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Resume Introduction Depuis 2003, la thrombolyse intraveineuse des infarctus cerebraux par rt-PA s’est developpee a l’hopital militaire de Toulon. Nous rapportons les resultats de l’evaluation de notre pratique, compares aux donnees de la litterature. Patients et methode Les patients thrombolyses entre septembre 2003 et juin 2009 pour un infarctus en territoire carotidien ont ete inclus prospectivement. Le score de Rankin (m-RS) a ete evalue a trois mois (evolution defavorable si m-RS superieur a 2), puis des parametres correles au pronostic ont ete recherches par analyse multivariee. Resultats Cent un patients ont ete inclus (National Institute Health Stroke Scale [NIHSS] initial moyen = 15,2). Le taux d’evolution favorable a trois mois etait de 53,4 %. L’absence de diabete, un score NIHSS bas, un delai de thrombolyse court, et la prise anterieure de statines etaient independamment predictifs d’evolution favorable a trois mois. Conclusions En termes de securite et d’efficacite, nos resultats sont comparables aux donnees de la litterature. L’efficacite du rt-PA intraveineux est mediocre dans notre serie pour les infarctus cerebraux par atherome cervical et dissection arterielle, vraisemblablement du fait d’un taux eleve de thrombose carotidienne interne dans ces groupes. Notre etude suggere aussi que la prise precessive de statines puisse etre un facteur independant d’evolution favorable post-thrombolyse.

Points essentiels Le syndrome FXTAS (Fragile X associated Tremor Ataxia Syndrome) est un syndrome neurodegeneratif specifique des sujets porteurs d’une premutation du gene FMR1 (fragile X mental retardation 1). Il touche essentiellement les hommes premutes âges de plus de 50 ans . Ce syndrome est distinct du syndrome de l’X fragile . Le syndrome FXTAS reste encore sous estime . Il devrait etre evoque chez un patient de plus de 50 ans qui presente un tremblement d’action avec une ataxie cerebelleuse, d’autant plus qu’il existe un parkinsonisme et/ou des troubles cognitifs, des antecedents familiaux d’infertilite, de menopause precoce ou de retard mental. La presence d’hypersignaux T2 et FLAIR des pedoncules cerebelleux moyens en IRM justifie, dans ce contexte, la recherche de la premutation FMR1.

Resume Introduction La retinopathie liee a la prise d’interferon-α est un fait etabli chez les patients atteints d’hepatite C. Seuls cinq cas de retinopathie secondaire a l’interferon-s ont ete rapportes dans la litterature. Observation Un homme, âge de 58 ans, atteint d’une sclerose en plaques traitee par interferon-s 1a 44 μg sous-cutane (SC) trois fois par semaine depuis 2001, presenta une gene visuelle bilaterale en juillet 2007. L’acuite visuelle etait normale. A l’examen du fond d’œil, etaient retrouves des nodules cotonneux de maniere bilaterale et isolee a l’origine d’une retinopathie. Il n’avait aucun facteur de risque cardiovasculaire. L’ensemble du bilan etiologique etait normal. La iatrogenie de l’interferon-s 1a SC fut evoquee. Ce traitement fut arrete en juillet 2007. Le fond d’œil de controle a deux mois se normalisa et le patient etait asymptomatique sur le plan visuel. Conclusion Tout nouveau symptome visuel chez un patient traite par interferon (IFN) doit inciter a realiser un fond d’œil a la recherche d’une retinopathie. En cas de negativite du bilan etiologique, l’imputabilite de l’IFN pourrait etre evoquee et justifier eventuellement de l’arret du traitement.

