Bioavailable Trace Metals in Neurological Diseases

Medical treatment in Wilson’s disease includes chelators (d-penicillamine and trientine) or zinc salts that have to be maintain all the lifelong. This pharmacological treatment is categorised into two phases; the first being a de-coppering phase and the second a maintenance one. The best therapeutic approach remains controversial, as only a few non-controlled trials have compared these treatments. During the initial phase, progressive increase of chelators’ doses adjusted to exchangeable copper and urinary copper might help to avoid neurological deterioration. Liver transplantation is indicated in acute fulminant liver failure and decompensated cirrhosis; in cases of neurologic deterioration, it must be individually discussed. During the maintenance phase, the most important challenge is to obtain a good adherence to lifelong medical therapy. Neurodegenerative diseases that lead to a mislocalisation of iron can be caused by a culmination of localised overload (pro-oxidant siderosis) and localised deficiency (metabolic distress). A new therapeutic concept with conservative iron chelation rescues iron-overloaded neurons by scavenging labile iron and, by delivering this chelated metal to endogenous apo-transferrin, allows iron redistribution to avoid systemic loss of iron

Nouveaux circuits intégrés MOS analogiques et traitement du signal

Les récentes acquisitions de la technologie MOS offrent la possibilité de réaliser des retards purs analogiques ainsi que de mémoriser,pour de très longues durées, des signaux analogiques. Ces deux fonctions permettent d'envisager l'intégration sur une même puces, de micro calculateurs calculant, par exemple, une fonction de convolution discrète de corrélation, ou de transformée de Fourier discrète. A l'heure actuelle, nous étudions l'intégration d'un filtre non récursif. Ce dispositif n'en est qu'aux premiers stades de l'étude en laboratoire

Risque périnéal et mesures de protection obstétricale : enquête auprès des professionnels de la naissance

International audienc

Republication de : Risque périnéal et mesures de protection obstétricale : enquête auprès des professionnels de la naissance

International audienceObjectivesThe main objective was to evaluate the practices declared by birth professionals in the Lorraine region about perineal obstetric protection (any perineal tear combined). The secondary objective was to evaluate prevention measures performed in practice by birth professionals according to the perineal risk subjectively estimated for each obstetric situation.MethodsThis is a practice survey conducted through an anonymous questionnaire distributed to birth professionals (gynecologists, interns, midwife and midwife student) in October 2016. Topics covered concerned maternal, obstetric and fetal risk factors associated with perineal (1st to 4th degrees) lesions and the associated protective measures. A descriptive analysis of the data collected was conducted.ResultsOne hundred and five professionals answered the questionnaire. The identified risk factors were consistent with those highlighted in the literature. Other factors, not known as associated with perineal risk, were cited by professionals (smoking, phototype). If the professional perceived a significant perineal risk, they more frequently practiced an episiotomy (15% vs. 0%, P < 0.001) or considered that the delivery should be performed by an obstetrician (34% vs 8%, P < 0.001).ConclusionThis evaluation shows that birth professionals know the main risk factors for perineal injury. On the other hand, they easily use perineal protection maneuvers (episiotomy for example) without real demonstrated effectiveness.ObjectifsL’objectif de notre étude était de réaliser une évaluation des pratiques déclarées par les professionnels de la naissance de la région Lorraine à propos de la protection périnéale obstétricale (toute déchirure périnéale confondue). L’objectif secondaire était d’évaluer les mesures de prévention réalisées en pratique par les professionnels de la naissance en fonction du risque périnéal subjectivement estimé pour chaque situation obstétricale.MéthodesIl s’agit d’une enquête de pratique réalisée grâce à un questionnaire anonyme distribué aux professionnels de la naissance (gynécologues-obstétriciens, internes de spécialité, sage-femme, étudiants sage-femme) en octobre 2016. Les thèmes abordés concernaient les facteurs de risque maternels, obstétricaux et fœtaux associés aux lésions périnéales (du 1er au 4e degrés) et les mesures de protection associées. Il a été réalisé une analyse descriptive des données recueillies.RésultatsCent cinq professionnels ont répondu au questionnaire. Les facteurs de risque identifiés étaient en accord avec ceux mis en évidence dans la littérature. D’autres facteurs, non connus comme associés à un risque périnéal, étaient cités par les professionnels (tabagisme, phototype). En cas de perception, subjective, par le professionnel d’un risque périnéal important, ils pratiquaient plus fréquemment une épisiotomie (15 % vs 0 %, p < 0,001) ou considéraient que l’accouchement devaient être réalisé par un obstétricien (34 % vs 8 %, p < 0,001).ConclusionCette évaluation montre que les professionnels de la naissance connaissent les principaux facteurs de risque de lésion périnéale. En revanche, ils utilisent facilement des manœuvres de protection périnéale (épisiotomie par exemple) sans réelle efficacité démontrée

Validation of a non-motor fluctuations questionnaire in Parkinson's disease

International audienc

Liver transplantation as a rescue therapy for severe neurologic forms of Wilson disease

OBJECTIVE: To evaluate the effect of liver transplantation (LT) in patients with Wilson disease (WD) with severe neurologic worsening resistant to active chelation. METHODS: French patients with WD who underwent LT for pure neurologic indication were retrospectively studied. Before LT and at the last follow-up, neurologic impairment was evaluated with the Unified Wilson's Disease Rating Scale (UWDRS) score, disability with the modified Rankin Scale (mRS) score, and hepatic function with the Model for End-stage Liver Disease score, together with the presence of a Kayser-Fleischer ring (KFR), brain MRI scores, and copper balance. The survival rate and disability at the last follow-up were the coprimary outcomes; evolution of KFR and brain MRI were the secondary outcomes. Prognosis factors were further assessed. RESULTS: Eighteen patients had LT. All were highly dependent before LT (median mRS score 5). Neurologic symptoms were severe (median UWDRS score 105), dominated by dystonia and parkinsonism. The cumulated survival rate was 88.8% at 1 year and 72.2% at 3 and 5 years. At the last follow-up, 14 patients were alive. Their mRS and UWDRS scores improved (p \textless 0.0001 and p = 0.0003). Eight patients had a major improvement (78% decrease of the UWDRS score), 4 a moderate one (41% decrease), and 2 a stable status. KFR and brain MRI scores improved (p = 0.0007). Severe sepsis (p = 0.011) and intensive care unit admission (p = 0.001) before LT were significantly associated with death. CONCLUSIONS: LT is a rescue therapeutic option that should be carefully discussed in selected patients with neurologic WD resistant to anticopper therapies (chelators or zinc salts) as it might allow patients to gain physical independency with a reasonable risk. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that for patients with WD with severe neurologic worsening resistant to active pharmacologic therapy, LT might decrease neurologic impairment