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International audience; BACKGROUND: In order to reduce the risk of progressive multifocal leucoencephalopathy when using natalizumab for more than 12 months, a 6-month drug holiday has been discussed. However, the consequences on short term disease activity have been poorly assessed. OBJECTIVE: The aim of this study was to assess clinical and radiological disease activity within 6 months after stopping natalizumab in very active relapsing remitting Multiple Sclerosis (RRMS) patients. METHODS: In 8 hospitals from Western France, we retrospectively collected clinical and MRI data from consecutive RRMS patients treated with natalizumab for at least 6 months, and who stopped the drug for various reasons except therapeutic failure. Patients didn't receive any other disease modifying treatment after discontinuing natalizumab. RESULTS: A total of 27 patients with very active RRMS before natalizumab start (mean annualized relapse rate of 2.3, MRI activity in 21 of 27 patients) were studied. Within 6 months after discontinuing natalizumab, 18 patients (67%) experienced clinical relapse and 3 additional patients had radiological activity, without clinical relapse. Four patients (15%) experienced a rebound activity, with severe relapse and 20 or more gadolinium enhancing lesions on MRI. CONCLUSION: Such observational data didn't support the concept of drug holiday when using natalizumab in very active RRMS.

International audience; Background and objective: Management of transient ischemic attacks (TIAs) is of vital importance in an attempt to prevent stroke. However, suboptimal management still raise concern among general practitioners (GPs) and emergency department (ED) physicians - the first medical contact of most TIA patients. This may relate to their poorly updated knowledge about TIA. The study was designed to assess knowledge of TIA among these non-neurologists. Methods: The study was a post-mailed questionnaire survey among GPs and ED physicians. The questionnaire related to selective clinical aspects on TIA. Results: There were a total of 85 respondents for analysis, mostly GPs (n = 64; 75.3%), out of 177 mailed physicians. Response rate was 52.7%. Many of these respondents were unaware of the newly proposed TIA definition (59%), unfamiliar with TIA mimics and predictors of post-TIA early stroke recurrence and therefore with the rationales underlying the need of emergency management of TIA. More than one third (39%) were unaware of the relevant national guidelines. Guidelines-aware respondents performed better in most part of the mailed questionnaire. Conclusion: Our results show that poorly updated knowledge about TIA among non-neurologists represents a potential contributing factor to the persisting sub-optimal management of the disorder. Although further studies are needed to confirm this, improved continuous medical education of this group of health care professionals appears warranted

Epileptic seizures (ESs) in the elderly are recognized as frequent, and potentially difficult to diagnose. Their clinical features and relevant diagnostic problems still remain poorly investigated in hospital populations outside the setting of tertiary referral centres. In this study we attempted to improve the understanding of these aspects in community institutions. We conducted a four-year retrospective observational study of 104 consecutive elderly patients with the diagnosis of ES, in 2 French community hospitals. Most ESs were partial (n=50; 48.07%) but generalized ESs were also clinically frequent (n=41; 39.42%). Brain imaging was highly contributive for the diagnosis of partial ESs by demonstrating causative focal structural lesions. ESs were often unprovoked (n=82; 78.84%). Fifty six of these (68.29%) were symptomatic. Stroke lesions were the most identified cause (n=17; 20.73%). In 26 patients (31.70%) aetiology was unknown. Various diagnostic problems were identified. Inter-observer agreement between neurologists and non-neurologists based on clinical judgement was only “fair” (kappa coefficient: 0.28; 95% CI; p=0.002). ESs were initially misdiagnosed in 28 patients (26.92%). The misdiagnosis rate was higher among non-neurologists (n=25; 89.28%) as compared to neurologists (n=8; 28.57%) (p

13 cases of sclero (dermato)myositis are reported with cutaneous signs specific of dermatomyositis in 6 cases. Scleroderma was rather benign, without any visceral involvement. In contrast, myositis was much more severe that usually encountered in systemic sclerosis, threatening the vital prognosis and requiring aggressive therapeutic measures to be controlled. In 6 cases, the axial musculature was involved. Occurrence of each component was not simultaneous. No case was paraneoplastic. Antinuclear antibodies were present in all cases. Antibodies against soluble nuclear antigens, specifically found by others in sclerodermatomyositis, were absent. The very peculiar clinical spectrum of this syndrome associated with immunologic abnormalities, elsewhere described, confirm the individualization of sclerodermatomyositis.

An X-linked spinal muscular atrophy is reported in one family. Four of the five patients were examined. In three, electromyography, conduction nerve velocities and muscle biopsy were consistent with anterior horn cell disease. Similar families in the literature were reviewed and characteristic data were: 1) adult-onset, 2) proximal, bulbar and facial involvement, 3) prominent perioral fasciculations, 4) frequent association of cramps, tremor and sexual dysfunction (hypogonadism and gynecomastia), 5) very slow progression and favorable prognosis. These characteristics define, among the spinal muscular atrophies, a distinct entity named by several authors Kennedy's disease.