rt-PA- and vehicle-treated animals and no correlation was found between serum BDNF levels and t-PA/plasmin activity. Overall, the data suggest that serum BDNF levels may not be useful as a prognostic biomarker of stroke outcome and that endothelial dysfunction could be a confounding factor when serum BDNF levels after stroke are used to reflect of brain BDNF levels.

Cyclosporine in acute ischemic stroke.

OBJECTIVES: We examined whether IV administration of cyclosporine in combination with thrombolysis might reduce cerebral infarct size. METHODS: Patients aged 18 to 85 years, presenting with an anterior-circulation stroke and eligible for thrombolytic therapy, were enrolled in this multicenter, single-blinded, controlled trial. Fifteen minutes after randomization, patients received either an IV bolus injection of 2.0 mg/kg cyclosporine (Sandimmune, Novartis) or placebo. The primary endpoint was infarct volume on MRI at 30 days. Secondary endpoints included infarct volume according to the site (proximal/distal) of arterial occlusion and recanalization after thrombolysis. RESULTS: From October 2009 to July 2013, 127 patients were enrolled. The primary endpoint was assessed in 110 of 127 patients. The reduction of infarct volume in the cyclosporine compared with the control group was overall not significant (21.8 mL [interquartile range, IQR 5.1, 69.2 mL] vs 28.8 mL [IQR 7.7, 95.0 mL], respectively; p = 0.18). However, in patients with proximal occlusion and effective recanalization, infarct volume was significantly reduced in the cyclosporine compared with the control group (14.9 mL [IQR 1.3, 23.2 mL] vs 48.3 mL [IQR 34.5, 118.2 mL], respectively; p = 0.009). CONCLUSIONS: Cyclosporine was generally not effective in reducing infarct size. However, a smaller infarct size was observed in patients with proximal cerebral artery occlusion and efficient recanalization. CLASSIFICATION OF EVIDENCE: This study provides Class I evidence that in patients with an acute anterior-circulation stroke, thrombolysis plus IV cyclosporine does not significantly decrease 30-day MRI infarct volume compared with thrombolysis alone.

Diagnostic procedures in ischaemic stroke patients with dementia. a population-based study.

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.

Stroke associated with giant cell arteritis: a population-based study.

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.

Factors associated with early recurrence at the first evaluation of patients with transient ischemic attack.

We aimed to identify factors easily collected at admission in patients with transient ischemic attack (TIA) that were associated with early recurrence, so as to guide clinicians' decision-making about hospitalization in routine practice. From September 2011 to January 2013, all TIA patients who were referred to the University Hospital of Dijon, France, were identified. Vascular risk factors and clinical information were collected. The etiology of the TIA was defined according to the results of complementary examinations performed at admission as follows: large artery atherosclerosis (LAA-TIA) TIA, TIA due to atrial fibrillation (AF-TIA), other causes, and undetermined TIA. Logistic regression analyses were performed to identify factors associated with any recurrence at 48 hours (stroke or TIA). Among the 312 TIA patients, the etiology was LAA-TIA in 33 patients (10.6%), AF-TIA in 57 (18.3%), other causes in 23 (7.3%), and undetermined in 199 (63.8%). Early recurrence rates were 12.1% in patients with LAA-TIA, 5.3% in patients with AF-TIA, 4.3% in patients with another cause of TIA, and 1.0% in patients with undetermined TIA. In multivariable analysis, the LAA etiology was independently associated with early recurrence (odds ratio [OR]: 12.03; 95% confidence interval [CI]: 1.84-78.48, p=0.009). A non-significant trend was also observed for AF-TIA (OR: 3.82; 95% CI: 0.40-36.62, p=0.25) and other causes (OR: 3.73; 95% CI: 0.30-46.26, p=0.31). A simple initial assessment of TIA patients in the emergency room would be helpful in targeting those with a high risk of early recurrence and who therefore need to be hospitalized.

Association between serum concentration of vitamin D and 1-year mortality in stroke patients.

BACKGROUND: The prevalence of 25-hydroxyvitamin D [25(OH)D] deficiency is high in patients presenting with an acute stroke, and it may be associated with greater clinical severity and a poor early functional prognosis. However, no data about its impact on long-term prognosis is available. In this study, we aimed to assess the association between 25(OH)D levels and 1-year mortality in stroke patients. METHODS: From February to December 2010, 382 Caucasian stroke patients admitted to the Department of Neurology of the University Hospital of Dijon, France, were enrolled prospectively. Demographics and clinical information including stroke severity assessed using the National Institutes of Health Stroke Scale score were collected. The serum concentration of 25(OH)D was measured at baseline. Multivariable Cox regression models were used to evaluate the association between 1-year all-cause mortality and serum 25(OH)D levels treated as either a log-transformed continuous variable or dichotomized (<25.7 and ≥25.7 nmol/l) at the first tertile of their distribution. RESULTS: Of the 382 stroke patients included, 63 (16.5%) had died at 1 year. The mean 25(OH)D level was lower in these patients (32.3 ± 22.0 vs. 44.6 ± 28.7 nmol/l, p < 0.001), and survival at 1 year was worse in patients in the lowest tertile of 25(OH)D levels (defined as <25.7 nmol/l); log-transformed 25(OH)D levels were inversely associated with 1-year mortality (hazard ratio, HR = 0.62; 95% confidence interval, 95% CI: 0.44-0.87; p = 0.007), and patients with 25(OH)D levels <25.7 nmol/l were at a higher risk of death at 1 year (HR = 1.95; 95% CI: 1.14-3.32; p = 0.014). In multivariable analyses, the association was no longer significant but a significant interaction was found for age, and stratified analyses by age groups showed an inverse relationship between 25(OH)D levels and 1-year mortality in patients aged <75 years [HR = 0.38; 95% CI: 0.17-0.83; p = 0.015 for log-transformed 25(OH)D levels, and HR = 3.12; 95% CI: 0.98-9.93; p = 0.054 for 25(OH)D levels <25.7 vs. >25.7 nmol/l]. CONCLUSION: A low serum 25(OH)D level at stroke onset may be associated with higher mortality at 1 year in patients <75 years old. Further studies are needed to confirm these findings and to determine whether vitamin D supplementation could improve survival in stroke patients.

