La prise en charge thérapeutique du syndrome des jambes sans repos (SJSR) ne doit être envisagée qu’après un diagnostic de certitude. Le SJSR doit donc être phénotypé précisément, une carence martiale doit être recherchée systématiquement et les facteurs favorisants doivent être éliminés lorsque cela est possible. Un traitement médicamenteux sera envisagé pour les formes sévères ou très sévères. Le traitement médicamenteux sera basé sur l’utilisation d’agonistes dopaminergiques, et/ou de ligands α2δ-1, et/ou d’opiacés. En première intention, le traitement sera une monothérapie, à faible posologie et le choix de la molécule sera fonction de l’interrogatoire et du bilan réalisé.Treatment of restless legs syndrome (RLS) must only be considered after a definite positive diagnosis. The RLS phenotype must be characterised precisely, iron deficiency always tested for, and aggravating factors eliminated when possible. Medical treatment is considered for severe or very severe forms and based on dopaminergic agonists, α2δ-1 ligands and/or opioids. First line treatment will be a low-dose monotherapy and the choice of treatment depends on the results of the clinical examination and investigations.

La première étape, dans la prise en charge d'un malade atteint du syndrome des jambes sans repos (SJSR), est d'identifier et traiter une cause à l'origine d'un SJSR secondaire, tel qu'une carence en fer et d'identifier des médicaments qui peuvent provoquer ou aggraver la maladie.Les patients atteints d'un SJSR doivent être encouragés à maintenir une bonne hygiène de sommeil.L'instauration d'un traitement pharmacologique est limitée aux patients avec des critères diagnostiques stricts, est évaluée sur une base individuelle et dictée par les répercussions cliniques du SJSR.Quatre classes thérapeutiques sont au centre du traitement pharmacologique symptomatique du SJSR : les agents dopaminergiques, certains anti-épileptiques, les opioïdes et les benzodiazépines.Les agonistes dopaminergiques constituent actuellement le traitement de choix du SJSR, en particulier si un traitement quotidien est nécessaire ou si la symptomatologie clinique est sévère. Deux agonistes dopaminergiques disposent de l'AMM en France pour le traitement du SJSR modéré à sévère : le ropinirole (Adartrel®) et le pramipexole (Sifrol®).Après l'instauration du traitement, les patients doivent avoir un suivi régulier pour déterminer l'efficacité du traitement et la survenue éventuelle d'effets secondaires. Une attention particulière est portée à l'apparition d'un phénomène d'augmentation, résultat d'une aggravation paradoxale iatrogène de la maladie sous traitement dopaminergique.The first step in the management of restless legs syndrome (RLS) is to identify, and if possible to treat any condition which might cause or worsen RLS, such as iron deficiency or some drug treatments.The patients suffering from RLS should be prompted to keep a healthy sleep schedule.Drug treatment should be restricted to patients with a clear clinical diagnosis, decided on an individual basis, when the clinical impact is serious.Four drug classes are central to the treatment of RLS: dopaminergic agents, some antiepileptics, opioids, and benzodiazepines.Dopaminergic agonists are the treatment of choice, especially when daily treatment is indicated, or if the symptoms are severe. Two dopaminergic agonists are licensed in France for the treatment of RLS: ropinirole (Adartrel®) and pramipexole (Sifrol®).After initiation of treatment, the patients should benefit from a regular follow-up in order to evaluate the efficacy of treatment and to identify possible side-effects. Special care should be given to the detection of augmentation, a phenomenon characterized by a paradoxical worsening of the symptoms with treatment. Some particular conditions, such as RLS comorbid with renal insufficiency, during pregnancy, and in the child are discussed.

