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Resume Nous rapportons le cas d’un patient de 68 ans presentant une encephalite a anticorps anti-Ma2 responsable d’une narcolepsie secondaire. Le patient presentait une somnolence diurne excessive persistante malgre la ventilation nocturne en pression positive continue dans le cadre de son syndrome d’apnees obstructives du sommeil severe. L’IRM cerebrale etait evocatrice d’encephalite a anticorps anti-Ma2, qui etaient effectivement retrouves dans le sang et le LCS, avec des hypersignaux FLAIR autour du 3e ventricule et de l’aqueduc du mesencephale. Les tests iteratifs de latence d’endormissement (TILE) etaient pathologiques, avec une latence moyenne d’endormissement de 6,2 min (normale > 8 min) mais sans endormissement en sommeil paradoxal. En revanche, le taux d’hypocretine effondre dans le liquide cerebro-spinal permettait de porter le diagnostic de narcolepsie de type 1.

Resume Nous rapportons deux cas originaux de myoclonies corticales d’apparition subaigue et d’origine probablement auto-immune — et paraneoplasique dans l’un des cas — bien qu’aucun auto-anticorps n’ait ete identifie. L’IRM cerebrale etait normale dans les deux cas mais la tomographie par emission de positron (TEP)-scanner cerebral montrait un hypermetabolisme inhabituel du cervelet, rarement rapporte dans la litterature. L’aggravation dans un cas sous traitement inhibiteur de point de controle immunitaire (« immune checkpoint inhibitor ») ainsi que l’amelioration clinique des deux patients sous traitement immunosuppresseur nous conforte dans l’hypothese d’une origine auto-immune. En nous basant sur ces deux cas, nous discutons les differentes etiologies d’atteintes cerebelleuses tardives en axant la discussion sur les atteintes auto-immunes, encore imparfaitement comprises pour la plupart et au sein desquelles de nouvelles entites cliniques emergent comme l’ataxie cerebelleuse auto-immune primitive.

We report three cases of vermian cerebellar hypermetabolism in patients with autoimmune encephalitis. One of our patients was positive for anti-Ma2 antibodies and one for anti-Zic4 antibodies while the remaining patient did not present any known antibodies. The seronegative patient deteriorated after immune checkpoint inhibitor treatment for a pulmonary adenocarcinoma and improved with immunosuppressive drugs, which is in favour of an underlying autoimmune mechanism. They all presented with subacute neurological symptoms. Brain magnetic resonance imaging was normal except in one patient, where hyperintensities were present on FLAIR sequence around the third ventricle and the cerebral aqueduct. 18F-FDG brain positron emission tomography with computed tomography (18F-FDG PET-CT) demonstrated an unusual vermian cerebellar hypermetabolism in the three cases. While cerebellar hypermetabolism on 18F-FDG PET-CT has been described in various neurological diseases, such vermian – and more broadly cerebellar – hypermetabolism was seldom described in previous studies on autoimmune encephalitis. When differential diagnoses have been ruled out, this pattern may be of interest for the positive diagnosis of autoimmune encephalitis in difficult diagnostic cases.

Radiation-induced leukoencephalopathy (RIL) is the most threatening delayed complication of cerebral radiotherapy (RT) and remains roughly defined by cognitive dysfunction associated with diffuse FLAIR MRI white matter hyperintensities after brain irradiation. We documented clinical, neuropsychological, and radiological aspects of RI in order to refine diagnostic criteria. Patients referred to our center for deterioration in cognitive complaint at least 6 months after completing a focal or whole brain RT underwent a systematic cross-sectional assessment including clinical examination, neuropsychological tests, and a standardized MRI protocol. Patients with progressive tumor were excluded. Forty patients were prospectively enrolled. Of these, 26 had received a focal RT, median dose of 53 Gy (range 50 to 60), and 14 had received a whole brain RT, median dose of 30 Gy. Cognitive complaints, gait apraxia, and urinary troubles were reported in 100, 67, and 38% of cases, respectively. On neuropsychological examination, patients displayed a global and severe cognitive decline through a subcortical frontal mode. The cognitive changes observed were not hippocampic, but related to executive dysfunction. On MRI, 68% of the patients had extensive FLAIR hyperintensities with anterior predominance, 87% had brain atrophy, and 21% had intraparenchymal cysts. T2*-weighted MRI showed small asignal areas in 53% of the patients. These abnormalities are evocative of cerebral small vessel disease. Fractional anisotropy in the corpus callosum correlated with the cognitive evaluation. No differentiation in terms of cognitive and MRI features could be made between patients treated with focal brain RT (glioma) and patients treated with WBRT (for brain metastases or PCNSL). RIL can be defined by clinical symptoms (subcortical frontal decline, gait apraxia, urinary incontinence) and MRI criteria (cortico-subcortical atrophy, spread FLAIR HI, T2* asignals). This condition mimics a diffuse progressive cerebral small vessel disease triggered by RT, independent of RT protocol.