Resume Le rare syndrome d’hypersensibilite aux antiepileptiques, defini par la triade fievre elevee, rash cutane et defaillance multiviscerale, peut etre mortel dans 10 % des cas et se doit donc d’etre connu. Il survient une a 12 semaines apres le debut de la prise de l’antiepileptique responsable, le plus souvent un antiepileptique aromatique. Le syndrome d’hypersensibilite aux antiepileptiques est de diagnostic parfois difficile, en particulier vis-a-vis du drug rash with eosinophilia and systemic symptoms (DRESS) ou l’hypereosinophilie est par definition constante. Les mecanismes physiopathologiques du syndrome d’hypersensibilite aux antiepileptiques sont discutes. Il s’agirait vraisemblablement d’une accumulation de metabolites aromatiques devenant toxiques, piste etiopathogenique prouvee in vitro par lymphocyte toxicity assay et in vivo par biopsie cutanee (erytheme multiforme ou d’angeite leucocytoclastique typique). D’autres hypotheses plus fragiles ont ete egalement emises (immunoallergique, infection virale). Le syndrome d’hypersensibilite a la lamotrigine (SHAL), de mecanisme encore hypothetique (antiepileptique non aromatique), a ete decrit pour la premiere fois en 1998. Nous en decrivons deux nouveaux cas survenus en add-on therapie avec du phenobarbital et discutons les limites entre SHAL et DRESS a propos d’un troisieme cas exemplaire. Apres revue de la litterature, 14 cas exploitables de SHAL ont ete retrouves. Le SHAL ne surviendrait que dans deux configurations : soit lamotrigine seule mais dans des circonstances atypiques (croissance trop rapide, surdosage, terrain immunodeprime, âges limites), soit lamotrigine en association systematique avec valproate de sodium ou plus rarement phenobarbital.

Objective: The literature on propriospinal myoclonus (PSM) is poor and there are no systematic reviews of the subject. We sought to clarify the spectrum of PSM. Methods: We first prospectively investigated all patients seen in our movement disorders clinic with a firm diagnosis of PSM between 2002 and 2007. All had a standardized interview, detailed clinical examination, laboratory investigations, comprehensive neurophysiologic examination, and spinal cord MRI, including diffusion tensor imaging with fiber tracking (DTI-FT). We also collected drug responses. Finally, we conducted a systematic review of the literature. Results: We enrolled 10 patients meeting the strict criteria for PSM, and also analyzed data on 50 patients from 26 previous reports. PSM occurred predominantly in male and middle-aged patients. The typical clinical picture consisted of myoclonic jerks consistently involving abdominal wall muscles, which worsen in the lying position. A premonitory sensation preceding the jerks and wake-sleep transition phase worsening were frequent. Most patients had a myoclonic generator at the thoracic level, with a myoclonus duration between 200 msec and 2 s. An underlying cause was infrequently found. DTI-FT detected cord abnormalities all of our patients. Conclusion: The clinico-physiologic spectrum of propriospinal myoclonus (PSM) is homogenous. Involvement of the abdominal wall muscles, worsening in the lying position, premonitory sensation, and wake-sleep transition phase worsening are helpful clinical clues. Diffusion tensor imaging with fiber tracking appears more sensitive than conventional MRI for detecting associated microstructural abnormalities of the spinal cord. Symptomatic treatment of PSM is not straightforward, and clonazepam is reported to be the most effective drug. Zonisamide may be an interesting option.

Resume Introduction L’hypotension intracrânienne spontanee du liquide cerebrospinal est definie par une hypovolemie du liquide cephalorachidien survenant en l’absence de toute breche durale connue. Ce syndrome se caracterise cliniquement par des cephalees recidivantes soulagees par le decubitus. Nous rapportons le cas d’un patient de 38 ans atteint de ce syndrome et effectuons une revue de la litterature. Exegese De diagnostic parfois difficile dans les formes atypiques ou anciennes, il est source d’une gene fonctionnelle souvent invalidante et de complications engageant parfois le pronostic vital. Son diagnostic, avant tout clinique, a ete facilite depuis une dizaine d’annees par le developpement de l’imagerie par resonance magnetique et permet la mise en place de traitements efficaces dont le principal est la technique du blood patch. Une fuite de liquide cerebrospinal par une breche de la dure-mere est suspectee a l’origine des symptomes, en l’absence de traumatisme evident comme une ponction lombaire ou un traumatisme medullaire. Conclusion Un interrogatoire precis permet d’en faire rapidement le diagnostic, evitant ainsi le stade des complications.