Thrombolytic therapy for stroke in patients with preexisting cognitive impairment.

OBJECTIVE: We aimed to evaluate the influence of prestroke cognitive impairment (PSCI) on outcomes in stroke patients treated with IV recombinant tissue plasminogen activator (rtPA). METHODS: 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 score >3. 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. RESULTS: Of 205 patients, 62 (30.2%) met criteria for PSCI. They were 11 years older (p < 0.001). 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. CONCLUSIONS: Ischemic stroke patients with PSCI should receive rtPA if they are eligible. This conclusion cannot be extended to severe cognitive impairment or severe strokes. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that in patients with PSCI presenting with acute ischemic stroke, IV rtPA improves outcomes.

Silent atrial fibrillation after ischemic stroke or transient ischemic attack: interest of continuous ECG monitoring.

BACKGROUND: Since atrial fibrillation (AF) may be undiagnosed when asymptomatic and paroxysmal, we aimed to investigate the incidence and determinants of silent AF in patients with acute ischemic stroke or transient ischemic attack (TIA). METHODS: Consecutive patients admitted to the Stroke Unit of the University Hospital of Dijon, France, for acute ischemic stroke or TIA were prospectively enrolled from March to December 2012. Silent AF was assessed by continuous electrocardiography (ECG) monitoring for 24 h after admission. An echocardiography was performed at day 2 ± 1 to measure left ventricular ejection fraction (LVEF) and left auricular (LA) dimensions. RESULTS: Among the 187 patients included, 19 (10%) developed silent AF. Patients with silent AF were markedly older (76 vs. 66 years, p < 0.002), with lower creatinine levels (90 vs. 80 µmol/l, p = 0.030) and were less often smokers (5 vs. 24%, p = 0.058) than patients without silent AF. They also showed a trend towards more frequent hypertension and a recent history of infection. Patients with silent AF had a larger indexed LA volume (37.4 vs. 30.8 ml/m(3), p = 0.057) and LA diameter (23.2 vs. 20.8 mm/m(2), p = 0.059). LVEF in the two groups was similar. In multivariate analysis, only age remained an independent estimate of silent AF. CONCLUSION: Silent AF detected by continuous ECG monitoring is common and closely associated with older age. Further studies are needed to investigate the interest of systematically screening for silent AF for secondary prevention after ischemic stroke/TIA.

Post-stroke cognitive impairment: high prevalence and determining factors in a cohort of mild stroke.

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 <26/30 or if the neuropsychological battery confirmed PSCI when the MMSE and MOCA were discordant. Multivariable logistic models were used to determine factors associated with 3-month PSCI. RESULTS: Among the 280 patients included, 220 were assessable at 3 months. The overall frequency of 3-month PSCI was 47.3%, whereas that of dementia was 7.7%. In multivariable analyses, 3-month PSCI was associated with age, low education level, a history of diabetes mellitus, acute confusion, silent infarcts, and functional handicap at discharge. MMSE and MOCA scores during hospitalization were associated with 3-month PSCI (OR = 0.63; 95% CI: 0.54-0.74; p < 0.0001 and OR = 0.67; 95% CI: 0.59-0.76; p < 0.0001, respectively). CONCLUSION: Our study underlines the high frequency of PSCI in a cohort of mild stroke. The early cognitive diagnosis of stroke patients could be useful by helping physicians to identify those at a high risk of developing PSCI.

Exogenous t-PA administration increases hippocampal mature BDNF levels. plasmin- or NMDA-dependent mechanism?

Brain-derived neurotrophic factor (BDNF) through TrkB activation is central for brain functioning. Since the demonstration that plasmin is able to process pro-BDNF to mature BDNF and that these two forms have opposite effects on neuronal survival and plasticity, a particular attention has been paid to the link between tissue plasminogen activator (tPA)/plasmin system and BDNF metabolism. However, t-PA via its action on different N-methyl-D-aspartate (NMDA) receptor subunits is also considered as a neuromodulator of glutamatergic transmission. In this context, the aim of our study was to investigate the effect of recombinant (r)t-PA administration on brain BDNF metabolism in rats. In the hippocampus, we found that rt-PA (10 mg/kg) administration induced a progressive increase in mature BDNF levels associated with TrkB activation. In order to delineate the mechanistic involved, plasmin activity was assessed and its inhibition was attempted using tranexamic acid (30 or 300 mg/kg, i.v.) while NMDA receptors were antagonized with MK801 (0.3 or 3 mg/kg, i.p.) in combination with rt-PA treatment. Our results showed that despite a rise in rt-PA activity, rt-PA administration failed to increase hippocampal plasmin activity suggesting that the plasminogen/plasmin system is not involved whereas MK801 abrogated the augmentation in mature BDNF levels observed after rt-PA administration. All together, our results show that rt-PA administration induces increase in hippocampal mature BDNF expression and suggests that rt-PA contributes to the control of brain BDNF synthesis through a plasmin-independent potentiation of NMDA receptors signaling.

A de novo nonsense PDGFB mutation causing idiopathic basal ganglia calcification with laryngeal dystonia.

Idiopathic basal ganglia calcification (IBGC) is characterized by brain calcification and a wide variety of neurologic and psychiatric symptoms. In families with autosomal dominant inheritance, three causative genes have been identified: SLC20A2, PDGFRB, and, very recently, PDGFB. Whereas in clinical practice sporadic presentation of IBGC is frequent, well-documented reports of true sporadic occurrence are rare. We report the case of a 20-year-old woman who presented laryngeal dystonia revealing IBGC. Her healthy parents' CT scans were both normal. We identified in the proband a new nonsense mutation in exon 4 of PDGFB, c.439C>T (p.Gln147*), which was absent from the parents' DNA. This mutation may result in a loss-of-function of PDGF-B, which has been shown to cause IBGC in humans and to disrupt the blood-brain barrier in mice, resulting in brain calcification. The c.439C>T mutation is located between two previously reported nonsense mutations, c.433C>T (p.Gln145*) and c.445C>T (p.Arg149*), on a region that could be a hot spot for de novo mutations. We present the first full demonstration of the de novo occurrence of an IBGC-causative mutation in a sporadic case.

Prevalence and prognostic value of headache on early mortality in acute stroke: the Dijon Stroke Registry.

OBJECTIVE: To evaluate the prevalence of headache at onset and its association with 1-month mortality in stroke patients. METHODS: All patients with stroke in Dijon, France (2006-2011), were prospectively identified using a population-based registry. Cox regression models were used to evaluate the association between headache and 30-day all-cause mortality. RESULTS: Among 1411 stroke patients, data about headache were obtained for 1391 (98.6%) of whom 1185 had an ischemic stroke (IS), 201 had an intracerebral hemorrhage (ICH) and five had a stroke of undetermined etiology. Headache was found in 253 (18.2%) patients and was more frequent in those with ICH than in those with IS (46.3% vs 13.5%, p < 0.001). Overall 30-day mortality was 11.7%, and was greater for patients with than those without headache (17.0% vs 10.5%, unadjusted HR 1.70; 95% CI 1.20-2.41, p = 0.003). In multivariable analysis, an association between headache and 30-day mortality was observed (HR 1.51; 95% CI 1.02-2.25, p = 0.042). In stratified analyses, headache was associated with 30-day mortality in ICH (HR 2.09; 95% CI 1.18-3.71, p = 0.011) but not in IS (HR 1.01; 95% CI 0.53-1.92, p = 0.97). CONCLUSION: Headache at stroke onset is associated with a higher risk of early mortality in patients with ICH.

Unusual features of Creutzfeldt-Jakob disease followed-up in a memory clinic.

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.

Trends in the incidence of ischaemic stroke in young adults between 1985 and 2011: the Dijon Stroke Registry.

BACKGROUND: 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 aged <55 years old had changed over the last three decades. METHODS: 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. RESULTS: Over the 27-year study period, 4506 patients were recorded (53% women, mean age 74.6±14.4, 10.1% aged <55 years). An increase in overall stroke incidence was noted, as was a rise in ischaemic stroke in individuals aged <55 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, p<0.001 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%). CONCLUSIONS: Multiple factors may account for the increased incidence of ischaemic stroke in people aged <55 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.

The impact of World and European Football Cups on stroke in the population of Dijon, France: a longitudinal study from 1986 to 2006.

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) = 1.3; 95% confidence interval [CI] = 1.0-1.6; P = .029) but not with that of preceding year (RR = 1.1; 95% CI = .9-1.3; P = .367). This was mostly explained by a 40% decrease in stroke numbers during European Championships, compared with the unexposed following periods (RR = 1.4; 95% CI = 1.0-1.9; P = .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 validate these findings.

Smoking status and severity of ischemic stroke. A population-based study.

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.

Psychiatric Presentation of Frontotemporal Dementia Associated with Inclusion Body Myopathy due to the VCP Mutation (R155H) in a French Family.

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.

Population-based validation of the iScore for predicting mortality and early functional outcome in ischemic stroke patients.

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.