Purpose This phase I study investigated bortezomib in solid tumors used as a daily subcutaneous regimen. Previous regimens showed only modest activity in solid tumors which was potentially related to sub-optimal tumor penetration. We aimed at exploring if daily low dose administration of bortezomib may allow a greater and tolerable pharmacokinetic exposure which might be required for antitumor activity in solid tumors. Patients and methods This 3 + 3 design, dose escalation, monocentric study aimed at defining the maximum tolerated dose of daily low dose schedule of bortezomib. Tolerability, pharmacokinetics, pharmacodynamics, antitumor activity, biomarkers for proteasome inhibition, pre- and post-treatment tumor biopsies were also evaluated. Results A total of eighteen patients were dosed in 3 bortezomib cohorts (0.5, 0.6 and 0.7 mg/m2), with 3, 11 and 4 patients respectively. Three patients experienced dose-limiting toxicities: Grade (G) 3 Sweet's syndrome (at 0.6 mg/m2), G3 asthenia and anorexia or ataxia (2 patients at 0.7 mg/m2). The most common study drug-related adverse events (all grades) were thrombocytopenia (72%), fatigue (56%), neuropathy (50%), anorexia (44%) and rash (39%). Dose 0.6 mg/m2 of bortezomib was considered as the recommended phase II dose. A significant tumor shrinkage (-36% according to WHO criteria) was observed in one patient with heavily pre-treated GIST, and 2 minor responses (-20%) were recorded in two patients with melanoma and mesothelioma. Conclusion This daily subcutaneous regimen of bortezomib showed a dose dependent plasma exposure, evidence of target inhibition and preliminary signs of clinical activity. However, cumulative neurological toxicity of this dose-dense daily regimen might preclude its further clinical development.

Background: Obstetrical analgesia remains a matter of controversy because of the fear of neurotoxicity of local anesthetics on demyelinated fibers or their potential relationship with subsequent relapses. Objective: To assess the impact of neuraxial analgesia on the risk of relapse during the first 3 months post-partum, with a focus on women who experienced relapses during pregnancy. Methods: We analyzed data of women followed-up prospectively during their pregnancies and at least 3 months post-partum, collected in the Pregnancy in Multiple Sclerosis (PRIMS) and Prevention of Post-Partum Relapses with Progestin and Estradiol in Multiple Sclerosis (POPARTMUS) studies between 1992–1995 and 2005–2012, respectively. The association of neuraxial analgesia with the occurrence of a post-partum relapse was estimated by logistic regression analysis. Results: A total of 389 women were included, 215 from PRIMS and 174 from POPARTMUS. In total, 156 women (40%) had neuraxial analgesia. Overall, 24% experienced a relapse during pregnancy and 25% in the 3 months post-partum. Women with a pregnancy relapse were more likely to have a post-partum relapse (odds ratio (OR) = 1.83, p = 0.02), independently of the use of neuraxial analgesia. There was no association between neuraxial analgesia and post-partum relapse (OR = 1.08, p = 0.78). Conclusion: Neuraxial analgesia was not associated with an increased risk of post-partum relapses, whatever multiple sclerosis (MS) activity during pregnancy.