Damage to the central nervous system induced by treatment of brain tumors is common and impairs the patient quality-of-life. Neurotoxicity is induced by synergistic effects of different cytotoxic treatments such as radiotherapy and chemotherapies administered concurrently or sequentially. Recent progress in the management of brain tumors has led to new neurotoxicities. The growing concern about the neuropsychological performance of patients has disclosed another type of brain damage which has been largely neglected to date. Neurological toxicity can be acute, requiring dose adaptation or a change of drugs. But it also often occurs late and can be irreversible. To date, treatments have been ineffective. The early diagnosis of neurotoxicity is thus a major challenge. Numerous clinical studies suggest an individual sensitivity which is not only related to age or vascular status, but also to genetic predisposition that remains to be detailed. Understanding the mechanisms of personal susceptibilities would be helpful in designing more tailored treatments. In this review we address the question of adverse effects of brain radiation as well as those of chemotherapy protocols which are particularly toxic for the central nervous system that is, methotrexate, platin and aracytin.

Resume Introduction La listeriose se manifeste le plus souvent par une atteinte du systeme nerveux central dont la meningoencephalite et la rhomboencephalite sont les formes les plus frequentes. Les abces cerebraux restent exceptionnels. Observation Un homme âge de 63 ans, sous corticoides au long cours, a ete hospitalise pour des troubles de la vigilance et de la marche dans un contexte febrile. L’examen clinique mit en evidence un syndrome meninge, une hemiparesie droite epargnant la face, une paralysie complete du III gauche et un syndrome de Parinaud pedonculaire. Le scanner cerebral initial etait normal. L’etude du liquide cerebrospinal objectivait une pleiocytose a polynucleaires. Une meningo-rhomboencephalite aigue chez ce sujet immunodeprime etait evoquee. L’origine listerienne etait etayee par la positivite des hemocultures. Une antibiotherapie par amoxicilline et gentamycine fut debutee. Mais l’evolution immediate fut pejorative avec installation d’un coma et d’une tetraparesie. L’imagerie par resonance magnetique revelait un abces de la region pedonculaire gauche s’etendant a la region sous-thalamique. L’evolution clinique fut favorable en six mois, avec amelioration de l’hemiparesie droite, disparition du syndrome de Parinaud, mais persistance de l’atteinte du III gauche. Conclusion Les abces listeriens du tronc cerebral sont exceptionnels. Tout signe neurologique focal febrile chez un patient immunodeprime doit faire evoquer le diagnostic et conduire a la realisation d’hemocultures repetees. Une antibiotherapie precoce par amoxicilline et gentamycine est la condition d’un meilleur pronostic.

Resume Introduction La paramyotonie congenitale d’Eulenburg est une canalopathie de transmission autosomique dominante, liee a des mutations sur le gene codant pour la sous-unite α du canal sodium musculaire. Observation Un homme âge de 38 ans presentait depuis l’enfance des contractures musculaires qui survenaient electivement a l’effort lors d’une exposition au froid. Leur topographie etait cheiro-facio-orale. Elles etaient parfois suivies d’acces de faiblesse musculaire. Plusieurs membres de sa famille presentaient une symptomatologie similaire. L’examen clinique mit en evidence une myotonie mecanique a la percussion musculaire. L’electromyogramme revela des salves myotoniques diffuses. L’analyse de l’ADN a mis en evidence une mutation faux-sens Arg1448Cys a l’etat heterozygote dans l’exon 24 du gene codant pour la sous-unite α du canal sodium musculaire (SCN4A) sur le chromosome 17. Conclusion Cette affection est rarement evolutive. Le traitement repose essentiellement sur des mesures preventives. A visee symptomatique, differentes molecules peuvent etre proposees : des stabilisateurs de membrane comme les antiarythmiques (mexiletine, tocainide), ou l’inhibiteur de l’anhydrase carbonique (acetazolamide). Des precautions sont a prendre en cas d’anesthesie generale compte tenu du risque de crises paralytiques et de myotonie, notamment du diaphragme.

