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Patients with pre-stroke disability, defined as a modified Rankin Scale (mRS) ≥3, were excluded from most trials of endovascular thrombectomy (EVT) for acute stroke. We sought to evaluate the prognostic factors associated with favorable outcome in stroke patients with known disability undergoing EVT, and the impact of successful reperfusion.Consecutive acute stroke patients with pre-stroke disability, undergoing EVT, were retrospectively collected between 2016 to 2019 from a Canadian cohort and a multicenter French cohort (Endovascular Treatment in Ischemic Stroke registry-ETIS). Favorable outcome was defined as an mRS equal to pre-stroke mRS. Patients achieving successful reperfusion (defined as a modified Thrombolysis in Cerebral Infarction score of 2b/3) were compared with patients without successful reperfusion to determine if successful EVT was associated with better functional outcomes.Among 6220 patients treated with EVT, 280 (4.5%) patients with a pre-stroke mRS ≥3 were included. Sixty-one patients (21.8%) had a favorable outcome and 146 (52.1%) died at 3 months. Patients with successful reperfusion had a higher proportion of favorable 90-day mRS (27.6% versus 19.6%, p = 0.025) and a lower mortality (48.3% versus 69.6%, p = 0.008) than patients without successful reperfusion. After adjusting for baseline prognostic factors, successful reperfusion defined by TICI ≥2b was associated with favorable functional outcome (OR 3.16 CI95% [1.11-11.5]; p 0.048).In patients with pre-stroke disability, successful reperfusion is associated with a greater proportion of favorable outcome and lower mortality.

ObjectivesTo assess the diagnosis of somatic symptom disorder (SSD) in patients with unexplained neurological symptoms occurring after SARS-CoV-2 infection, also referred to as long COVID.DesignSingle-centre observational study.ParticipantsAdult patients experiencing unexplained long-lasting neurological symptoms after mild COVID. Of the 58 consecutive patients referred in our centre, 50 were included.InterventionPatients were contacted for a standardised psychometric evaluation by phone, followed by a self-survey.Main outcomePositive diagnosis of SSD according to the criteria of the Diagnostic and Statistical Manual of Mental Disorders-5 (DSM-5).ResultsAlthough the patients did not meet the DSM-5 criteria for a functional neurological symptom disorder specifically, SSD diagnosis based on DSM-5 criteria was positive in 32 (64%) patients. In the remaining 18 patients, SSD was considered possible given the high score on diagnostic scales. Physical examination were normal for all. Brain MRI showed unspecific minor white matter hyperintensities in 8/46 patients. Neuropsychological assessment showed exclusively mild impairment of attention in 14 out of 15 tested patients, in discrepancy with their major subjective complaint. Forty-five (90%) patients met criteria for Chronic Fatigue Syndrome. Seventeen (32%) patients were screened positive for mood-anxiety disorders, 19 (38%) had a history of prior SSD and 27 (54%) reported past trauma. Additional self-survey highlighted post-traumatic stress disorder in 12/43 (28%), high levels of alexithymia traits and perfectionism. Long-lasting symptoms had a major impact with a high rate of insomnia (29/43, 67%), psychiatric follow-up (28/50, 56%) and work or pay loss (25/50, 50%).ConclusionA majority of patients with unexplained long-lasting neurological symptoms after mild COVID met diagnostic criteria for SSD and may require specific management.Trial registration numberNCT04889313.

International audience; PURPOSE: Infectious encephalitis (IE) is a severe disease which requires intensive care unit (ICU) admission in up to 50% of cases. We aimed to describe characteristics, management and outcomes of IE patients who required ICU admission. MATERIALS AND METHODS: Ancillary study focusing on patients with ICU admission within the ENCEIF cohort, a French prospective observational multicentre study. The primary criteria for outcome was the functional status at hospital discharge, categorized using the Glasgow outcome scale (GOS). Logistic regression model was used to identify risk factors for poor outcome, defined as a GOS ≤ 3. RESULTS: We enrolled 198 ICU patients with IE. HSV was the primary cause (n = 72, 36% of all IE, 53% of IE with microbiological documentation). Fifty-two patients (26%) had poor outcome at hospital discharge, including 22 deaths (11%). Immunodeficiency, supratentorial focal signs on admission, lower cerebrospinal fluid (CSF) white cells count (2 days were independent predictors of poor outcome. CONCLUSION: HSV is the primary cause of IE requiring ICU admission. IE patients admitted in ICU have a poor prognosis with 11% of in-hospital mortality and 15% of severe disabilities in survivors at discharge.

International audience; OBJECTIVES: To provide an overview of the spectrum, characteristics and outcomes of neurologic manifestations associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. METHODS: We conducted a single-centre retrospective study during the French coronavirus disease 2019 (COVID-19) epidemic in March-April 2020. All COVID-19 patients with de novo neurologic manifestations were eligible. RESULTS: We included 222 COVID-19 patients with neurologic manifestations from 46 centres in France. Median (interquartile range, IQR) age was 65 (53-72) years and 136 patients (61.3%) were male. COVID-19 was severe or critical in 102 patients (45.2%). The most common neurologic diseases were COVID-19-associated encephalopathy (67/222, 30.2%), acute ischaemic cerebrovascular syndrome (57/222, 25.7%), encephalitis (21/222, 9.5%) and Guillain-Barré syndrome (15/222, 6.8%). Neurologic manifestations appeared after the first COVID-19 symptoms with a median (IQR) delay of 6 (3-8) days in COVID-19-associated encephalopathy, 7 (5-10) days in encephalitis, 12 (7-18) days in acute ischaemic cerebrovascular syndrome and 18 (15-28) days in Guillain-Barré syndrome. Brain imaging was performed in 192 patients (86.5%), including 157 magnetic resonance imaging (70.7%). Among patients with acute ischaemic cerebrovascular syndrome, 13 (22.8%) of 57 had multiterritory ischaemic strokes, with large vessel thrombosis in 16 (28.1%) of 57. Brain magnetic resonance imaging of encephalitis patients showed heterogeneous acute nonvascular lesions in 14 (66.7%) of 21. Cerebrospinal fluid of 97 patients (43.7%) was analysed, with pleocytosis found in 18 patients (18.6%) and a positive SARS-CoV-2 PCR result in two patients with encephalitis. The median (IQR) follow-up was 24 (17-34) days with a high short-term mortality rate (28/222, 12.6%). CONCLUSIONS: Clinical spectrum and outcomes of neurologic manifestations associated with SARS-CoV-2 infection were broad and heterogeneous, suggesting different underlying pathogenic processes.