Chemotherapy-induced peripheral neurotoxicity (CIPN) is a common, potentially severe and dose-limiting adverse effect; however, it is poorly investigated at an early stage due to the lack of a simple assessment tool. As sweat glands are innervated by small autonomic C-fibers, sudomotor function testing has been suggested for early screening of peripheral neuropathy. This study aimed to evaluate Sudoscan, a non-invasive and quantitative method to assess sudomotor function, in the detection and follow-up of CIPN. Eighty-eight patients receiving at least two infusions of Oxaliplatin only (45.4%), Paclitaxel only (14.8%), another drug only (28.4%) or two drugs (11.4%) were enrolled in the study. At each chemotherapy infusion the accumulated dose of chemotherapy was calculated and the Total Neuropathy Score clinical version (TNSc) was carried out. Small fiber neuropathy was assessed using Sudoscan (a 3-min test). The device measures the Electrochemical Skin Conductance (ESC) of the hands and feet expressed in microSiemens (µS). For patients receiving Oxaliplatin mean hands ESC changed from 73 ± 2 to 63 ± 2 and feet ESC from 77 ± 2 to 66 ± 3 µS (p < 0.001) while TNSc changed from 2.9 ± 0.5 to 4.3 ± 0.4. Similar results were observed in patients receiving Paclitaxel or another neurotoxic chemotherapy. During the follow-up, ESC values of both hands and feet with a corresponding TNSc < 2 were 70 ± 2 and 73 ± 2 µS respectively while they were 59 ± 1.4 and 64 ± 1.5 µS with a corresponding TNSc ≥ 6 (p < 0.0001 and p = 0.0003 respectively). This preliminary study suggests that small fiber neuropathy could be screened and followed using Sudoscan in patients receiving chemotherapy.

International audience; BACKGROUND AND OBJECTIVES:The symptomatic status of unruptured aneurysms has to be looked for. The objective of this retrospective case-control study was to identify the headache semiologic characteristics of symptomatic aneurysms during the 3 months prior to patient admission.PATIENTS AND METHODS:The case cohort was composed of 40 consecutive patients admitted for the treatment of a ruptured intracranial aneurysm (IA) and able to answer a standardized questionnaire by the same neurologist. This cohort was matched with a control cohort of 40 patients operated on for a degenerative lumbar pathology. This questionnaire, using the criteria for headache characteristics according to the International Headache Society (IHS) enabled us to classify headaches during the previous 3 months prior to the rupture (study period) and during the year prior to the period studied (reference period) in both cohorts. Headache status was considered as unstable if there were modifications of semiologic headache characteristics, thunderclap headaches or de novo headaches, or on the contrary stable.RESULTS:During the status period, chronic headaches were reported by 31 patients (77.5%) in the studied cohort and 35 (87.5%) in the control cohort. During the study period, the cephalagia status was stable in 19 patients (47.5%) versus 35 patients (87.5%) in the control cohort (P

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.

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.

Resume Introduction Les accidents thromboemboliques sont des complications severes des maladies inflammatoires chroniques de l'intestin (MICI). Exegese Nous rapportons l'observation d'un patient âge de 18 ans, aux antecedents de rectocolite hemorragique evoluant depuis plus d'un an, hospitalise pour une thrombose veineuse cerebrale survenue durant la decroissance de la corticotherapie a l'issue d'une poussee. Sous traitement anticoagulant, les troubles neurologiques se sont rapidement ameliores. Aucune thrombophilie constitutionnelle n'etait retrouvee. Conclusion Le role des facteurs de risque acquis et constitutionnels, dans la genese des complications thromboemboliques au cours des MICI, est discute.

Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal-dominant peripheral neuropathy characterized by recurrent isolated nerve palsies, which are precipitated by trivial compression and trauma. Although HNPP has been well-described in literature, it often goes unrecognized. We report a case of HNPP occurring during military training to promote recognition and proper management of this entity.

BACKGROUND: Currently, the most common and serious delayed complication of cerebral RT is radiation-induced leukoencephalopathy (RIL), roughly defined, in absence of strictly consensual criteria, by cognitive dysfunctions associated with diffuse FLAIR MRI white matter hyperintensity (HI). The objective of our study is to clearly define the RIL criteria. METHODS: Patients, addressed to our center between June 2010 and June 2012, with medical history of focal or whole brain RT who developed a persistent cognitive complain at least six months after the end of the brain RT, and presenting brain MRI FLAIR abnormalities were eligible. There was a clinical evaluation by a neurologist and a neuropsychological assessment (Edinburgh scale for lateralization, educational level, Mattis dementia rating scale (DRS) and Folsteins'MMSE, Grober and Buschke memory evaluation, forward and backward digit span, phonemic and semantic verbal fluencies, DO80). All patients were scanned by a standardized MRI protocol. White matter FLAIR lesions were evaluated with a 1 to 14 points score according to a modified Scheltens rating scale. Brain atrophy was visually assessed. Consensually defined signs of small vessels diseases (CSVD) were assessed for each patient. RESULT: Fourty patients were enrolled in our study (mostly stage 3 gliomas (27.5%)). The mean fraction dose was 2 Gy (range 1,5-3) and a mean total dose of 49 Gy (range 30-60). Most patient had gait difficulties, urinary trouble and cognitive complain. Memory difficulties were reported by 77% of patients. Neuropsychological assessment showed global and severe cognitive decline. Mattis DRS was abnormal in 75% of the patients. Forward and backward digit span were pathological in 80% patients. On brain MRI, an extensive FLAIR HI with anterior predominance was shown in 68,5% patients. Brain atrophy was present in 87% cases. T2*-weighted- MRI showed hypointensities like “black dots” probably corresponding to microbleeds or radiation induced cavernomas in 52,6% patients. We found no recent small cortical infarct. We found no correlation between FLAIR HI extension, brain atrophy, microbleeds numbers or location, perivascular spaces number, lacunes of presumed vascular origin and the cognitive compound score. We found no correlation between the delay from RT and one of the MRI lesions described above. The patients displayed a loss of executive functions responsible of severe cognitive disability and dementia as previously described in patients with CSVD. Our study give strong clinical and MRI arguments to consider that the RIL is a delayed progressive CSVD, with characteristic evolution and distribution pattern namely implicating distal brain superficial perforating arterioles that serve dorsolateral loops of white matter in the corona radiata and sparing basal ganglia perforating arterioles that spring from the cerebral medial artery.