a Service de neurologie, hopital d’instruction des Armees Sainte-Anne, boulevard Sainte-Anne, BP 20545, 83041 Toulon cedex 09, France b Service de neurochirurgie, hopital d’instruction des Armees Sainte-Anne, boulevard Sainte-Anne, BP 20545, 83041 Toulon cedex 09, France c Service de radiologie, hopital d’instruction des Armees Sainte-Anne, boulevard Sainte-Anne, BP 20545, 83041 Toulon cedex 09, France

Un marin de 39 ans sans antecedent a consulte pour des cephalees inhabituelles installees progressivement depuis un mois suite a une chute banale. Ces cephalees severes apparaissaient uniquement en station debout, s’associaient parfois a des acouphenes et des sensations de flous visuels et cedaient quasi immediatement au passage en decubitus. L’examen clinique etait strictement normal. L’IRM cerebrale (figure 1)montrait une prise de contraste et un epaississement diffus des meninges avec un aplatissement des ventricules et une ptose des amygdales cerebelleuses et permit de faire le diagnostic d’hypotension intracrânienne spontanee (HICS) du liquide cephalo-rachidien (LCR). L’IRM medullaire ne montrait pas de breche meningee. Le patient a ete traite par blood-patch epidural qui fit ceder immediatement les cephalees. Un mois plus tard, les cephalees reapparurent et un second blood-patch a ete realise et permit de faire ceder definitivement les symptomes. Commentaires

Idiopathic intracranial hypertension is a rare disorder characterized by elevated intracranial pressure without hydrocephaly or intracranial process. Its mechanism is poorly understood. Most cases of benign intracranial hypertension are presumed to be idiopathic but some of them may be related to some treatment. We report a 26-year-old female with benign intracranial hypertension due to tetracycline, revealed by headaches and gradual visual loss. Standard investigations were unremarkable and favourable outcome after therapeutic lumbar puncture confirmed the diagnosis.

Migrainous infarction is a rare ischemic stroke developing during an attack of migraine, which is more prevalent in young woman and preferentially affects the posterior cerebral circulation. The case of a migrainous 35-old patient with patent foramen ovale admitted in neurology department for a typical migrainous infarction is reported and compared with a literature review. Furthermore, this case report highlights the existence of complex epidemiologic and physiopathologic links between cerebral ischemia, migraine and patent foramen ovale.

Since 2003, intravenous thrombolysis with rt-PA for stroke victims has been largely developed in the military hospital of Toulon. We report the results of our practice and compare them with the literature. We also sought to identify predictive factors of favorable outcome after thrombolysis.All patients treated with rt-PA for a stroke in the carotid territory between September 2003 and June 2009 were prospectively included. Disability was assessed at 3 months with the modified Rankin Scale (m-RS); outcome was considered unfavorable if m-RS score was above 2. Multivariate analysis was then performed to identify parameters correlating with poor and favorable outcome at 3 months follow-up.One hundred and one patients were included in this study (mean initial National Institute of Health Stroke Scale [NIHSS]: 15.2). 53.4% had a Rankin score higher than 2 at 3 months follow-up. The absence of diabetes mellitus, low NIHSS score on admission, short time from stroke onset to treatment, and prior statin use were identified as independent predictive factors of favorable functional outcome.After 6 years of activity, our stroke unit has results that appear similar to those of the French and international trials in terms of safety and efficacy. Efficacy of rt-PA in our series is poor for strokes caused by large-vessel atherothrombotic changes and cervical artery dissection due to high incidence of internal carotid thrombosis in these cases. Our studies also suggest that prior statin use may be an independent predictive factor of favorable outcome after thrombolysis.

Interferon-alpha associated retinopathy is an ocular complication of hepatitis C treatment well established in the literature. But, there are far fewer reports on multiple sclerosis related interferon-beta retinopathy.A 58-year-old male while receiving subcutaneous interferon-beta 1a 44microg thrice a week since 2001 for multiple sclerosis developed blurred vision. Visual acuity remained stable throughout the course of surveillance. Cotton wool spots were found on fundus exam. The retinopathy disappeared without specific therapy 2 months after discontinuing interferon injections. The diagnosis of interferon-beta 1a retinopathy was retained due to the lack of any other etiology.An ophthalmological examination including a fundus examination to search for a retinopathy should be undertaken when new ocular symptoms develop in a multiple sclerosis patient receiving interferon. An adverse event linked to interferon can be discussed and favored if the retinopathy resolves after interferon withdrawal.

