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1. Pathologic and MRI analysis in acute atypical inflammatory demyelinating lesions

Author

Thierry Vincent, A.-M. Guennoc, Pierre Labauge, Anne Kerbrat, François Cotton, Stéphane Kremer, Béatrice Carsin-Nicol, Mahmoud Charif, Sandra Vukusic, Thibaut Allou, Jérôme De Seze, Aurélie Ruet, Laurent Magy, Nicolas Menjot de Champfleur, Clarisse Carra-Dalliere, Guido Ahle, Eric Thouvenot, Françoise Durand-Dubief, Hélène Oesterle, Valérie Rigau, Denis Sablot, Romain Marignier, Nicolas Collongues, Gilles Edan, Mikael Cohen, Sonia Hebbadj, Benoit Lhermitte, Frederic Taithe, M. P. Boncoeur-Martel, Xavier Ayrignac, Grelier, Elisabeth, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Institut des Neurosciences de Montpellier (INM), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM), Service d'Anatomie Pathologique Générale, CHU Strasbourg-Hôpital de Hautepierre [Strasbourg], Département d’Immunologie, Département de neurologie [Montpellier], Université Montpellier 1 (UM1)-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Hôpital Gui de Chauliac [Montpellier]-Université de Montpellier (UM), Service de Neurologie [Strasbourg], CHU Strasbourg-Hopital Civil, Service de radiologie [Strasbourg], Service de Neurologie [Hôpitaux Civils de Colmar], Hôpitaux Civils Colmar, Hôpitaux Civils de Colmar, Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Hospices Civils de Lyon (HCL), RMN et optique : De la mesure au biomarqueur, Centre de Recherche en Acquisition et Traitement de l'Image pour la Santé (CREATIS), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Hospices Civils de Lyon (HCL)-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Hospices Civils de Lyon (HCL)-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Pierre Wertheimer, Département de Neurologie, Service de Neurologie [Lyon], CHU Lyon, Service de Neurologie [CHU Clermont-Ferrand], CHU Gabriel Montpied [Clermont-Ferrand], CHU Clermont-Ferrand-CHU Clermont-Ferrand-CHU Estaing [Clermont-Ferrand], CHU Clermont-Ferrand, Hôpital Pasteur [Nice] (CHU), Service de Neurologie [Tours], Centre Hospitalier Régional Universitaire de Tours (CHRU Tours)-Hôpital Bretonneau, Service de Neurologie [Rennes] = Neurology [Rennes], CHU Pontchaillou [Rennes], Service de neurologie [Rennes], Université de Rennes (UR), Service de neurochirurgie [Rennes] = Neurosurgery [Rennes], Centre Hospitalier Universitaire de Nîmes (CHU Nîmes), Service de Neurologie [CHU Nimes] (Pôle NIRR), Hôpital Universitaire Carémeau [Nîmes] (CHU Nîmes), Centre Hospitalier Universitaire de Nîmes (CHU Nîmes)-Centre Hospitalier Universitaire de Nîmes (CHU Nîmes), Neuroinflammation: imagerie et thérapie de la sclérose en plaques, Université Bordeaux Segalen - Bordeaux 2-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Neurologie [CHU Limoges], CHU Limoges, Neuroépidémiologie Tropicale (NET), CHU Limoges-Institut d'Epidémiologie Neurologique et de Neurologie Tropicale-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Génomique, Environnement, Immunité, Santé, Thérapeutique (GEIST), Université de Limoges (UNILIM)-Université de Limoges (UNILIM), Service de Radiologie et Imagerie Médicale [CHU Limoges], Institut des Neurosciences de Montpellier - Déficits sensoriels et moteurs (INM), Hôpital Gui de Chauliac [Montpellier]-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Université Montpellier 1 (UM1)-Université de Montpellier (UM), Université Jean Monnet [Saint-Étienne] (UJM)-Hospices Civils de Lyon (HCL)-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Institut National des Sciences Appliquées (INSA)-Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université de Lyon-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Jean Monnet [Saint-Étienne] (UJM)-Hospices Civils de Lyon (HCL)-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM), Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université Claude Bernard Lyon 1 (UCBL), Hôpital Bretonneau-Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Université Montpellier 1 (UM1)-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Hôpital Gui de Chauliac [CHU Montpellier], Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Université de Montpellier (UM), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Claude Bernard Lyon 1 (UCBL), and Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)