The Guillain-Barre syndrome (GBS) is an acute inflammatory polyradiculoneuritis. The pathophysiology remains unknown but the existence of triggering factors such as external antigens is regularly suspected. We report the case of a 30-year-old patient with a past medical history of Graves disease, who presented with GBS within the month after receiving an anti-hepatitis A vaccination. GBS rarely happens after a hepatitis A vaccination. However, the responsibility of this vaccine should be considered in the clinical presentation of an acute polyradiculoneuritis.

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.

Resume Introduction Les manifestations neurologiques dues a l’ergotisme chronique sont rares. Observation Nous rapportons le cas d’une patiente de 40 ans ayant presente des signes cliniques de souffrance medullaire ainsi qu’une mononeuropathie des nerfs sciatique poplite interne et externe droits apres consommation de methysergide. L’evolution fut favorable sous inhibiteurs calciques. Conclusion L’ergotisme doit etre envisage devant toute manifestation clinique inhabituelle chez tout patient traite par derives ergotes.

Purpose Ipilimumab is a T-cell-potentiating monoclonal antibody directed against cytotoxic T-lymphocyte antigen-4 (CTLA-4) to promote antitumoural immunity. In phase III trials, ipilimumab was shown to be the first agent to improve survival in advanced melanoma patients, regardless of previous treatment. We report a case of severe neurologic disease after ipilimumab treatment. Patient and methods Neurologic symptoms including facial diplegia, tetraplegia, areflexia progressed with time a few days after the fourth monthly ipilimumab infusion. Analysis of the cerebro-spinal fluid showed elevated proteinorachy and lymphocytic meningitis. Despite high doses of steroids and symptomatic treatment, the symptoms worsened. Results Veinoglobulins were then infused and the patient began to improve and recovered almost normal activity two years later. Conclusion The adverse event profile associated with ipilimumab was primarily immune-related. This is the first case in which such a severe event has been reported.

The Guillain-Barré syndrome (GBS) is an acute inflammatory polyradiculoneuritis. The pathophysiology remains unknown but the existence of triggering factors such as external antigens is regularly suspected. We report the case of a 30-year-old patient with a past medical history of Graves disease, who presented with GBS within the month after receiving an anti-hepatitis A vaccination. GBS rarely happens after a hepatitis A vaccination. However, the responsibility of this vaccine should be considered in the clinical presentation of an acute polyradiculoneuritis.

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.

Thromboembolic events are serious complications in patients with inflammatory bowel disease.An 18-year-old patient, with a one year history of ulcerative colitis, presented with cerebral venous thrombosis during the decreasing period of corticotherapy after an active phase of the disease. Under treatment, the neurological disorder rapidly improved. No inherited thrombophilia was found.The role of acquired and inherited risks factors is discussed.

Neuropathologic manifestations due to chronic ergotism are rare.We report the case of a 40-year-old patient who presented clinical signs and symptoms of a spinal lesion and also the symptoms of neuropathy involving the right sciatic nerve, more precisely the internal and external popliteal nerves, following ingestion of methysergide. Complete recovery was achieved with calcium blocker treatment.Ergotism should be considered in patients treated by ergot alkaloids presenting an atypical clinical manifestations.