Idiopathic intracranial hypertension is a rare disorder characterized by elevated intracranial pressure without hydrocephaly or intracranial process. Its mechanism is poorly understood. Most cases of benign intracranial hypertension are presumed to be idiopathic but some of them may be related to some treatment. We report a 26-year-old female with benign intracranial hypertension due to tetracycline, revealed by headaches and gradual visual loss. Standard investigations were unremarkable and favourable outcome after therapeutic lumbar puncture confirmed the diagnosis.

Anticonvulsant hypersensitivity syndrome (AHS) is defined by the association of high fever, cutaneous rash and multiorgan-system abnormalities (incidence, one in 1000 to one in 10,000 exposures). Fatal complications are described in 10%. This reaction usually develops 1 to 12 weeks after initiation of an aromatic anticonvulsant. Drug rash with eosinophilia and systemic symptoms (DRESS) can be discussed as differential diagnosis. Several hypotheses have been put forward to explain the pathogenesis of AHS. These include accumulation of toxic metabolites, antibody production and viral infection. The one based on toxic metabolites has found the greatest acceptance due to the fact that it can be proven by an in vitro test, the lymphocyte toxicity assay. In vivo, skin biopsies show characteristic findings of erythema multiform or typical leucocytoclastic angitis. The patch-test is positive in 80% of the cases. Lamotrigine-associated anticonvulsant hypersensitivity syndrome (LASH) is rare and was described in 1998. We report two new cases demonstrating the two particular configurations of apparition of LASH found in the 14 cases from the review of literature (Pubmed: anticonvulsant hypersensitivity syndrome - lamotrigine): high doses of lamotrigine (or lamotrigine in very young or old patients), and lamotrigine associated with another anti-epileptic (phenobarbital or sodium valproate). We discuss the links between DRESS after lamotrigine and LASH as illustrated in a new case.

Damage to the central nervous system induced by treatment of brain tumors is common and impairs the patient quality-of-life. Neurotoxicity is induced by synergistic effects of different cytotoxic treatments such as radiotherapy and chemotherapies administered concurrently or sequentially. Recent progress in the management of brain tumors has led to new neurotoxicities. The growing concern about the neuropsychological performance of patients has disclosed another type of brain damage which has been largely neglected to date. Neurological toxicity can be acute, requiring dose adaptation or a change of drugs. But it also often occurs late and can be irreversible. To date, treatments have been ineffective. The early diagnosis of neurotoxicity is thus a major challenge. Numerous clinical studies suggest an individual sensitivity which is not only related to age or vascular status, but also to genetic predisposition that remains to be detailed. Understanding the mechanisms of personal susceptibilities would be helpful in designing more tailored treatments. In this review we address the question of adverse effects of brain radiation as well as those of chemotherapy protocols which are particularly toxic for the central nervous system that is, methotrexate, platin and aracytin.

Paramyotonia congenita is an autosomal dominant sodium channelopathy, caused by mutations in gene coding for muscle voltage-gated sodium channel alpha subunit.We report the case of a 38-year-old man who described since childhood muscle stiffness with attacks ok weakness induced by two provocative stimuli: cold exposure and exercise. It primarily concerned eyelids and hands, occasionally limbs. Family history suggested an autosomal dominant mode of transmission. Clinical examination revealed myotonia at the thenar eminence percussion. Generalized myotonic discharges were observed on electromyography. Molecular diagnosis reported an Arg1448Cys mutation in exon 24 in gene coding for muscle voltage-gated sodium channel alpha subunit (SCN4A) in chromosome 17.Paramyotonia congenita is not evolutive. Treatment is essentially preventive. Some medications could be proposed: membrane stabilizing agents like antiarrhythmic drugs (mexiletine, tocainide), or the carbonic anhydrase inhibitor (acetazolamide). Precautions may be taken during general anaesthesia because of diaphragm myotonia risk.