Subjects
Adult, Male, Atypical inflammatory demyelinating syndrome, medicine.medical_specialty, Pathology, Multiple Sclerosis, Histopathology, Context (language use), Immunopathology, Cohort Studies, Lesion, Young Adult, 03 medical and health sciences, 0302 clinical medicine, medicine, Humans, 030212 general & internal medicine, Pathological, Aged, Retrospective Studies, Neuroradiology, Aquaporin 4, medicine.diagnostic_test, business.industry, Multiple sclerosis, Brain biopsy, Neuromyelitis Optica, food and beverages, Magnetic resonance imaging, Middle Aged, medicine.disease, Atypical demyelinating lesions, Magnetic Resonance Imaging, 3. Good health, Neurology, [SDV.SPEE] Life Sciences [q-bio]/Santé publique et épidémiologie, Acute Disease, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, Female, Neurology (clinical), medicine.symptom, business, Aquaporin-4, 030217 neurology & neurosurgery, Demyelinating Diseases
Abstract

The diagnosis of atypical inflammatory demyelinating lesions can be difficult. Brain biopsy is often required to exclude neoplasms. Moreover, the relationship between these lesions and multiple sclerosis and NMOSD is not clear. Our objectives were to describe radiological and pathological characteristics of patients with acute inflammatory demyelinating lesions. We retrospectively identified patients with brain biopsy performed for diagnostic uncertainty revealing a demyelinating lesion. A complete clinical, biological, radiological and pathological analysis was performed. Twenty patients (15 with a single lesion) were included. MRI disclosed a wide range of lesions including infiltrative lesions (40%), ring-like lesion (15%) Balo-like lesion (15%) and acute haemorrhagic leukoencephalitis (20%). In spite of a marked heterogeneity, some findings were common: a peripheral B1000 hyperintense rim (70%), a slight oedema with mild mass effect (75%) and an open-rim peripheral enhancement (75%). Histopathology revealed that all cases featured macrophages distributed throughout, extensive demyelination, axonal preservation and absence of haemorrhagic changes. In the majority of cases, macrophages were the predominant inflammatory infiltrate and astrocytes were reactive and dystrophic. Aquaporin-4 staining was systematically preserved. After a mean follow-up of 5 years (1–12), 16/20 patients had a diagnosis of monophasic acute atypical inflammatory demyelinating lesion. One patient was diagnosed with MS and 3 with AQP4 negative NMOSD. Although imaging findings in patients with atypical inflammatory demyelinating lesions are heterogeneous, some common features such as peripheral DWI hyperintense rim with open-rim enhancement and absence of oedema argue in favour of a demyelinating lesion and should preclude a brain biopsy. In this context, AQP4 staining is systematically preserved and argues against an AQP4-positive NMOSD. Moreover, long-term follow-up is characterized by low recurrence rate.