La thrombolyse intraveineuse (TIV) reste à l’heure actuelle le seul traitement médicamenteux de reperfusion validé dans la prise en charge de l’ischémie cérébrale en phase aiguë. Depuis l’autorisation de mise sur le marché du rt-PA, les stratégies thérapeutiques impliquant la TIV ont évolué, validées par des essais randomisés qui ont permis d’étendre ses indications initiales. Les recommandations de l’European Stroke Organisation 2021 sur la TIV dans l’ischémie cérébrale de phase aiguë abordent différentes situations cliniques en déclinant pour chacune d’entre elles les niveaux de preuve issus de la littérature. Comprendre d’où proviennent ces recommandations ainsi que leurs limites au regard de l’état actuel des connaissances permettent de mieux étayer les décisions thérapeutiques.Intravenous thrombolysis (IVT) remains the only approved systemic reperfusion therapy for the management of acute cerebral ischaemia. Therapeutic strategies involving IVT have evolved, validated by randomised trials allowing for the extension of its initial indications. The European Stroke Organisation (ESO) 2021 guidelines on IVT in acute cerebral ischaemia address different clinical situations, stating for each of them the levels of evidence derived from the literature. Understanding where these recommendations come from and their limitations in light of the current state of knowledge is a useful support for clinical decision-making.

Cet article propose d’examiner, auprès de populations d’étudiants, les liens entre le climat de justice établi par le supérieur hiérarchique, son style de management différencié sur les deux dimensions structure et considération, et le vécu d’un groupe de travail en termes de harcèlement moral mais aussi en examinant ses conséquences sur l’anxiété et le stress. L’élaboration d’outils permettant d’évaluer ces liens est présentée. Il est montré dans une étude 1, utilisant le « paradigme d’identification », que les sujets pensent que le contexte professionnel est caractérisé plutôt par un climat de justice et assez peu de harcèlement. Ils ne considèrent pas spécialement que le milieu professionnel engendre stress et anxiété. Une étude 2 montre que les dimensions de leadership ont un impact sur les perceptions du climat de justice : des comportements de considération de leaders influencent positivement le sentiment de justice et c’est toujours quand la considération est forte que les sujets estiment qu’il y aura moins de harcèlement.Using student participants, this article examines the links between the justice climate established by a supervisor, the leadership style determined by the dimensions of initiating structure and consideration, and the group’s experiences in terms of morale harassment and its consequences on anxiety and stress. The development of questionnaires necessary for testing these links is presented. In addition, in Study 1, using the identification paradigm, the results indicated that the participants thought they would work in a rather just climate and that they would not be subjected to harassment. They did not believe that their work environment would especially produce stress and anxiety. Study 2 showed that leadership has an impact on perceptions of the justice climate; leaders’ consideration behaviors having a positive effect on justice climate and creating conditions where one expects there to be less harassment.

This paper investigates the implications of different prize structures on effort provision in dynamic (two-stage) elimination contests. Theoretical results show that, for risk-neutral participants, a structure with a single prize for the winner of the contest maximizes total effort, while a structure with two appropriately chosen prizes (a runner-up prize and a final prize) ensures incentive maintenance across stages. In contrast, a structure with two prizes may dominate a winner-takes-all contest in both dimensions if participants are risk-averse. Evidence from laboratory experiments is largely consistent with these predictions, suggesting that contest design should account for risk attitudes of participants.