Spontaneous low cerebrospinal fluid pressure syndrome is a spontaneous intracranial hypotension pressure due to a cerebrospinal fluid leak without any known dural effraction. It is clinically characterised by postural headaches relieved by supine position. We report a 38-year-old patient with this syndrome and review the literature.The diagnosis is sometimes difficult in atypical presentation of the syndrome and can lead to incapacitating chronic headache and rarely to complications. Cerebral magnetic resonance imaging has dramatically improved identification, diagnosis and management of this syndrome. Treatment is mainly based on blood patch realisation. Cerebrospinal fluid leak probably due to a spontaneous defect in the dural mater is suspected to be the main mechanism of this syndrome without any history of lumbar puncture or penetrating trauma.Early diagnosis, often easy on the basis of clinical characteristics of the headache may avoid complications.

Listeriosis commonly involves the central nervous system. Meningoencephalitis and rhomboencephalitis are the most frequent manifestations. Brain abscesses are rare.We report the case of a 63-year-old man treated with steroids for a long period; he was hospitalized for hemiparesis, confusion and fever. Clinical examination revealed meningeal signs, right hemiparesis and Parinaud syndrome. Initial CT scan was normal. The CSF contained 520 white cells/mm3 with predominance of polymorphonuclear neutrophils. An acute meningo- rhombencephalitis in an immunodepressed patient was suggested. The diagnosis of listeriosis was confirmed by blood cultures. Amoxicillin and gentamycin were started. The outcome on day 4 was severe with coma and tetraparesis. Brain MRI revealed a left peduncle abscess which descended deep into the brain reaching the internal capsule. The final clinical outcome involved residual right hemiparesis and left oculomotor nerve (III) palsy.Brain stem abscess is an uncommon form of listerial central nervous system infection. Listeria monocytogenes infection should be considered in patients with altered cell-mediated immunity that develop local neurologic deficits, a diagnosis which pursued rapidly with repeated blood cultures. Successful treatment requires early antibiotic therapy with ampicillin and gentamycin.

Introduction La fatigue est omnipresente dans la SEP, souvent en l’absence de handicap important. Objectifs Reveler des marqueurs objectifs quantitatifs de la fatigue permettant d’aider le clinicien et d’approfondir la comprehension physiopathologique de la fatigue dans la SEP. Methodes Vingt-six patients, a distance d’une poussee, ont evalue leur fatigue par l’echelle EMIF-SEP puis ont ete soumis (1) a la mesure de variables refletant la fatigue chez le sujet sain surentraine, telles que la variabilite de la frequence cardiaque (VFC) au changement de posture, la force musculaire des membres superieurs et des dosages de marqueurs salivaires ou sanguins, et (2) a la mesure de variables plus specifiques de la SEP telles que les taux sanguins de cytokines et la baisse de performance au test cognitif PASAT. Resultats Un tiers des patients s’est evalue fatigue (score EMIF total ≥ 79/104). La frequence cardiaque matinale et la VFC etaient correlees aux scores EMIF. Le profil des autres marqueurs du surentrainement ne permettait pas de caracteriser la fatigue des patients. Les taux sanguins de TNFα et ADH etaient correles a la fatigue physique des patients. Enfin la fatigabilite revelee par le test PASAT n’etait correlee qu’a la fatigue sociale de l’EMIF et ne rendait pas compte de la plainte dans sa globalite. Discussion L’etude confirme l’intervention d’un dysfonctionnement neuro-vegetatif dans la fatigue observee dans la SEP qui differe de celui lie au surentrainement. De plus, l’individualisation de marqueurs inflammatoires objectifs tel que le TNFα sanguin suggere que le controle de la regulation immune peut influencer le symptome fatigue. Enfin, la notion de fatigabilite demasquee par le PASAT ne reflete pas la fatigue ressentie par les patients. Conclusion Il existe un pattern biologique specifique a la fatigue dans la SEP. La plainte de fatigue ne correspond pas a la mesure objective de la fatigabilite. La reponse immune pourrait etre en lien avec la fatigue ressentie.