Background and Purpose: In the settings of thrombectomy, the first-pass effect (FPE), defined by a complete recanalization after one pass with no rescue therapy, has been shown to be associated with an improved outcome. As this phenomenon has been predominantly described in anterior circulation strokes, we aimed to study the prevalence, outcomes, and predictors of FPE in patients with a basilar artery occlusion. Methods: From a prospective multicentric registry, we collected the data of all consecutive basilar artery occlusion patients who underwent thrombectomy and compared the outcomes of patients who achieved FPE and those who did not. We also compared FPE patients with those who achieved a complete recanalization with >1 pass. Finally, a multivariate analysis was performed to determine the predictors of FPE. Results: Data from 280 patients were analyzed in our study, including 84 of 280 patients (30%) with an atheromatous etiology. An FPE was achieved in 93 patients (33.2%), with a significantly higher proportion of good outcomes (modified Rankin Scale score 0-2 at 3 months) and lower mortality than non-FPE patients. An FPE was also associated with improved outcomes compared with patients who went on to have full recanalization with >1 pass. Contact aspiration as first-line strategy was a strong predictor of FPE, whereas baseline antiplatelets and atheromatous etiology were negative predictors. Conclusions: In our study, an FPE was achieved in approximately one-third of patients with a basilar artery occlusion and was associated with improved outcomes. More research is needed to improve devices and techniques to increase the incidence of FPE. Registration: URL: https://www.clinicaltrials.gov ; Unique identifier: NCT03776877.

Background: Management of extracranial internal carotid artery steno-occlusive lesion during endovascular therapy remains debated. Stent occlusion within 24 hours of endovascular therapy is a frequent event after acute carotid artery stenting, and we currently lack large population results. We investigated the incidence, predictors, and clinical impact of stent occlusion after acute carotid artery stenting in current clinical practice. Methods: Patients treated by endovascular therapy with acute carotid artery stenting between 2015 and 2019 in 5 large-volume endovascular-capable centers were retrospectively analyzed. Patients were separated in 2 groups according to the stent patency at 24 hours after carotid artery stenting. We compared baseline characteristics, treatment modalities, and clinical outcome depending on 24-hour stent patency. Primary end point was favorable outcome, defined as a modified Rankin Scale score 0–2 at 3 months. Results: A stent occlusion was observed in 47/225 patients (20.9%). Patients with stent patency had a lower baseline National Institutes of Health Stroke Scale (median [interquartile range]: 13 [7–17] versus 18 [12–21]) and had more often stroke of atherothrombotic origin (77.0% versus 53.2%). A higher stent patency rate was found for patients treated with P2Y 12 antagonists at the acute phase (odds ratio [OR]‚ 2.95 [95% CI‚ 1.10–7.91]; P =0.026) and treated with angioplasty (OR‚ 2.42 [95% CI‚ 1.24–4.67]; P =0.008). A better intracranial angiographic reperfusion was observed in patients with 24-hour stent patency compared with patients without stent patency (OR‚ 8.38 [95% CI‚ 3.07–22.78]; P P P =0.009). Conclusions: One out of 5 patients treated with carotid artery stenting during endovascular therapy presented a stent occlusion within 24 hours. This event was associated with worse functional outcome. Stroke etiology, P2Y 12 antagonist administration, quality of intracranial reperfusion, and angioplasty were associated with 24-hour stent patency.

Background and ObjectivesTo report the clinical, biological, and imaging features and clinical course of a French cohort of patients with glial fibrillary acidic protein (GFAP) autoantibodies.MethodsWe retrospectively included all patients who tested positive for GFAP antibodies in the CSF by immunohistochemistry and confirmed by cell-based assay using cells expressing human GFAPα since 2017 from 2 French referral centers.ResultsWe identified 46 patients with GFAP antibodies. Median age at onset was 43 years, and 65% were men. Infectious prodromal symptoms were found in 82%. Other autoimmune diseases were found in 22% of patients, and coexisting neural autoantibodies in 11%. Tumors were present in 24%, and T-cell dysfunction in 23%. The most frequent presentation was subacute meningoencephalitis (85%), with cerebellar dysfunction in 57% of cases. Other clinical presentations included myelitis (30%) and visual (35%) and peripheral nervous system involvement (24%). MRI showed perivascular radial enhancement in 32%, periventricular T2 hyperintensity in 41%, brainstem involvement in 31%, leptomeningeal enhancement in 26%, and reversible splenial lesions in 4 cases. A total of 33 of 40 patients had a monophasic course, associated with a good outcome at last follow-up (Rankin Score ≤2: 89%), despite a severe clinical presentation. Adult and pediatric features are similar. Thirty-two patients were treated with immunotherapy. A total of 11/22 patients showed negative conversion of GFAP antibodies.DiscussionGFAP autoimmunity is mainly associated with acute/subacute meningoencephalomyelitis with prodromal symptoms, for which tumors and T-cell dysfunction are frequent triggers. The majority of patients followed a monophasic course with a good outcome.

BACKGROUND: Multiple sclerosis (MS) is a chronic degenerative neurological disease with visual, sensitive, motor, or cognitive symptoms. Physical activities are recommended for people with MS (pwMS) who are at home instead of in a rehabilitation program, to help them to maintain their autonomy. Since a lack of motivation appears to be the principal barrier for pwMS to practice exercises, it is necessary to evaluate whether mobile health (mHealth) tools can offer a solution to stimulate their motivation. OBJECTIVE: This literature review analyzes papers to investigate to how a mobile application can motivate pwMS to practice physical activities and manage their fatigue. METHODS: We performed an automatic query from digital libraries and analyzed the studies. RESULTS: We selected seven articles that responded to our criteria. These studies tested mobile applications that used different strategies to motivate pwMS to practice physical exercises at home and manage their fatigue. A lack of motivation was the main obstacle, but depending on the type of exercises chosen, some application settings seemed to resolve this issue. CONCLUSIONS: Studies in this area are limited, although this literature review highlights the need for mHealth tools in which pwMS and their therapists contribute to their use and conception.

Introduction: Acute ischemic stroke (AIS) and thrombotic events (TEs) were reported in patients with COVID-19. Clinical outcome of AIS in the course of COVID-19 remains unknown. We compared early clinical outcome and mortality of COVID-positive (+) patients admitted for AIS with COVID-negative (−) ones. We hypothesized that COVID+ patients would have poorer clinical outcomes and present a higher rate of TEs and mortality compared with COVID− ones. Methods: In this multicentric observational retrospective study, we enrolled patients over 18 years old admitted for AIS in 3 stroke units of the Parisian region during lockdown from March 17, 2020, to May 2, 2020. COVID-19 status as well as demographic, clinical, biological, and imaging data was collected retrospectively from medical records. Poor outcome was defined as modified Rankin score (mRS) 3–6 (3–6) at discharge. We also compared TE frequency and mortality rate through a composite criterion in both groups. Results: Two hundred and sixteen patients were enrolled; mean age was 68 years old, and 63% were male. Forty patients were CO­VID+ (18.5%) and 176 were COVID−. Obesity was statistically more frequent in the COVID+ group (36 vs. 13% p < 0.01). The percentage of patients with mRS (3–6) at discharge was higher in the COVID+ group compared with the COVID− group (60 vs. 41%, p = 0.034). The main predictor of presenting a mRS (3–6) at discharge was high NIHSS score at admission (OR, CI 95%: 1.325, 1.22–1.43). Mortality rate was higher in the COVID+ group (12 vs. 3.4%, p = 0.033) as well as TE frequency (15 vs. 2.8%, p < 0.01). Conclusion: In this study, patients with AIS infected by SARS-CoV-2 showed a poorer early outcome than COVID− ones. However, when compared to other factors, COVID-19 was not a significant predictor of poor outcome. Vascular morbidity and mortality rates were significantly higher in the COVID+ group compared with the COVID− group.

Rehabilitation is essential to Multiple Sclerosis (MS) treatment. Its efficacy can be maintained by self-rehabilitation but its execution often remains unpredictable. A mobile application could help people with MS (pwMS) practice these self-rehabilitation exercises at home. We are reporting the results of a study aiming to guide the design of a mobile application that would motivate pwMS to do so. With this aim in mind, a study has been conducted among the rehabilitation staff who work with these people. The results are steering the design of the application towards an occupation-centric application, in addition with some playful elements in order to provoke interest and commitment.

Background and Purpose: Approximately half of the patients with acute ischemic stroke due to anterior circulation large vessel occlusion do not achieve functional independence despite successful reperfusion. We aimed to determine influence of reperfusion strategy (bridging therapy, intravenous thrombolysis alone, or mechanical thrombectomy alone) on clinical outcomes in this population. Methods: From ongoing, prospective, multicenter, observational Endovascular Treatment in Ischemic Stroke registry in France, all patients with anterior circulation large vessel occlusion who achieved successful reperfusion (modified Thrombolysis in Cerebral Infarction 2b-3) following reperfusion therapy were included. Primary end point was favorable outcome, defined as 90-day modified Rankin Scale score ≤2. Patient groups were compared using those treated with bridging therapy as reference. Differences in baseline characteristics were reduced after propensity score-matching, with a maximum absolute standardized difference of 14% for occlusion site. Results: Among 1872 patients included, 970 (51.8%) received bridging therapy, 128 (6.8%) received intravenous thrombolysis alone, and the remaining 774 (41.4%) received MT alone. The rate of favorable outcome was comparable between groups. Excellent outcome (90-day modified Rankin Scale score 0–1) was achieved more frequently in the bridging therapy group compared with the MT alone (odds ratio after propensity score-matching, 0.70 [95% CI, 0.50–0.96]). Regarding safety outcomes, hemorrhagic complications were similar between the groups, but 90-day mortality was significantly higher in the MT alone group compared with the bridging therapy group (odds ratio, 1.60 [95% CI, 1.09–2.37]). Conclusions: This real-world observational study of patients with anterior circulation large vessel occlusion demonstrated a similar rate of favorable outcome following successful reperfusion with different therapeutic strategies. However, our results suggest that bridging therapy compared with MT alone is significantly associated with excellent clinical outcome and lower mortality. REGISTRATION: URL: https://www.clinicaltrials.gov ; Unique identifier: NCT03776877.

Background and Purpose: Ischemic stroke has been reported in various conditions associated with eosinophilia. FIP1L1-PDGFRA fusion ([Fip1-like 1-platelet-derived growth factor receptor alpha]; F/P ) leads to the proliferation of the eosinophilic lineage and thus to a clonal hypereosinophilic syndrome that is highly responsive to imatinib. Methods: We previously reported on a nationwide retrospective study of 151 patients with F/P -associated clonal hypereosinophilic syndrome. Patients from this cohort with a clinical history of ischemic stroke (as well as 2 additional cases) were further analyzed to better define their clinical picture and outcomes. Results: Sixteen male patients (median age, 51 [43–59] years) with low-to-intermediate cardiovascular risk were included. Median National Institutes of Health Stroke Scale was 4 (range, 1–6). Most cerebral imaging disclosed multiple bilateral infarctions of watershed distribution (69%). Despite frequent cardiac involvement (50%), cardiac thrombus was evidenced in a single patient and, according to the TOAST classification (Trial of ORG 10172 in Acute Stroke Treatment), 62.5% of strokes were presumably of undetermined etiology. Among the 15 patients treated with imatinib, and after a median follow-up of 4.5 years, stroke recurred in only 3 patients (consisting of either cardio embolic or hemorrhagic events, unrelated to the first episode). Conclusions: F/P + clonal hypereosinophilic syndrome is a diagnosis to consider in patients with unexplained ischemic stroke and hypereosinophilia (especially in the setting of multiple cortical borderzone distribution) and warrants prompt initiation of imatinib.

To investigate the prevalence of neuro-functional disability and its determinants 12 months after community-acquired bacterial meningitis (CABM) in adult patients.In a prospective multicenter cohort study (COMBAT), all consecutive cases of CABM were enrolled and followed up for 12 months. Neuro-functional disability at 12 months was evaluated using a combination of the Glasgow Outcome Scale (functional disability), and the modified Rankin Disability Scale (physical disability). Factors associated with neuro-functional disability were identified by multivariate logistic regression.Among 281 patients, 84 (29.9%) patients exhibited neuro-functional disability at 12 months: 79 (28.1%) with functional disability and 51 (18.1%) with physical disability. Overall, 6 patients (2.1%) died during the follow-up. The most common pathogen identified was Streptococcus pneumoniae (131/272, 48.2%); 77/268 patients (28.7%) had a physical disability at hospital discharge. Factors independently associated with 12-month neuro-functional disability were a pneumococcal meningitis (adjusted OR = 2.8; 95% confidence interval (CI) = [1.3; 6.7]), the presence of a physical disability at hospital discharge (aOR = 2.3; 95%CI = [1.2; 4.4]) and the presence of behavioral disorders at hospital-discharge (aOR = 5.9; 95%CI = [1.6; 28.4]). Dexamethasone use was not significantly associated with neuro-functional disability (OR = 0.2; 95%CI = [ 0.1;1.3]).Neuro-functional disability is frequently reported 12 months after CABM. Detailed neurological examination at discharge is needed to improve the follow-up.NCT01730690.

Background and Purpose: The influence of prior antiplatelet therapy (APT) uses on the outcomes of patients with acute ischemic stroke treated with endovascular therapy is unclear. We compared procedural and clinical outcomes of endovascular therapy in patients on APT or not before stroke onset. Methods: We analyzed 2 groups from the ongoing prospective multicenter Endovascular Treatment in Ischemic Stroke registry in France: patients on prior APT (APT+) and patients without prior APT (APT−) treated by endovascular therapy, with and without intravenous thrombolysis. Multilevel mixed-effects logistic models including center as random effect were used to compare angiographic (rates of reperfusion at the end of procedure, procedural complications) and clinical (favorable and excellent outcome, 90-day all-cause mortality, and hemorrhagic complications) outcomes according to APT subgroups. Comparisons were adjusted for prespecified confounders (age, admission National Institutes of Health Stroke Scale score, Alberta Stroke Program Early CT Score, intravenous thrombolysis, and time from onset to puncture), as well as for meaningful baseline between-group differences. Results: A total of 2939 patients were analyzed, of whom 877 (29.8%) were on prior APT. Patients with prior APT were older, had more frequent vascular risk factors, cardioembolic stroke mechanism, and prestroke disability. Rates of complete reperfusion (37.9% in the APT− group versus 42.7 % in the APT+ group; aOR, 1.09 [95% CI, 0.88–1.34]; P =0.41) and periprocedural complication (16.9% versus 13.3%; aOR, 0.90 [95% CI, 0.7–1.2]; P =0.66) did not differ between the two groups. Symptomatic intracerebral hemorrhage (aOR, 0.93 [95% CI, 0.63–1.37]; P =0.73), 3 months favorable clinical outcome (modified Rankin Scale score of 0–2; aOR, 0.98 [95% CI, 0.77–1.25]; P =0.89), and mortality (aOR, 0.95 [95% CI, 0.72–1.26]; P =0.76) at 90 days did not differ between the groups. Conclusions: Prior APT does not influence angiographic and functional outcomes following endovascular therapy and should not be taken into account for acute revascularization strategies.