Published
2019

2. Risk Factors and Time to Clinical Symptoms of Multiple Sclerosis Among Patients With Radiologically Isolated Syndrome

Author

Olivier Casez, Mikael Cohen, Eric Thouvenot, Adil Maarouf, C. Bensa, Céline Callier, Jonathan Ciron, Pierre Labauge, David Laplaud, Naila Makhani, Bertrand Bourre, Abdulatif Al Khedr, Guillaume Mathey, Thibault Moreau, Sfsep Risc, Sandra Vukusic, Pierre Clavelou, Fabien Rollot, Céline Louapre, Jérôme De Seze, Eric Berger, Daniel Pelletier, A.-M. Guennoc, Aksel Siva, Christina J. Azevedo, Ayman Tourbah, Darin T. Okuda, Alain Créange, F. Durand-Dubief, Philippe Cabre, Jean-Philippe Camdessanché, Hélène Zéphir, Emmanuelle Le Page, Nathalie Derache, Orhun H. Kantarci, Jean-Philippe Neau, Patrick Vermersch, Aurélie Ruet, Lydiane Mondot, Ofsep Investigators, Christine Lebrun-Frénay, Université Côte d'Azur (UCA), Observatoire Français de la Sclérose En Plaques [Lyon] (OFSEP), Centre de recherche en neurosciences de Lyon (CRNL), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Université Jean Monnet [Saint-Étienne] (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Université de Lyon, Lille Neurosciences & Cognition - U 1172 (LilNCog (ex-JPARC)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre d'Investigation Clinique [Rennes] (CIC), Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Hôpital Pontchaillou-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Pontchaillou [Rennes], Hospices Civils de Lyon (HCL), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Université de Montpellier (UM), Hôpital de la Fondation Ophtalmologique Adolphe de Rothschild [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre Hospitalier Universitaire de Nîmes (CHU Nîmes), Institut de Génomique Fonctionnelle (IGF), Université de Montpellier (UM)-Université Montpellier 1 (UM1)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Montpellier 2 - Sciences et Techniques (UM2)-Centre National de la Recherche Scientifique (CNRS), Université de Nantes (UN), CHU Strasbourg, CHU Toulouse [Toulouse], Neurologie générale et maladies inflammatoires du système nerveux [Toulouse], Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-CHU Toulouse [Toulouse]-Hôpital Purpan [Toulouse], CHU Rouen, Normandie Université (NU), CHU de la Martinique [Fort de France], Centre Hospitalier Universitaire [Grenoble] (CHU), Physiopathologie de la Plasticité Neuronale (Neurocentre Magendie - U1215 Inserm), Université de Bordeaux (UB)-Institut François Magendie-Institut National de la Santé et de la Recherche Médicale (INSERM), Adaptation, mesure et évaluation en santé. Approches interdisciplinaires (APEMAC), Université de Lorraine (UL), Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Centre d'épidémiologie des populations (CEP), Université de Bourgogne (UB)-Centre Régional de Lutte contre le cancer Georges-François Leclerc [Dijon] (UNICANCER/CRLCC-CGFL), UNICANCER-UNICANCER, Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), CHU Amiens-Picardie, CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), Neuro-Dol (Neuro-Dol), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Clermont Auvergne (UCA), Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Hôpital Henri Mondor, Centre hospitalier universitaire de Poitiers (CHU Poitiers), Hôpital Raymond Poincaré [AP-HP], Maladies et hormones du système nerveux (DHNS), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris-Saclay, Centre Hospitalier Universitaire de Saint-Etienne (CHU de Saint-Etienne), Hôpital de la Timone [CHU - APHM] (TIMONE), Mayo Clinic [Jacksonville], Cerrahpasa Faculty of Medicine, Istanbul University, University of Southern California (USC), Yale University School of Medicine, University of Texas Southwestern Medical Center [Dallas], This study was funded through the Observatoire Français de la Sclérose En Plaques, which is supported by grant ANR-10-COHO-002 provided by the French State and handled by the Agence Nationale de la Recherche within the framework of the Investments for the Future program by the Eugène Devic European Database for Multiple Sclerosis Foundation against multiple sclerosis and by the Aide pour la Recherche sur la Sclérose En Plaques Foundation., ANR-10-COHO-0002,OFSEP,Observatoire Français de la Sclérose en Plaques(2010), Centre de recherche en neurosciences de Lyon - Lyon Neuroscience Research Center (CRNL), Université de Lyon-Université de Lyon-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Lille Neurosciences & Cognition - U 1172 (LilNCog), Université de Rennes (UR)-Hôpital Pontchaillou-Institut National de la Santé et de la Recherche Médicale (INSERM), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Neurocentre Magendie : Physiopathologie de la Plasticité Neuronale (U1215 Inserm - UB), Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), Yale School of Medicine [New Haven, Connecticut] (YSM), Jonchère, Laurent, and Cohortes - Observatoire Français de la Sclérose en Plaques - - OFSEP2010 - ANR-10-COHO-0002 - COHO - VALID

Subjects
Adult, Male, Pediatrics, medicine.medical_specialty, Multivariate analysis, Multiple Sclerosis, Adolescent, [SDV]Life Sciences [q-bio], 01 natural sciences, 010104 statistics & probability, 03 medical and health sciences, 0302 clinical medicine, Risk Factors, medicine, Humans, 0101 mathematics, Original Investigation, Clinically isolated syndrome, [SDV.MHEP] Life Sciences [q-bio]/Human health and pathology, medicine.diagnostic_test, Radiotherapy, business.industry, Multiple sclerosis, Research, Hazard ratio, Magnetic resonance imaging, Conversion, General Medicine, Middle Aged, medicine.disease, 3. Good health, Clinical trial, [SDV] Life Sciences [q-bio], Online Only, Cross-Sectional Studies, Neurology, Sample size determination, Female, France, business, 030217 neurology & neurosurgery, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology, Cohort study
Abstract

This cohort study investigates risk factors associated with time to a multiple sclerosis clinical event among patients with radiologically isolated syndrome., Key Points Question Are there clinical or demographic factors associated with time to clinical symptoms of multiple sclerosis among patients with radiologically isolated syndrome? Findings In this cohort study of 372 individuals with radiologically isolated syndrome, young age, the presence of spinal cord lesions, and gadolinium-enhancing lesions on the index magnetic resonance imaging scan were associated with increased risk of onset of clinical symptoms of multiple sclerosis. Meaning These findings identify 3 risk factors associated with multiple sclerosis at the preclinical stage of central nervous system demyelinating disease defined by radiologically isolated syndrome., Importance Younger age, oligoclonal bands, and infratentorial and spinal cord lesions are factors associated with an increased 10-year risk of clinical conversion from radiologically isolated syndrome (RIS) to multiple sclerosis (MS). Whether disease-modifying therapy is beneficial for individuals with RIS is currently unknown. Objectives To evaluate the 2-year risk of a clinical event (onset of clinical symptoms of MS) prospectively, identify factors associated with developing an early clinical event, and simulate the sample size needed for a phase III clinical trial of individuals with RIS meeting 2009 RIS criteria. Design, Setting, and Participants This cohort study used data on prospectively followed-up individuals with RIS identified at 1 of 26 tertiary centers for MS care in France that collect data for the Observatoire Français de la Sclérose en Plaques database. Participants were aged 10 to 80 years with 2 or more magnetic resonance imaging (MRI) scans after study entry and an index scan after 2000. All diagnoses were validated by an expert group, whose review included a double centralized MRI reading. Data were analyzed from July 2020 to January 2021. Exposure Diagnosis of RIS. Main Outcomes and Measures Risk of clinical event and associated covariates at index scan were analyzed among all individuals with RIS. Time to the first clinical event was compared by covariates, and sample size estimates were modeled based on identified risk factors. Results Among 372 individuals with RIS (mean [SD] age at index MRI scan, 38.6 [12.1] years), 354 individuals were included in the analysis (264 [74.6%] women). A clinical event was identified among 49 patients (13.8%) within 2 years, which was associated with an estimated risk of conversion of 19.2% (95% CI, 14.1%-24.0%). In multivariate analysis, age younger than 37 years (hazard ratio [HR], 4.04 [95% CI, 2.00-8.15]; P

Published
2021

3. Relapses in Patients Treated with High-Dose Biotin for Progressive Multiple Sclerosis

Author

Eric Berger, Sophie Mathais, A.-M. Guennoc, Laurent Magy, Thibault Moreau, Bruno Pereira, Jérôme De Seze, David Laplaud, Olivier Gout, Giovanni Castelnovo, Elisabeth Maillart, Olivier Heinzlef, Gilles Defer, Frederic Taithe, Pierre Labauge, Jonathan Ciron, Sandra Vukusic, Cécile Dulau, Pierre Clavelou, Christine Lebrun-Frenay, Bertrand Bourre, Jean Pelletier, Xavier Moisset, Gilles Edan, Patrick Vermersch, Jean-Philippe Camdessanché, Guillaume Mathey, CHU Clermont-Ferrand, Neuro-Dol (Neuro-Dol), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Clermont Auvergne [2017-2020] (UCA [2017-2020]), Département de Neurologie [Toulouse], Institut des Neurosciences [Toulouse], Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées, CHU Montpellier, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Université de Montpellier (UM), INSERM, Neurocentre Magendie, U1215, Physiopathologie de la Plasticité Neuronale, F-33000 Bordeaux, France, CIC Bordeaux, Université Bordeaux Segalen - Bordeaux 2-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre hospitalier universitaire de Nantes (CHU Nantes), CIC - Nantes, Université de Nantes (UN)-IFR26-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre de Recherche en Transplantation et Immunologie (U1064 Inserm - CRTI), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN), CIC Strasbourg (Centre d’Investigation Clinique Plurithématique (CIC - P) ), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Strasbourg (UNISTRA)-Hôpital de Hautepierre [Strasbourg]-Nouvel Hôpital Civil de Strasbourg, Service de Neurologie [Strasbourg], CHU Strasbourg-Hopital Civil, Assistance Publique - Hôpitaux de Marseille (APHM), Service de Neurologie [CHRU Besançon], Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Hôpital Pasteur [Nice] (CHU), Centre Hospitalier Universitaire de Nice (CHU Nice), Service de Neurologie [CHU Nimes] (Pôle NIRR), Hôpital Universitaire Carémeau [Nîmes] (CHU Nîmes), Centre Hospitalier Universitaire de Nîmes (CHU Nîmes)-Centre Hospitalier Universitaire de Nîmes (CHU Nîmes), Centre d'Investigation Clinique [Rennes] (CIC), Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Hôpital Pontchaillou-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Pontchaillou [Rennes], Service de Neurologie [CHU Caen], Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Tumorothèque de Caen Basse-Normandie (TCBN), Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Lille Neurosciences & Cognition - U 1172 (LilNCog (ex-JPARC)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), CHU Rouen, Normandie Université (NU), Service de neurologie [CHU de Saint-Étienne], Centre Hospitalier Universitaire de Saint-Etienne (CHU de Saint-Etienne), Service de Neurochirurgie [CHU Limoges], CHU Limoges, Service de neuroradiologie [Tours], Centre Hospitalier Régional Universitaire de Tours (CHRU TOURS), Maladies chroniques, santé perçue, et processus d'adaptation (APEMAC), Université de Lorraine (UL), Service de neurologie [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Service de Neurologie générale, vasculaire et dégénérative (CHU de Dijon), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Service de neurologie [Paris] (Fondation Ophtalmologique Adolphe de Rothschild), Fondation Ophtalmologique Adolphe de Rothschild [Paris], Service de Neurologie [CHU de Poissy], CHU De Poissy, Service de neurologie 1 [CHU Pitié-Salpétrière], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Hospices Civils de Lyon, Departement de Neurologie (HCL), Centre de recherche en neurosciences de Lyon (CRNL), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Université Jean Monnet [Saint-Étienne] (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Université de Lyon, Observatoire Francais de la Sclerose en Plaques (OFSEP), Université de Toulouse (UT)-Université de Toulouse (UT)-Université Toulouse III - Paul Sabatier (UT3), Université de Toulouse (UT)-Université de Toulouse (UT), Neurocentre Magendie : Physiopathologie de la Plasticité Neuronale (U1215 Inserm - UB), Université de Bordeaux (UB)-Institut François Magendie-Institut National de la Santé et de la Recherche Médicale (INSERM), Université de Strasbourg (UNISTRA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Nouvel Hôpital Civil de Strasbourg-Hôpital de Hautepierre [Strasbourg], Université de Rennes (UR)-Hôpital Pontchaillou-Institut National de la Santé et de la Recherche Médicale (INSERM), Lille Neurosciences & Cognition - U 1172 (LilNCog), Service de Neurologie [CHU de Saint-Étienne], Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E)-Université Jean Monnet - Saint-Étienne (UJM), CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre de recherche en neurosciences de Lyon - Lyon Neuroscience Research Center (CRNL), Université de Lyon-Université de Lyon-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Service de Neurologie [CHU Pitié-Salpêtrière], IFR70-CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)

Subjects
0301 basic medicine, Adult, Male, medicine.medical_specialty, Neurology, Propensity score, [SDV]Life Sciences [q-bio], Biotin, 03 medical and health sciences, chemistry.chemical_compound, symbols.namesake, 0302 clinical medicine, Recurrence, Internal medicine, Medicine, Humans, Pharmacology (medical), In patient, Poisson regression, Relapse, Clinical trials observational study, Retrospective Studies, Pharmacology, Progressive multiple sclerosis, business.industry, Retrospective cohort study, Middle Aged, Multiple Sclerosis, Chronic Progressive, 3. Good health, 030104 developmental biology, chemistry, Case-Control Studies, Propensity score matching, symbols, Original Article, Neurology (clinical), Neurosurgery, business, 030217 neurology & neurosurgery
Abstract

High-dose biotin (HDB) is a therapy used in non-active progressive multiple sclerosis (PMS). Several reports have suggested that HDB treatment may be associated with an increased risk of relapse. We aimed to determine whether HDB increases the risk of clinical relapse in PMS and describe the characteristics of the patients who experience it. We conducted a French, multicenter, retrospective study, comparing a group of PMS patients treated with HDB to a matched control group. Poisson regression was applied to model the specific statistical distribution of the annualized relapse rate (ARR). A propensity score (PS), based on the inverse probability of treatment weighting (IPTW), was used to adjust for indication bias and included the following variables: gender, primary PMS or not, age, EDSS, time since the last relapse, and co-prescription of a DMT. Two thousand six hundred twenty-eight patients treated with HDB and 654 controls were analyzed with a follow-up of 17 ± 8 months. