Sciatic paralysis following uterine artery embolization

articleInternational audienceEarly postpartum bleeding remains in France the leading cause of maternal mortality in perinatal period. In association with obstetrical and medical measures to control bleeding, uterine arteries embolization constitutes an efficient non-surgical measure whose potential side effects must be kept in mind. We report the case of a patient that presented a popliteal sciatic paralysis in the hours following the procedure. Through this case, we will review the different types of embolization complications

Diffusion-weighted magnetic resonance imaging in Marchiafava-Bignami disease: follow-up studies

articleMarchiafava-Bignami disease (MBD), an acute toxic demyelination of the corpus callosum in alcoholics, is associated with poor evolution in the majority of patients. We report here the early and late diffusion magnetic resonance imaging (MRI) and apparent diffusion coefficient (ADC) studies of two patients suffering from MBD with favourable outcome. Diffusion and anatomical MRI changes were parallel to the clinical evolution, suggesting that MRI studies can be helpful for diagnosis and follow-up. Unlike in stroke, restricted diffusion on ADC maps does not seem to be a sign of irreversibility

Mononeuropathie multiple dans le cadre d’une forme sévère d’infection par le virus H1N1

Introduction : Les atteintes neurologiques périphériques liées aux virus de la grippe saisonnière sont bien connues. Par contre durant la pandémie de la grippe A H1N1, les atteintes périphériques ont été très peu rapportées. Nous rapportons un cas d’atteinte périphérique plurifocale concomitante d’une infection sévère par le virus H1N1.Observation : Il s’agit d’une patiente de 42 ans hospitalisée pour une infection à grippe A H1N1 compliquée d’une pneumopathie hypoxémiante ayant nécessité un transfert en réanimation. A son réveil, il a été constaté une tétraparésie et une atteinte multiple des paires crâniennes. L’ENMG avait noté une mononévrite multiple axonale touchant aussi le nerf médian gauche, sans anomalies plus diffuses comme on l’aurait observé dans un processus inflammatoire démyélinisant. Il n’avait pas été documenté d’autre étiologie, notamment infectieuse, ni de carence vitaminique. L’étiologie infectieuse liée au virus H1N1 était donc la plus probable. Les différents contrôles ENMG à 3 et 9 mois ont noté qu’Il n’y avait plus d’atteinte neurogène très significative dans les muscles dépendant du médian.Discussion : bien que l’imputabilité de l’atteinte périphérique au virus H1N1 soit difficile, l’absence d’autres causes potentielles suggère que la neuropathie périphérique soit très probablement due au virus H1N1.Conclusion : Cette observation suggère que l’infection à H1N1 peut se compliquer d’atteintes neurologiques périphériques touchant même les paires crâniennes.Mots clés : H1N1, Mononeuropathie multiple, EMNG.English AbstractMononeuropathy multiplex in a severe h1n1 infection We present a case of a 42 years old woman who presents a mononeuropathy multiplex during severe acute H1N1 infection. She was admitted because of flu complicated of pneumopahy with hypoxemia. She was transfer in the intensive word. After intensive care she presents tetraparesia with cranial nerves attempt. The nerve conduction and the electromyography showed mononeuropathy multiplex of cranial nerves (left IX, X, XI and XII) and the left median. The H1N1 serology was positive. The study of the cerebral fluid was normal. The HIV, HBV and HBC serologies were negative. The Lyme disease test was negative. The control of nerve conduction and the electromyography at 3 and 9 month chow a good outcome. Even though it is difficult to attribute this mononeuropathy of the cranial nerves and the left median to the H1N1 infection, there was any other cause explaining this peripheral neuropathy. This case report suggests that mononeuropthy multiplex with cranial nerves attempt can complicate a H1N1 infectionKeywords: H1N1, mononeuropathy multiplex, electromyograph

An exploratory randomised double-blind and placebo-controlled phase 2 study of a combination of baclofen, naltrexone and sorbitol (PXT3003) in patients with Charcot-Marie-Tooth disease type 1A

BACKGROUND: Charcot-Marie-Tooth type 1A disease (CMT1A) is a rare orphan inherited neuropathy caused by an autosomal dominant duplication of a gene encoding for the structural myelin protein PMP22, which induces abnormal Schwann cell differentiation and dysmyelination, eventually leading to axonal suffering then loss and muscle wasting. We favour the idea that diseases can be more efficiently treated when targeting multiple disease-relevant pathways. In CMT1A patients, we therefore tested the potential of PXT3003, a low-dose combination of three already approved compounds (baclofen, naltrexone and sorbitol). Our study conceptually builds on preclinical experiments highlighting a pleiotropic mechanism of action that includes downregulation of PMP22. The primary objective was to assess safety and tolerability of PXT3003. The secondary objective aimed at an exploratory analysis of efficacy of PXT3003 in CMT1A, to be used for designing next clinical development stages (Phase 2b/3). METHODS: 80 adult patients with mild-to-moderate CMT1A received in double-blind for 1 year Placebo or one of the three increasing doses of PXT3003 tested, in four equal groups. Safety and tolerability were assessed with the incidence of related adverse events. Efficacy was assessed using the Charcot-Marie-Tooth Neuropathy Score (CMTNS) and the Overall Neuropathy Limitations Scale (ONLS) as main endpoints, as well as various clinical and electrophysiological outcomes. RESULTS: This trial confirmed the safety and tolerability of PXT3003. The highest dose (HD) showed consistent evidence of improvement beyond stabilization. CMTNS and ONLS, with a significant improvement of respectively of 8% (0.4% - 16.2%) and 12.1% (2% - 23.2%) in the HD group versus the pool of all other groups, appear to be the most sensitive clinical endpoints to treatment despite their quasi-stability over one year under Placebo. Patients who did not deteriorate over one year were significantly more frequent in the HD group. CONCLUSIONS: These results confirm that PXT3003 deserves further investigation in adults and could greatly benefit CMT1A-diagnosed children, usually less affected than adults. TRIAL REGISTRATION: EudraCT Number: 2010-023097-40. ClinicalTrials.gov Identifier: NCT01401257. The Committee for Orphan Medicinal Products issued in February 2014 a positive opinion on the application for orphan designation for PXT3003 (EMA/OD/193/13)