Background New diagnostic tools have been developed to improve the diagnosis of infectious encephalitis. Using a prospective cohort of encephalitis patients, our objective was to identify possible clusters of patients with similar patterns among encephalitis of unknown cause (EUC) and to describe to what extent a patient’s initial presentation may be predictive of encephalitis etiology, particularly herpes simplex virus (HSV) and varicella-zoster virus (VZV). Methods The National Cohort of Infectious Encephalitis in France is an ongoing prospective cohort study implemented in France in 2016. Patients who present with documented or suspected acute infectious encephalitis were included. Focusing on the variables that describe the initial presentation, we performed a factor analysis of mixed data (FAMD) to investigate a pattern of association between the initial presentation of a patient and the etiologic pathogen. Results As of 1 August 2018, data from 349 patients were analyzed. The most frequent pathogens were HSV (25%), VZV (11%), tick-borne encephalitis virus (6%), Listeria (5%), influenza virus (3%), and EUC (34%). Using the FAMD, it was not possible to identify a specific pattern related to the group of EUC. Age, temporal or hemorrhagic lesions, and cerebral spinal fluid lymphocytosis were significantly associated with HSV/VZV encephalitis. Conclusions No initial clinical/imaging/biology pattern was identified at admission among EUC, despite the improvement in diagnostic tools. In this context, the recommendation for a universal, early, probabilistic, initial treatment against HSV and VZV is still relevant, regardless of the initial clinical presentation of the encephalitis.

International audience; No abstract available

To evaluate outcomes of thrombectomy in patients with a basilar artery occlusion (BAO) and mild symptoms, defined by an initial National Institutes of Health Stroke Scale (NIHSS) score ≤6.We performed a retrospective analysis of a multicenter prospective cohort of consecutive patients with acute ischemic stroke with BAO who underwent thrombectomy. We compared baseline and procedural characteristics, as well as outcomes between patients with BAO with an NIHSS score ≤6 and6. Multivariate analyses were performed to determine baseline and procedural predictors of good outcome (modified Rankin Scale score 0-2) among patients with an NIHSS score ≤6.A total of 269 patients were included: 50 (19%) had an initial NIHSS score ≤6 and 219 (81%) had an NIHSS score6. Patients with mild strokes (NIHSS score ≤6) had better outcomes (68% of modified Rankin Scale score 0-2 vs. 27% for NIHSS score6; P0.0001), lower mortality (14% vs. 48; P0.0001) and fewer parenchymal hematomas at day 1 (0% vs. 10%; P = 0.016). A multivariate analysis identified the following predictors for good outcome among patients with BAO with an NIHSS score ≤6: younger age, fewer passes, a cardioembolic cause, and the absence of need for angioplasty/stenting.Thrombectomy seems to be safer and more effective for mild BAO strokes with NIHSS score ≤6 than for more severe patients. Even although thrombectomy showed high rates of recanalization, a substantial proportion (32%) nevertheless had a poor long-term clinical outcome. The number of passes, patient's age, and stroke cause seem to be predictors of clinical outcome.

Limbic encephalitis with antibodies against adenylate kinase 5 (AK5) has been difficult to characterize because of its rarity. In this study, we identified 10 new cases and reviewed 16 previously reported patients, investigating clinical features, IgG subclasses, human leucocyte antigen and CSF proteomic profiles. Patients with anti-AK5 limbic encephalitis were mostly male (20/26, 76.9%) with a median age of 66 years (range 48–94). The predominant symptom was severe episodic amnesia in all patients, and this was frequently associated with depression (17/25, 68.0%). Weight loss, asthenia and anorexia were also highly characteristic, being present in 11/25 (44.0%) patients. Although epilepsy was always lacking at disease onset, seizures developed later in a subset of patients (4/25, 16.0%). All patients presented CSF abnormalities, such as pleocytosis (18/25, 72.0%), oligoclonal bands (18/25, 72.0%) and increased Tau (11/14, 78.6%). Temporal lobe hyperintensities were almost always present at disease onset (23/26, 88.5%), evolving nearly invariably towards severe atrophy in subsequent MRIs (17/19, 89.5%). This finding was in line with a poor response to immunotherapy, with only 5/25 (20.0%) patients responding. IgG1 was the predominant subclass, being the most frequently detected and the one with the highest titres in nine CSF-serum paired samples. A temporal biopsy from one of our new cases showed massive lymphocytic infiltrates dominated by both CD4+ and CT8+ T cells, intense granzyme B expression and abundant macrophages/microglia. Human leucocyte antigen (HLA) analysis in 11 patients showed a striking association with HLA-B*08:01 [7/11, 63.6%; odds ratio (OR) = 13.4, 95% confidence interval (CI): 3.8–47.4], C*07:01 (8/11, 72.7%; OR = 11.0, 95% CI: 2.9–42.5), DRB1*03:01 (8/11, 72.7%; OR = 14.4, 95% CI: 3.7–55.7), DQB1*02:01 (8/11, 72.7%; OR = 13.5, 95% CI: 3.5–52.0) and DQA1*05:01 (8/11, 72.7%; OR = 14.4, 95% CI: 3.7–55.7) alleles, which formed the extended haplotype B8-C7-DR3-DQ2 in 6/11 (54.5%) patients (OR = 16.5, 95% CI: 4.8–57.1). Finally, we compared the CSF proteomic profile of five anti-AK5 patients with that of 40 control subjects and 10 cases with other more common non-paraneoplastic limbic encephalitis (five with antibodies against leucine-rich glioma inactivated 1 and five against contactin-associated protein-like 2), as well as 10 cases with paraneoplastic neurological syndromes (five with antibodies against Yo and five against Ma2). These comparisons revealed 31 and seven significantly upregulated proteins in anti-AK5 limbic encephalitis, respectively mapping to apoptosis pathways and innate/adaptive immune responses. These findings suggest that the clinical manifestations of anti-AK5 limbic encephalitis result from a distinct T cell-mediated pathogenesis, with major cytotoxicity-induced apoptosis leading to a prompt and aggressive neuronal loss, likely explaining the poor prognosis and response to immunotherapy.

International audience; Objectives: The aim of this study was to determine whether individual operator characteristics have an impact on reperfusion and procedural complication rates.Background: Mechanical thrombectomy (MT) is a Level IA treatment in acute ischemic stroke (AIS) patients. The operator's effect has been found to be an independent predictor for clinical outcome and technical performance in interventional cardiology.Methods: From the ETIS (Endovascular Treatment in Ischemic Stroke) study, a prospective, multicenter, observational real-world MT registry, the authors included all AIS patients consecutively treated by MT between January 2012 and March 2017 in 3 high-volume comprehensive stroke centers by 19 operators. We assessed the effect of individual operator characteristics on successful reperfusion, defined as modified Thrombolysis In Cerebral Infarction 2b/3 at the end of MT, and procedural complications using multivariable hierarchical logistic regression models.Results: A total of 1,541 patients with anterior and posterior AIS were enrolled (mean age 67 years; median NIHSS 16). There was a significant operator effect on successful reperfusion, with an intraclass correlation coefficient of 0.036 (p = 0.046), but not on complications (intraclass correlation coefficient = 0). There was a dose-response relationship between annual operator volume and successful reperfusion rate (p = 0.003) with an adjusted odds ratio for successful reperfusion equal to 2.52 (95% confidence interval: 1.37 to 4.64) for patients treated by an operator with an annual volume ≥40 MT/year compared with those treated by an operator with

ObjectiveTo study blood anti-Aβ antibodies in the context of spontaneous inflammatory or hemorrhagic CAA manifestations, which are similar to complications occurring after monoclonal anti-Aβ antibody immunotherapies.MethodsIn this case-control study, serum anti-Aβ antibody isotype, concentration, avidity, and reactivity toward soluble or fibrillary Aβ1-40 and Aβ1-42 isoforms were assessed using an ELISA-based multiplex analysis. Anti-Aβ serologic patterns were defined in CAA and CAA subgroups using multivariable logistic regression analyses.ResultsFourty-one healthy aged controls and 64 CAA patients were recruited: 46 with hemorrhagic features (CAA-he) and 18 with CAA-related inflammation (CAA-ri). As compared to controls, the most striking features of CAA-related serological profiles were the following: i) both CAA-he and CAA-ri patients displayed lower binding diversity of anti-soluble Aβ1-40 IgM; ii) CAA-he patients displayed higher anti-soluble Aβ1-40 / fibrillary Aβ1-42 IgG4 concentrations ratio and higher anti-soluble Aβ1-42 IgG4 and IgA avidity; iii) CAA-ri patients displayed higher binding diversity of anti-soluble Aβ1-40 IgG3 and higher anti-fibrillary/soluble Aβ1-42 IgG4 dilution curve steepness ratio.ConclusionThis proof-of-concept study revealed anti-Aβ antibody variations in CAA patients, some of which were associated to CAA clinical phenotypes, unveiling pathophysiological insights regarding CAA-hemorrhagic and inflammatory related events.

Background and Purpose: Although the efficacy of endovascular treatment (EVT) in patients with anterior circulation ischemic stroke (AIS) is well documented, early neurological deterioration after EVT remains a serious issue associated with poor outcome. Besides obvious causes, such as lack of reperfusion, procedural complications, or parenchymal hemorrhage, early neurological deterioration may remain unexplained (UnEND). Our aim was to investigate predictors of UnEND after EVT in patients with AIS. Methods: Patients who underwent EVT for AIS, with an initial National Institutes of Health Stroke Scale score >5, Alberta Stroke Program Early CT Score ≥6, and included in a multicenter prospective observational registry were analyzed. Predictors of UnEND, defined as ≥4-point increase in the National Institutes of Health Stroke Scale score between baseline and day 1 after EVT, were determined via center-adjusted analyses. Results: Among the 1925 included in the analysis, 128 UnEND (6.6%) were recorded. In multivariate analysis, predictors of UnEND were diabetes mellitus (odds ratio [OR], 2.17 [95% CI, 1.32–3.56]), prestroke modified Rankin Scale score ≥2 (OR, 2.22 [95% CI, 1.09–4.55]), general anesthesia (OR, 2.55 [95% CI, 1.51–4.30]), admission systolic blood pressure (OR, 1.10 [95% CI, 1.01–1.20]), age (OR, 1.38 [95% CI, 1.14–1.67]), number of passes (OR, 1.16 [95% CI, 1.04–1.28]), direct admission or not to a comprehensive stroke center (OR, 0.49 [95% CI, 0.30–0.81]), and initial National Institutes of Health Stroke Scale score (OR, 0.65 [95% CI, 0.52–0.81]). Conclusions: Severely impaired AIS patients with nonmodifiable factors are more likely to develop UnEND. Some modifiable predictors of UnEND such as the number of EVT passes could be the object of improvement in AIS management.

BACKGROUND AND PURPOSE: Ischaemic stroke has been described in association with COVID-19. Several pathophysiological mechanisms have been suggested, i.e. prothrombotic state, cardiac injury etc. It was sought to assess the potential association between ischaemic stroke associated with SARS-CoV-2 infection and underlying atherosclerotic lesions. METHODS: A retrospective analysis of stroke related to large vessel occlusion was conducted amongst patients with SARS-CoV-2 infection and underlying mild atherosclerotic disease, between 19 March and 19 April 2020 in six different stroke centers in the Ile-de France area, France. RESULTS: The median age was 52 years, median body mass index was 29.5 kg/m2 . All patients displayed previous vascular risk factors such as high blood pressure, diabetes, dyslipidemia or body mass index > 25. The delay between the first respiratory symptoms of COVID-19 and stroke was 11.5 days. At baseline, all had tandem occlusions, i.e. intracerebral and extracerebral thrombus assessed with computed tomography or magnetic resonance imaging. Cases displayed a large thrombus in the cervical carotid artery with underlying mild non-stenosing atheroma, after an etiological workup based on angio-computed tomography or magnetic resonance imaging and/or cervical echography. CONCLUSION: Our study should alert clinicians to scrutinize any new onset of ischaemic stroke during COVID-19 infection, mainly in patients with vascular risk factors or underlying atherosclerotic lesions.

Background and Purpose: The efficiency of prehospital care chain response and the adequacy of hospital resources are challenged amid the coronavirus disease 2019 (COVID-19) outbreak, with suspected consequences for patients with ischemic stroke eligible for mechanical thrombectomy (MT). Methods: We conducted a prospective national-level data collection of patients treated with MT, ranging 45 days across epidemic containment measures instatement, and of patients treated during the same calendar period in 2019. The primary end point was the variation of patients receiving MT during the epidemic period. Secondary end points included care delays between onset, imaging, and groin puncture. To analyze the primary end point, we used a Poisson regression model. We then analyzed the correlation between the number of MTs and the number of COVID-19 cases hospitalizations, using the Pearson correlation coefficient (compared with the null value). Results: A total of 1513 patients were included at 32 centers, in all French administrative regions. There was a 21% significant decrease (0.79; [95%CI, 0.76–0.82]; P P P R 2 −0.51; P =0.04). Patients treated during the COVID outbreak were less likely to receive intravenous thrombolysis and to have unwitnessed strokes (both P Conclusions: Our study showed a significant decrease in patients treated with MTs during the first stages of the COVID epidemic in France and alarming indicators of lengthened care delays. These findings prompt immediate consideration of local and regional stroke networks preparedness in the varying contexts of COVID-19 pandemic evolution.

Background and Purpose: The best anesthetic management for mechanical thrombectomy of large vessel occlusion strokes is still uncertain and could impact the quality of reperfusion and clinical outcome. We aimed to compare the efficacy and safety outcomes between local anesthesia (LA) and conscious sedation in a large cohort of acute ischemic stroke patients with anterior circulation large vessel occlusion strokes treated with mechanical thrombectomy in current, everyday clinical practice. Methods: Patients undergoing mechanical thrombectomy for anterior large vessel occlusion strokes at 4 comprehensive stroke centers in France between January 1, 2018, and December 31, 2018, were pooled from the ongoing prospective multicenter observational Endovascular Treatment in Ischemic Stroke Registry in France. Intention-to-treat and per-protocol analyses were used. Results: Among the included 1034 patients, 762 were included in the conscious sedation group and 272 were included in the LA group. In the propensity score matched cohort, the rate of favorable outcome (90-day modified Rankin Scale score 0–2) was significantly lower in the LA group than in the conscious sedation group (40.0% versus 52.0%, matched relative risk=0.76 [95% CI, 0.60–0.97]), as well as the rate of successful reperfusion (modified Thrombolysis in Cerebral Infarction grade 2b–3; 76.6% versus 87.1%; matched relative risk=0.88 [95% CI, 0.79–0.98]). There was no difference in procedure time between the 2 groups. In the inverse probability of treatment weighting-propensity score-adjusted cohort, similar significant differences were found for favorable outcomes and successful reperfusion. In inverse probability of treatment weighting-propensity score-adjusted cohort, a higher rate of 90-day mortality and a lower parenchymal hematoma were observed after LA. The sensitivity analysis restricted to our per-protocol sample provided similar results in the matched- and inverse probability of treatment weighting-propensity cohorts. Conclusions: In the Endovascular Treatment in Ischemic Stroke registry mainly included patients in early time window (

Myalgia or muscle weakness is frequently observed during HIV infection (1), and 15% of HIV patients may have an increased serum creatine kinase (CK) level.1 Case reports or case series have suggested that HIV-associated myopathies represent a large spectrum of disease.2 To date, data on muscle involvement during HIV infection remain sparse and we aimed to describe the clinical characteristics and evolution of biopsy-proven HIV-associated myopathies. All HIV-positive patients who had a muscle biopsy performed at the Pitie-Salpetriere University Hospital between 2001 and 2012 were identified. Medical records were retrospectively reviewed to assess muscle disease features and HIV infection characteristics. Patients were classified histologically as polymyositis (PM), non-specific myositis (NSM), immune-mediated necrotizing myopathy (IMNM), or inclusion body myositis (IBM) according to the European Neuromuscular Center (ENMC) criteria.3 4 Isolated mitochondrial abnormality (IMA) was defined as presence of cytochrome c oxidase negative fibres and/or succinate dehydrogenase positive staining without inflammatory cell infiltrate or rimmed vacuoles. PM or NSM evolution towards IBM was evaluated at follow-up. Uncontrolled viral load was defined as >40 copies/mL. Categorical variables are reported herein as numbers and/or percentages and were compared using a χ2 or Fisher’s exact test. Quantitative variables are reported as median (IQR1–IQR3) and compared using non-parametric one-way one-way analysis of variance (ANOVA). For all statistical analyses, p

Background and Purpose— Proximal large vessel occlusion (LVO) is present in up to 30% of minor strokes. The effectiveness of mechanical thrombectomy (MT) in the subgroup of minor stroke with LVO in the anterior circulation is still open to debate. Data about MT in this subgroup of patients are sparse, and their optimal management has not yet been defined. The purpose of this multicenter cohort study was to evaluate the effectiveness of MT in patients experiencing acute ischemic stroke (AIS) because of LVO in the anterior circulation, presenting with minor-to-mild stroke symptoms (National Institutes of Health Stroke Scale score of Methods— Multicenter cohort study involving 4 comprehensive stroke centers having 2 therapeutic approaches (urgent thrombectomy associated with best medical treatment [BMT] versus BMT first and MT if worsening occurs) about management of patients with minor and mild acute ischemic stroke harboring LVO in the anterior circulation. An intention-to-treat analysis was conducted. The primary end point was the rate of excellent outcome defined as the achievement of a modified Rankin Scale score of 0 to 1 at 3 months. Results— Three hundred one patients were included, 170 with urgent MT associated with BMT, and 131 with BMT alone as first-line treatment. Patients treated with MT were younger, more often received intravenous thrombolysis, and had shorter time to imaging. Twenty-four patients (18.0%) in the medical group had rescue MT because of neurological worsening. Overall, excellent outcome was achieved in 64.5% of patients, with no difference between the 2 groups. Stratified analysis according to key subgroups did not find heterogeneity in the treatment effect size. Conclusions— Minor-to-mild stroke patients with LVO achieved excellent and favorable functional outcomes at 3 months in similar proportions between urgent MT versus delayed MT associated with BMT. There is thus an urgent need for randomized trials to define the effectiveness of MT in this patient subgroup.

Background and Purpose— In acute ischemic stroke patients, diffusion-weighted imaging (DWI)–Alberta Stroke Program Early Computed Tomography Score (ASPECTS) is correlated with infarct volume and is an independent factor of functional outcome. Patients with pretreatment DWI-ASPECTS ≤6 were excluded or under-represented in the recent randomized mechanical thrombectomy trials. Our aim was to assess the impact of reperfusion in pretreatment DWI-ASPECTS ≤6 patients treated with mechanical thrombectomy. Methods— We analyzed data collected between January 2012 and August 2015 in a bicentric prospective clinical registry of consecutive acute ischemic stroke patients treated with mechanical thrombectomy. Every patient with a documented internal carotid artery or middle cerebral artery occlusion with pretreatment DWI-ASPECTS ≤6 was eligible for this study. The primary end point was a favorable outcome defined by a modified Rankin Scale score ≤2 at 90 days. Results— Two hundred and eighteen patients with a DWI-ASPECTS ≤6 were included. Among them, 145 (66%) patients had successful reperfusion at the end of mechanical thrombectomy. Reperfused patients had an increased rate of favorable outcome (38.7% versus 17.4%; P =0.002) and a decreased rate of mortality at 3 months (22.5% versus 39.1%; P =0.013) compared with nonreperfused patients. The symptomatic intracranial hemorrhage rate was not different between the 2 groups (13.0% versus 14.1%; P =0.83). However, in patients with DWI-ASPECTS P =0.68) with a high mortality rate (45.7% versus 57.1%; P =0.38) with or without successful reperfusion. Conclusions— Successful reperfusion is associated with reduced mortality and disability in patients with a pretreatment DWI-ASPECTS ≤6. Further data from randomized studies are needed, particularly in patients with DWI-ASPECTS

Background Fever is a deceptive feature of autoimmune disorders. Although cases of MOG-IgG associated disorders (MOGAD) were rarely associated with fever, this association was not specifically described. Methods We report a case of MOGAD revealed by weeks of fever and meningitis. We reviewed the current literature to describe the association between fever and MOGAD. Results We analyzed 146 cases from the literature including ours. Fever was associated with 39% of MOGAD attacks and lasted more than a week in 74%. Fever was strongly associated with brain and spinal cord attacks, and with meningitis. Conclusion Among the various features of MOGAD, fever is a highly prevalent associated symptom that should be kept in mind.

Our purpose was to summarize the current knowledge about the neurological presentation of Zika virus infection after the perinatal period. Other Flaviviruses infections, such as West Nile virus (WNV) or Japanese encephalitis virus (JEV), can result in neuro-invasive disease such as myelitis, encephalitis, or meningitis. We aimed at describing the specificities of ZV neurological infection. The recent outbreaks demonstrated clearly the neurotropism of ZV. However, by contrast with other Flaviviruses, the most frequent neurological presentation of ZV infection beyond the perinatal period was Guillain-Barre syndrome, especially the demyelination form of GBS. Encephalitis and myelitis seem to occur less frequently after ZV infection than after WNV or JEV infection. The pathophysiology of neurological ZV infections is still poorly understood and no specific treatment is available. Moreover, no data is available about long-term persisting symptoms and possible impairment of patients after the acute clinical episode.

Introduction La sclerose en plaques est une maladie rare et plus severe chez les sujets originaires d’Afrique sub-saharienne. Objectifs Decrire la sclerose en plaques chez les chez les sujets originaires d’Afrique sub-saharienne. Patients et methodes Etude descriptive retrospective de tous les patients presentant une SEP originaires d’Afrique sub-saharienne, dans les hopitaux generaux d’Ile de France. Resultats Trente sujets identifies. Sur les six premiers, predominance feminine (4F/2H), âge moyen de debut de 24,5 ans, 3 ont passe toute leur enfance et adolescence en France, et les 3 autres ont emigre en France apres 16 ans, atteinte optique chez 3 d’entre eux dont 2 NORB. Au moins une poussee par an. EDSS > 3,0 chez 80 % des sujets. Plusieurs lesions confluentes a l’IRM. Forme RR majoritaire. Recours aux traitements de seconde voire de troisieme ligne. Discussion Chez les sujets de race noire, la SEP debute a un âge beaucoup plus tardif, le risque de la developper depend du lieu de naissance et de l’âge d’arrivee en France, la NORB est le mode de debut preferentiel, grave d’emblee avec une evolution severe, plus difficile a equilibrer sur le plan therapeutique. L’evolution a court et a moyen terme est favorable, mais incertaine a long terme. Conclusion Les patients presentant une SEP originaires d’Afrique sub-saharienne ont des caracteristiques relativement proches des patients d’origine caucasienne ayant une sclerose en plaques. Les donnees des 30 patients seront presentees lors du congres.

Introduction Les parkinsoniens fluctuants beneficient de la stimulation cerebrale et de la levodopa en gel intestinal, surtout utilisees en centres experts et CHU, et de l’Apomorphine en perfusion sous cutanee, plus largement diffusee, notamment en CHG. Objectifs Cette enquete a evalue les pratiques en matiere de prise en charge therapeutique des patients parkinsoniens fluctuants au sein des CHG, plus particulierement l’offre de soins en matiere de perfusion sous cutanee d’Apomorphine. Patients et methodes Un questionnaire a ete adresse a 106 chefs de service et responsables de structure de Neurologie de CHG, et 38 reponses analysees, provenant de l’ensemble des regions et illustrant un large panel allant de petites structures au centre expert. Il comportait une premiere partie demographique sur la patientele et les ressources neurologiques, et une seconde sur l’utilisation de l’Apomorphine. Resultats Une unite “Parkinson” existe dans 16 % des CHG et une consultation dediee 1 fois sur 4. Plus de la moitie suivent plus de 200 Parkinsoniens et 21 % plus de 200 patients fluctuants, a 90 km en moyenne du CHU. Plus de 40 % ne proposent jamais la levodopa en gel intestinal, alors que tous proposent l’Apomorphine. Les ¾ suivent plus de 5 patients sous pompe et 1/5 plus de 20. Plus de 40 % posent eux-memes les pompes (53 % plus de 5/an). Le manque de formation demeure la principale limitation a l’utilisation de la technique. Discussion Seuls 3 a 400 patients sont equipes chaque annee d’une stimulation cerebrale, et la levodopa en gel intestinal reste peu utilisee. Les patients sont souvent âges, lourdement handicapes, et leurs deplacements compliques. L’enquete montre que de nombreux CHG utilisent deja la pompe d’Apomorphine, mais qu’il existe un important besoin de formation a la technique, pour permettre au plus grand nombre de patients un suivi proche du domicile. Conclusion La pompe d’Apomorphine represente la technique la plus repandue pour traiter les parkinsoniens fluctuants. Son utilisation plus large encore en CHG, au plus proche du patient, representera une nette amelioration du parcours de soin de ce patient fragile.

Whipple disease (WD) is a rare multisystemic infection with a protean clinical presentation. The central nervous system (CNS) is involved in 3 situations: CNS involvement in classic WD, CNS relapse in previously treated WD, and isolated CNS infection. We retrospectively analyzed clinical features, diagnostic workup, brain imaging, cerebrospinal fluid (CSF) study, treatment, and follow-up data in 18 patients with WD and CNS infection. Ten men and 8 women were included with a median age at diagnosis of 47 years (range, 30–56 yr). The median follow-up duration was 6 years (range, 1–19 yr). As categorized in the 3 subgroups, 11 patients had classic WD with CNS involvement, 4 had an isolated CNS infection, and 3 had a neurologic relapse of previously treated WD. CNS involvement occurred during prolonged trimethoprim-sulfamethoxazole (TMP-SMX) treatment in 1 patient with classic WD. The neurologic symptoms were various and always intermingled, as follows: confusion or coma (17%) related to meningo-encephalitis or status epilepticus; delirium (17%); cognitive impairment (61%) including memory loss and attention defects or typical frontal lobe syndrome; hypersomnia (17%); abnormal movements (myoclonus, choreiform movements, oculomasticatory myorhythmia) (39%); cerebellar ataxia (11%); upper motor neuron (44%) or extrapyramidal symptoms (33%); and ophthalmoplegia (17%) in conjunction or not with progressive supranuclear palsy. No specific pattern was correlated with any subgroup. Brain magnetic resonance imaging (MRI) revealed a unique focal lesion (35%), mostly as a tumorlike brain lesion, or multifocal lesions (23%) involving the medial temporal lobe, midbrain, hypothalamus, and thalamus. Periventricular diffuse leukopathy (6%), diffuse cortical atrophy (18%), and pachymeningitis (12%) were observed. The spinal cord was involved in 2 cases. MRI showed ischemic sequelae at diagnosis or during follow-up in 4 patients. Brain MRI was normal despite neurologic symptoms in 3 cases. CSF cytology was normal in 62% of patients, whereas Tropheryma whipplei polymerase chain reaction (PCR) analysis was positive in 92% of cases with tested CSF. Periodic acid–Schiff (PAS)-positive cells were identified in cerebral biopsies of 4 patients. All patients were treated with antimicrobial therapy for a mean duration of 2 years (range, 1–7 yr) with either oral monotherapy (TMP-SMX, doxycycline, third-generation cephalosporins) or a combination of antibiotics that sometimes followed parenteral treatment with beta-lactams and aminoglycosides. Eight patients also received hydroxychloroquine. At the end of follow-up, the clinical outcome was favorable in 14 patients (78%), with mild to moderate sequelae in 9. Thirteen patients (72%) had stopped treatment for an average time of 4 years (range, 0.7–14 yr). Four patients had clinical worsening despite antimicrobial therapy; 2 of those died following diffuse encephalitis (n = 1) and lung infection (n = 1). In conclusion, the neurologic manifestations of WD are diverse and may mimic almost any neurologic condition. Brain involvement may occur during or after TMP-SMX treatment. CSF T. whipplei PCR analysis is a major tool for diagnosis and may be positive in the absence of meningitis. Immune reconstitution syndrome may occur in the early months of treatment. Late prognosis may be better than previously reported, as a consequence of earlier diagnosis and a better use of antimicrobial therapy, including hydroxychloroquine and doxycycline combination.

Background Incidence of infectious encephalitis in France is evaluated to be 0.5 to 1/100,000 inhabitants. That means encephalitis are rare infections, and not all physicians do not have expertise about this disease. In case of complex presentations, they may benefit from advices and guidance from a multidisciplinary group. The French infectious diseases society implemented a group of expertise in 2016 to address clinicians’ difficulties with complex cases in a timely manner. Methods Experts were delegated by scientific societies (Infectious Disease, Microbiology, Neurology, Intensive care and Public Health) with regards to their expertise in brain infections. Any physician facing difficulties to manage a patient presenting as a complex case can ask for advice, using a specific e-mail address (encephalite.spilf@infectiologie.com). They have to provide a detailed summary of the clinical case, together with all available biological and etiological results and, when possible, an access to brain images. The case file is then or circulated by mail or discussed in a conference call, within 48 hours. At the end of the discussion, a written answer is produced (detailed recommendations and justification). The traceability of the advice is kept by the French infectious diseases society for both teaching purposes and legal matters. Results So far we had to examine 32 cases, providing from various hospital in mainland France, French West Indies, and Polynesia: 15 from university hospital and 17 from nonuniversity hospitals. Questions (overlapping in some cases) were related to diagnosis procedure (12), to treatment (4), to interpretation of imaging (5), to management of failure (6), and interpretation of test results (10). Our answers were: investigation for autoimmune or inflammatory disease (15); investigation for tuberculosis and/or treatment (14); investigation for tumour (3); complementary tests for an unusual pathogen (10). Pertinence of the advices was adapted in 20 cases (30 evaluated). Conclusion Such a group seems to be useful, and the organization at a national-level works. It is also the opportunity to extend our network in the field of neurological infections, and to use the submitted cases as education material for young ID fellows. Disclosures All authors: No reported disclosures.

Introduction Seule la moitie des encephalites presumees infectieuses sont documentees, une veille sanitaire est justifiee pour permettre la detection d’infections rares ou emergentes. Objectifs En 2016, la cohorte ENCEIF a ete mise en place pour etudier les caracteristiques des encephalites en France metropolitaine, leur prise en charge et le pronostic a long terme. Nous presentons les resultats intermediaires de cette cohorte. Methodes Les centres volontaires ont inclus les patients adultes (≥ 18 ans) qui remplissaient les criteres de definition de cas de l’International Encephalitis Consortium. Des donnees epidemiologiques, cliniques et biologiques ont ete recueillies par un questionnaire dematerialise dans une application securisee (Voozanoo). Resultats Au 1 er sept 2017, 227 patients ont ete inclus : âge median 64 ans (18 a 94), 145 hommes (64 %). Une cause infectieuse a ete identifiee pour 134/217 (62 %) patients. Les causes les plus frequentes etaient HSV ( n = 46 ; 21 %), VZV ( n = 27 ; 12 %), TBE ( n = 12 ; 6 %) et Listeria Monocytogenes ( n = 10 ; 5 %). L’emergence des cas de TBE correspondait a une epidemie survenue en Alsace durant l’ete 2016. L’infection etait consideree comme importee pour 6 (2 %) cas. Discussion Dix-neuf (9 %) patients sont decedes. Parmi 183 non decedes, 121 (66 %) avaient des sequelles mineures ou etaient gueris lors de la sortie (GOS = 5), 45 (25 %) des sequelles moderees (GOS = 4) et 17 (9 %) des sequelles majeures (GOS = 3). Le recueil des sequelles a 6 mois et 1 an permettra de progresser dans la connaissance des consequences a long terme des encephalites infectieuses. Conclusion La cohorte Enceif permet de surveiller l’epidemiologie des encephalites infectieuses en France pour les etiologies classiques, pour la detection d’agents rares ou emergents, et pour la description des sequelles. Informations complementaires Financement : Societe de pathologie infectieuse de langue francaise.

A unique case of pyrimethamine-related stroke-like leukoencephalopathy is described. The imputability of the drug is discussed as well as the similarities with the well-known methotrexate neurotoxicity. Owing to the same mode of action of both drugs by inhibition of the enzyme dihydrofolate reductase, this case is highly suggestive of the pathogenetic role of methylation pathway blockade on myelin synthesis resulting in delayed demyelination. This complication could be avoided by a concurrent folinic acid supplementation.

Thrombomodulin is expressed at the surface of endothelial cells and controls thrombin generation and thrombin-induced platelets and vascular cell activation. Several thrombomodulin gene polymorphisms have been associated with coronary events and brain infarction. In a previous analysis from the Etude du Profil Génétique de l'Infarctus Cérébral (GENIC) study, we found that soluble thrombomodulin (sTM) concentration modulated the risk of and prognosis for brain infarction.In 474 brain infarction cases and 483 controls from the GENIC study, we investigated the relationship between three thrombomodulin gene polymorphisms (-1748G/C, -1208/-1209delTT, +1418C/T) and sTM levels, brain infarction risk and 5-year mortality after stroke.The three polymorphisms were in linkage disequilibrium and defined three major haplotypes with no influence on sTM concentration (all P values0.16). Single locus and haplotype analyses found no significant association with brain infarction, even when the analysis was restricted to individuals without a vascular history. After 5 years of follow-up, we found no relationship with vascular or total mortality (all P values0.64).Our results suggest that these three thrombomodulin gene polymorphisms do not contribute to sTM level variations and are not associated with risk of brain infarction and mortality after stroke.

JC virus (JCV) infection of glial cells can lead to progressive multifocal leukoencephalopathy (PML) in immunocompromised patients. A newly described phenotype of the infection is infection of neurons. This distinct clinical and radiological syndrome is named JCV granule cell neuronopathy, characterized by exclusive or predominant cerebellar atrophy. We report the clinical and radiological longitudinal findings of 5 HIV-infected patients referred to us between September 2004 and November 2011 who exhibited JCV granule cell neuronopathy (4 probable cases and 1 possible). The association of immunocompromised status, progressive cerebellar syndrome, MRI abnormalities with cortical cerebellar atrophy and cerebrospinal fluid positive for JCV on PCR allowed for a highly probable diagnosis. The reversal of the immunocompromised status is the only way to stop the disease evolution. Motor functioning can remain impaired, but the illness itself, unlike progressive multifocal leukoencephalopathy, does not seem to threaten life.