La maladie de Still de l'adulte (MSA) est une affection inflammatoire multisystémique d'étiologie inconnue caractérisée par une atteinte articulaire, une fièvre souvent hectique, une éruption fugace et des atteintes viscérales multiples. Au cours de cette affection systémique, l'atteinte rénale semble rare et polymorphe. Ce type d'atteinte est plus volontiers associé aux arthropathies sévères et à l'amylose de type AA. Des observations de néphropathies tubulo-interstitielles, membrano-polifératives ou mésangiales ont été rapportés. Toutefois, la présence d'une néphropathie nécrosante extracapillaire, jamais rapportée auparavant au cours de la MS, peut poser un problème de diagnostic différentiel.Nous rapportons l'observation d'une MSA compliquée d'une vascularite rénale.Une patiente âgée de 40 ans, sans antécédents, a été hospitalisée une fièvre au long cours évoluant un mois avant son admission. Elle se plaignait de polyarthralgies chroniques bilatérales et symétriques touchant les petites articulations associées à une éruption cutanée papuleuse fugace. L'examen physique était strictement normal. La biologie a révélé une hyperleucocytose à neutrophiles et une anémie microcytaire de type inflammatoire. On a noté un syndrome inflammatoire biologique (SIB) avec hyperferritinémie importante à 2 758 mg/mL. Un bilan infectieux comportant un bilan tuberculeux et des sérodiagnostics (Wright, Vidal, la maladie de Lyme, CMV, EBV, HVB, HVC, HIV, rubéole et toxoplasmose) étaient négatif. La recherche d'une cause maligne (les marqueurs tumoraux, un scanner thoracoabdominopelvien et la ponction sternale) a éliminé une hémopathie ou une néoplasie sousjacente. Le bilan immunologique (anticorps antinucléaires, antiphospholipides, anticorps anticytoplasme des polynucléaires et cryoglobulinémie) était négatif. Une MSA a alors été retenue devant trois critères majeurs (fièvre, arthralgies et un taux de PNN > à 80 %) et deux critères mineurs (éruption et l'hyperleucocytose > à 10 000/mm3) selon les critères de Fautrel. Durant son hospitalisation, elle a présenté une atteinte rénale avec une protéinurie de 24 heures à 0,6 g sans hématurie, ni d'insuffisance rénale ou hypertension artérielle. L'examen anatomopathologique de la ponctionbiopsie rénale a montré un aspect de glomérulonéphrite proliférative extra-capillaire pauci immune. Devant la sévérité de l'atteinte rénale, la patiente a été traitée par des boli de méthyl-prednisolone relayés par corticoïde orale en association à 6 boli de cyclophosphamide relayés par mycophénolate mofétil à la dose de 2 g/j. Après un recul de 12 mois, l'évolution était bonne, avec disparition de la fièvre, du SIB et de la protéinurie.La vascularite rénale est exceptionnelle au cours de la MSA quoiqu'il puisse s'agir d'une association fortuite entre deux pathologies différente.

Introduction. - The aim of our study was to compare the efficacy and safety of intravenous thrombolysis of cerebral ischemia as it has been established in a distant hospital (DH) through telemedicine tools or in neurovascular unit of the University Hospital of Besancon.Method. - Our work was conducted retrospectively at the University Hospital of Besancon from 1 January 2003 to December 31, 2009.Results. - Fibrinolysis was introduced at the university hospital in 98/161 patients (61%) and a DH in remote 63/161 patients (39%). A favorable neurological outcome (Rankin 0/1) was observed in 27/98 patients (27.5%) treated at University Hospital and in 25/63(39.5%) patients in a DH. There was no significant difference between the two subgroups. Symptomatic hemorrhagic transformation occurred in 5/98 (5%) patients treated at University Hospital and in 1/63 (1.5%) patients treated in DH. There was no significant difference between the two subgroups.Conclusion. - Our study shows that fibrinolysis remotely using the tools of telemedicine has, from 2003 to 2009, in Franche-Comte deal effectively and without risk of a significant proportion of patients. (C) 2011 Elsevier Masson SAS. All rights reserved.

OBJECTIVE:To describe CSF biomarker profiles in posterior cortical atrophy (PCA), which induces high-order visual deficits often associated with Alzheimer disease (AD) pathology, and relate these findings to clinical and neuropsychological assessment.METHODS:This prospective observational study included 22 patients with PCA who underwent CSF biomarker analysis of total tau (t-tau), phosphorylated tau on amino acid 181 (p-tau181), and amyloid β (Aβ(42)). At group level, the CSF profiles of patients with PCA were compared to those of patients with typical AD and patients with other dementia (OD). Individually, the clinical presentation of patients with PCA was correlated to their CSF profile to assess the predictability of clinical features for diagnosis of underlying AD pathology.RESULTS:At group level, the PCA biomarker profile was not different from that of the AD group, but very different from that of the OD group (p < 0.001). More than 90% of patients with PCA had CSF profiles consistent with AD. All patients with PCA with either isolated higher-order visual deficit (n = 8) or visual deficit associated with memory impairment (n = 11) had CSF profiles consistent with AD. Only one of the 3 patients with PCA with asymmetric motor signs fulfilled biological CSF criteria for AD.CONCLUSIONS:PCA syndrome is usually associated with CSF biomarkers suggestive of AD, as shown by previous neuropathologic studies. This does not apply in case of motor signs suggesting associated corticobasal syndrome. CSF biomarkers help to discriminate AD from non-AD processes associated with this condition.

Introduction. - Voltage-gated potassium channels (VGKCs) antibodies are associated with neuromyotonia, limbic encephalitis and Morvan syndrome.Case report. - We report the case of a patient who, after three weeks of fever, presented an anamnestic syndrome, associated with confusion and partial seizures. MRI showed left hyperintensity of mesial temporal structures on Flair images and right hippocampal atrophy on T1 weighted sequences. Laboratory tests only showed high level of anti-TPO antibodies. Thus, the patient was considered as having Hashimoto's encephalopathy. She was treated with intravenous methylprednisolone with no improvement of symptoms. On the contrary, the patient suffered from insomnia, deep diurnal drowsiness and complete disappearance of REM sleep. Episodes of hypothermia and severe hyponatremia were recorded. Serum VGKC antibodies were found at high level. After intravenous immunoglobulin treatment followed by methylprednisolone, we noted remarkable improvement of clinical status. Polysomnography showed reappearence of REM sleep.Conclusion. - This case report broadens the spectrum of clinical manifestations associated with VGKC antibodies and suggests that VGKC are implicated in regulation of sleep. The potential pathophysiological mechanisms linking sleep disturbances and VGKC antibodies are discussed. (c) 2007 Elsevier Masson SAS. Tous droits reserves.

l-2-hydroxyglutaric aciduria (l-2-HGA) is a metabolic disease with an autosomal recessive mode of inheritance. It was first reported in 1980. Patients with this disease have mutations in both alleles of the L2HDGH gene. The clinical presentation of individuals with L-2-HGA is somewhat variable, but affected individuals typically suffer from progressive neurodegeneration. Analysis of urinary organic acids reveals an increased signal of 2-hydroxyglutaric acid, mainly as the l-enantiomer. L-2-HGA is known to occur in individuals of various ethnic backgrounds, but up to now mutation analysis has been mainly focused on patients of Turkish and Portuguese origin. This led us to confirm the diagnosis on the DNA level and undertake the corresponding mutation analysis in individuals of diverse ethnicity previously diagnosed with l-2-HGA on the basis of urinary metabolites and clinical/neuroimaging data. In 24 individuals from 17 families with diverse ethnic and geographic origins, 13 different mutations were found, 10 of which have not been reported previously. At least eight of the patients were compound heterozygotes. The identification of two mutations (c.751C > T and c.905C > T in exon 7) in patients with different origins supports the view that they occurred independently in different families. In contrast, the mutation c.788C > T was detected in all six Venezuelan patients originating from the same Caribbean island of Margarita, but not in other patients, thus rendering a founder effect likely. None of the mutations was found in the control population, indicating that they are most probably causative. Mutation analysis may improve the quality of diagnosis and prenatal diagnosis of L-2-HGA.

With steroid therapy, it is commonly considered that prognosis is good in giant cell arteritis. However serious or even fatal complications may occur. Here we report the case of a patient who developed fatal giant cell arteritis with severe stenosis of both vertebral arteries and right carotid siphon. Several similar cases have been reported in the literature. Initially diagnosis may be difficult because neurological manifestations are intermittent and classical signs of giant cell arteritis may be lacking. In such condition the reason of poor outcome is unknown and therapy remains empiric.

La Grèce Antique est le berceau de la tradition alimentaire méditerranéenne marquée, non seulement par la triade du pain, de l'olivier et de la vigne, mais aussi par une culture du partage et de la commensalité. Ce modèle alimentaire a été adopté par les Romains qui l'on diffusé à une grande partie de leur Empire, avant qu'il ne replonge dans l'anonymat. C'est à des épidémiologistes américains, Leland Allbaugh et Ancel Keys, que l'on doit sa redécouverte, au milieu du XXe siècle. Leurs études ont permis d'établir une relation robuste entre l'alimentation et la mortalité coronarienne et, au-delà, la longévité. Aujourd'hui, le « régime » méditerranéen est promu en santé publique comme un modèle alimentaire équilibré, diversifié et vertueux, caractérisé par un apport restreint en graisses saturées, important en acides gras monoinsaturés, et une forte densité nutritionnelle. Symbolisé par une pyramide alimentaire, il apparaît le plus adapté pour répondre aux enjeux de prévention des maladies cardio-métaboliques et pour relever le défi de la longévité.Before the Christian era, Greece was the birthplace of the traditional Mediterranean diet, which is characterized by an alimentary triad based on the consumption of bread, wine and olive products but also by the culture of sharing and the joy of commensalism. This dietary model was adopted by the Romans who disseminated it throughout the Empire before a long period of waning over several centuries. At the end of the Second World War, the virtues of the Mediterranean diets were rediscovered following epidemiological surveys supervised by two American scientists, Leland Allbaugh and Ancel Keys. Both demonstrated the existence of a strong relationship between dietary patterns and death rates from coronary heart disease. At present, the so-called Mediterranean diet is promoted as a new public health reference since it appears as a well-balanced model for providing a high monounsaturated/saturated fat ratio and a large coverage of all nutritional needs. The alimentary pyramid is probably the most appropriate illustration that can be used for depicting this model aimed both at combating early deleterious cardiovascular outcomes and at prolonging the life expectancy.

A bstract : Vascular dementia (VaD) includes several different vascular mechanisms and changes in the brain. Among VaD, CADASIL is an inherited angiopathy caused by mutations in the Notch3 gene. The pathological hallmark of CADASIL is a granular osmiophilic material deposit (GOM) that is not only found in the brain, but also in the peripheral vascular tree. Consequently, a window into the brain was opened from a strictly neurological disease with tremendous consequences thanks to a skin biopsy. The latter was and continues to be used as a diagnostic tool for CADASIL, despite an immunohistochemical test that is now available. The skin biopsy first used as a diagnostic tool revealed the existence of numerous other VaDs presenting systemic vascular changes. Later, skin biopsy became a research tool, and a morphological skin vessel change classification was proposed on 300 patients. Interestingly, similar skin vessel lesions appear to be related to the same biological modifications. In addition, an early destruction of the medial muscle cells was noticed in 74% of cases. Because vascular smooth muscle cells secrete a powerful endothelial permeability factor (VEGF), their destruction could lead to a decrease in vascular permeability. Cocultures of endothelial cells with vascular muscle cells showed that their presence doubled vascular permeability. Thus, alteration or the loss of vascular muscle cells likely results in hypopermeability, in addition to vessel wall hypotonia and a watershed hypoperfusion. The wealth of information brought forth by knowledge of CADASIL provided new tools for research and clues for understanding the consequences of vascular impairments in dementia.

The authors studied a 47-year-old patient who presented with an association of deafness, acute cerebral stroke-like episode, leukoencephalopathy, and extensive basal ganglia calcifications. Late onset and neuroradiologic findings were atypical for MELAS syndrome (Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis, and Strokelike episodes). A heteroplasmic G to A transition at nucleotide 4332 in the tRNA glutamine gene was identified in the patient's muscle mitochondrial DNA. The pathogenicity of the mutation was shown in single muscle fibers by the correlation between high mutation load and cytochrome c oxidase defect.