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Competing Interests: S. Guettier déclare n’avoir aucun lien d’intérêts. E. Touzé déclare participer ou avoir participé à des interventions ponctuelles pour les entreprises BMS, Pfizer, Boerhinger-Ingelheim, Bayer, Daichi-Sankyo. H. Raoult, J.-Y. Gauvrit n'ont pas fourni de déclaration de liens d'intérêts.

Competing Interests: S. Guettier déclare n’avoir aucun lien d’intérêts. E. Touzé déclare participer ou avoir participé à des interventions ponctuelles pour les entreprises BMS, Pfizer, Boerhinger-Ingelheim, Bayer, Daichi-Sankyo. H. Raoult, J.-Y. Gauvrit n'ont pas fourni de déclaration de liens d'intérêts.

Competing Interests: A. de Roquemaurel, P. Branger, L. Plisson, E. Touzé déclarent n’avoir aucun lien d’intérêts.

Competing Interests: A. de Roquemaurel, P. Branger, L. Plisson, E. Touzé déclarent n’avoir aucun lien d’intérêts.

Oral anticoagulants dramatically reduce the risk of embolic stroke in patients with atrial fibrillation (AF). However, each year, 1 to 3 % of patients will present an ischemic stroke despite being on oral anticoagulant. These events can result from efficacy fluctuations, non-cardioembolic stroke, or insufficient efficacy despite appropriate anticoagulation. There are several therapeutic options depending on presumed mechanism of ischemic stroke. However, none of these options has been specifically evaluated in appropriate studies. These options include: reinforcement of education or change in VKA drugs in case of documented therapeutic fluctuations, adjunction of aspirin, change to direct oral anticoagulants, or percutaneous left-appendage closure., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Introduction: Cavitary white matter changes are mainly described in leukodystrophies and especially in vanishing white matter disease. Large cavitary lesions are not typical for multiple sclerosis (MS)., Methods: We studied MS patients with large cavitary brain lesions. Patient characteristics, disease onset/duration/subtype, expanded disability status scale (EDSS), mini mental state (MMS), vanishing white matter disease genetic analysis, and MRI characteristics of the cavitary lesions were analyzed., Results: Twenty patients were analyzed (6 men and 14 women). Mean age at disease onset was 37.6 (range 17-58). Mean disease duration was 10 years (range 2-20). Five patients had initial relapsing-remitting MS and nine patients had primary-progressive MS. Mean EDSS was 5.5 (range 2-8). Mean MMS was 20/30. Vanishing white matter disease genetic analysis was performed and negative in seven patients. Inferior corpus callosum lesions were seen in all patients with available sagittal FLAIR sequences. Cavitary lesions were strictly supratentorial, and located inside the diffuse leukoencephalopathy, with often a posterior predominance., Conclusion: MS patients with large cavitary lesions seem to represent a MS subgroup, predominantly women, with relatively late disease onset, predominantly primary-progressive type, relatively high EDSS scores, and severe cognitive dysfunction., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Fibromuscular dysplasia (FMD) is an uncommon, segmental, nonatherosclerotic arterial disease of unknown etiology, involving intermediate-sized arteries in many areas of the body. It affects primarily women. FMD is often asymptomatic and discovered as an incidental finding. It can, however, be associated with ischemic stroke spontaneous dissection, intracranial aneurysm, and hemodynamic compromise of the distal circulation, and therefore can be a cause of cerebral ischemic symptoms or subarachnoid hemorrhage in some patients., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Stroke is a disabling and very heterogeneous disease that has beneficiated from major therapeutic improvements over the past 25 years both in terms of preventive strategies and acute care. The better knowledge of the epidemiology of stroke has allowed to recognize the worldwide burden of the disease, and to identify the vascular risk factors. This article reviews the main current data about the epidemiology of stroke.

Background: Over the past 20 years, the neurology specialty has changed because of the explosion of information and new treatment modalities that became available, and has consequently become more complex and diversified. The satisfaction of residents concerning their training and the competencies that they acquire has never been thoroughly assessed in France., Methods: We conducted a national survey in order to assess (1) the methods for training and validation; (2) the level of knowledge that residents perceived to have acquired in different domains; and (3) their satisfaction towards training and their wishes., Results: One hundred and eight residents replied to the survey. The main sources of training were local teaching (74%), personal work (61%), scientific (57%) and didactic (54%) papers. Residents seemed unable to acquire knowledge on all domains of the curriculum established at a national level, particularly for neurophysiology, neuropsychology, comatose state and sleep disorders, oncology and psychiatry. Even postgraduate year four residents were not fully competent with several technical tools, particularly neurophysiological tests. Fifty eight percent of residents were satisfied with their training, but 16% were not and 26% were half-hearted. Overall the residents were in favor of more standardization in their training, national-level certification, and would be keen on having access to clinics and the use of a follow-up monitoring chart., Conclusion: Our results suggest that it would be useful to better identify the competencies that every neurologist should acquire and to use appropriate tools to reach these objectives.

Despite advances in diagnosis and treatment, atherosclerosis remains the second cause of death in the world. Due to technical advances, high resolution MRI (HR-MRI) allows depiction of the wall of cervical arteries, especially carotid atherosclerosis. HR-MRI allows visualization of the different components of atherosclerosis: necrotic lipid core, intraplaque hemorrhage, calcifications and fibrous cap. Global plaque volume as well as the volumes of individual plaque components can be calculated. Atherosclerotic plaque structure analysis, along with stenosis measurement, contribute to the stratification of the stroke risk. HR-MRI may also be used to assess treatment efficacy aimed at stabilizing or reducing plaque progression. Beyond the arterial lumen, direct evaluation of vessel wall should modify the management of atherosclerosis in the years to come.

Atherosclerotic carotid stenosis is a risk factor for stroke and other vascular events. Preventive medical treatment is based on the elimination of risk factors and the use of antithrombotic drugs. Surgery remains the standard revascularization treatment. The benefits of carotid surgery are especially important for severe (> or = 70%) symptomatic carotid stenosis. The benefits of surgery for moderate (> or =60%) asymptomatic stenosis or for moderate (50-69%) symptomatic stenosis are slight, particularly in women. The treatment decision requires consideration of individual factors to optimize the risk/benefit ratio.

Antiplatelet therapy is the reference treatment for secondary prevention after noncardioembolic ischemic stroke. The main aim of the Match study was to compare the combination of aspirin (75 mg) and clopidogrel (75 mg) with clopidogrel (75 mg) alone in secondary prevention after recent ischemic stroke or transcient ischemic attack in high-risk patients. The incidence of the composite principal endpoint (ischemic stroke, myocardial infarction, vascular death, or acute ischemic event causing hospitalization) was 15.7% at 18 months in patients in the aspirin-clopidogrel arm, compared with 16.7% in the placebo-clopidogrel arm. The relative risk reduction (6.4%) was not significant (95%CI, -4.6 to 16.3; p=0.244). Patients receiving the combination of aspirin and clopidogrel had more life-threatening bleeding than those treated by clopidogrel alone (2.6% vs 1.3%; 95%CI, 1.3 to 2.6; p<0.001). Recruitment that began too late and an over-selected population, with overrepresentation of patients with diabetes and small vessel disease in particular, may partly explain these negative results.

Objective: To estimate the prevalence of asymptomatic atherothrombotic lesions and the absolute risk of vascular events in patients with ischemic stroke., Methods: We conducted a systematic review of studies published between 1980 and 2004 which allowed calculating prevalence of asymptomatic lesions and absolute risks of vascular events in stroke patients. Studies included in the review of absolute risks had to include at least 100 patients followed for at least 1 year and with less than 5% of lost to follow-up., Results: Coronarography was used in only one study which does not provide any precise estimation of the prevalence of asymptomatic coronary lesions. Depending on the population and the method used for the diagnosis of coronary disease, the prevalence of asymptomatic coronary artery disease ranged from 25 through 60%. Very few studies have been devoted to the prevalence of abdominal aortic disease in stroke patients. The prevalence of abdominal aortic aneurysm has been estimated to 1% and that of peripheral arterial disease to 10%. Thirty-six cohorts were identified. The absolute risk of myocardial infarction was about 2%/year and that of vascular death 2%/year., Conclusion: Stroke patients carry a high risk of coronary and vascular events. Additional studies are required to better identify patients with a high prevalence of asymptomatic atherosclerotic lesions and with a high risk of vascular events.

Atherosclerosis is a ubiquitous inflammatory disease. Patients presenting an acute atherothrombotic event (acute coronary syndrom, stroke, aortic aneurysm, ...) have an increased risk of events in remote arterial territories affected by atherosclerosis. These patients could benefit from systematic screening of asymptomatic atherosclerotic lesions to avoid these complications. For each atherosclerotic territory (coronary artery, carotid artery, aorta, peripheral arteries including renal arteries), we review the methods for screening asymptomatic atherothrombotic lesions which could justify specific treatments: coronary artery stenosis > or = 50%, carotid artery stenosis > or = 60%, renal artery stenosis > or = 50%, and abdominal aortic aneurysm > or = 30 mm. This review shows that non invasive methods (ie, echography, tomodensitometry) are widely available for diagnosis of asymptomatic lesions in carotid and renal arteries, and in the aorta. Despite its invasive caracteristic, coronarory angiography remains the gold-standard for the diagnosis of coronary artery disease. However, cardiac multi-slices CT-scan appears a promising technique for asymptomatic patients.

Strokes are rarely secondary to spontaneous carotid artery thrombosis. The objectives of this retrospective analysis were to define characteristic features and the clinical course. The study population included eight patients (6 females/2 males) seen at six university neurological centers. Age of onset was 46.5 years (range 38-52). Half of the patients had no vascular risk factor. Symptoms were TIA (n=1), strokes (n=7). Echotomography revealed intraluminal thrombus, with occlusion in 2 cases. Thrombi were found in common carotid artery (n=3), carotid bifurcation (n=2) and internal carotid artery (n=3). The thrombus was mobile in 4 cases. Seven patients were treated by anticoagulation therapy, one by surgery because of recurrent TIA. Further echotomographic exams revealed total resolution (3 cases) or decrease of the thrombus (3 cases). Occlusion was definitive in one patient. A cause was identified in six patients: acute leukemia (n=1), thrombocytopenic purpura (n=1), iron deficiency anemia (n=4).

Introduction: The consequences of lightning injuries on the peripheral nervous system are widely unknown., Case Report: We report on a 31 year-old woman who developed a bilateral brachial plexopathy 15 days after a lightning strike. The patient recovered progressively., Conclusion: Peripheral neuropathies due to lightning strikes are probably unrecognized in most of the cases, because occurrence may be delayed and only few patients were adequately investigated. Pathophysiological hypotheses are unclear and lean on experimental studies using electrical current.

Atherosclerosis of carotid and vertebrobasilar arteries is a strong risk factor for ischemic stroke, as well as a strong and independent predictor of myocardial infarction and vascular death. Within the past years, the important progress made in the knowledge of carotid atherosclerosis prognosis has contrasted with the lack of studies for vertebral atherosclerosis. In patients with carotid atherosclerosis, the risk of stroke is clearly related to the severity of stenosis and the risk of recurrent ipsilateral stroke is much higher than the risk of a first-ever stroke. Patients with intracranial atherosclerosis seem to carry a high risk of stroke. Therapies for the prevention of vascular events in patients with carotid and vertebrobasilar atherosclerosis include modification of arterial risk factors, antithrombotic therapies and sometimes carotid surgery. When the benefit of surgery is very high for patients with symptomatic severe carotid stenosis, this benefit is small for those with symptomatic moderate or asymptomatic severe carotid stenosis. New medical strategies and the advantages of angioplasty are currently under investigation.

We report a unusual case of neuralgic amyotrophy (NA) occurring during the seroconversion stage of an HIV infection. Combined with previously published cases, our observation suggests that NA associated with HIV could belong to the group of early multiplex mononeuritis. Neurologists should be aware of HIV infection when managing a patient with NA.

Diffusion tensor imaging allows a 3D analysis of water molecular motion and an exploration of white matter tracts integrity and orientation. We report a diffusion tensor study of wallerian degeneration of the corticospinal tract following a capsulo-lenticular hemorrhage in a 49-year old man. Reduced anisotropy (loss of fiber coherence) with preserved diffusivity of the damaged corticospinal tract was observed. This highlights the feasibility of diffusion tensor imaging on clinical MR units and its ability to quantify the degree of white matter tract destruction.

Few studies have been devoted to the natural history of cervical artery dissection (CAD). This review shows that apart from neurological damage due to initial stroke, the prognosis of DAC is good. Indeed, the risk of recurrent DAC or recurrent stroke is very low, under 1% per year in patients without typical connective tissue disease. Patients with connective tissue disease (Ehlers Danlos syndrome or Marfan syndrome) or familial history of arterial dissection have an increased risk of recurrent CAD. However, the number of cases due to these diseases remains very low. The other risk factors for recurrent CAD or stroke remain unknown. The benefit of antithrombotic treatments at the acute phase of CAD or for secondary prevention has been never assessed in a randomized clinical trial.

Stiff limb syndrome is a recently described variant of stiff man syndrome and is characterized by limb stiffness and spasms. Unlike stiff man syndrome, stiff limb syndrome is rarely associated with anti GAD antibodies, poorly improves after symptomatic treatment, and has a relapsing and remitting course. Both stiff man and stiff limb syndromes are frequently associated with auto-immune diseases. We report a case of a 70-year old man who presented with a stiff limb syndrome associated with symptoms highly suggestive of Hashimoto's encephalopathy. The signs of encephalopathy dramatically resolved after corticosteroid treatment, and remissions was complete after 12 months. The stiff limb syndrome had a remitting course despite symptomatic treatment including diazepam, baclofen and vigabatrin and immunomodulating treatments including corticoid and intravenous immunoglobulins.

Background: Central nervous system (CNS) demyelinating episodes have been described following numerous vaccines but there is no definite conclusion about a causal relationship. Recently, in France, in the context of an Expanded Program on Immunization, several cases of CNS demyelination have been observed following injection of recombinant hepatitis B (HB) vaccine, leading to great concern., Methods: We performed a hospital-based case-control study of 121 patients with a first episode of CNS demyelination occuring between July 1993 and December 1995 and 121 age and sex matched controls seen in the same period. Data on vaccinations history of cases and controls were collected by a postal questionnaire and confirmed by a phone interview., Results: Adjusted odds ratio (OR) obtained from conditional logistic regression between a first episode of CNS demyelination and any vaccination were equal to 1.4 (95 p. 100 CI 0. 5-4.3) for an exposure within the 60 previous days and 2.1 (95 p. 100 CI 0.7-6.0) for an exposure within the 61-180 previous days. Similar results were found for HB vaccine exposure within the 60 previous days (adjusted OR=1.7, 95 p. 100 CI 0.5-6.3) or within the 61 to 180 previous days (adjusted OR= 1.5, 95 p. 100 CI 0.5-5.3)., Conclusion: These findings did not permit to exclude confidently an association between HB vaccine and the occurrence of a first CNS demyelinating episode.

Paraneoplastic limbic encephalitis (PLE) is a manifestation of clinico pathological entity encephalo-myelo-neuropathy associated with anti-neuronal antibodies type 1 (ANNA-1 also called anti-Hu). Isolated PLE is rare. We reported a case of PLE in a 61-year-old heavy smoker man. An inappropriate antidiuretic hormone secretion syndrome was associated. Cranial MRI showed hyperintensity in amygdalo-hippocampic regions on T2 weighted sequences which appeared hypointense on T1-weighted sequences without gadolinium enhancement. Anti-Hu antibodies were absent in serum and in CSF. Despite chemotherapy, he died 18 months after disease onset. Our patient presented PLE without myelonouropathy and without ANNA-1 which suggests a different immunopathology.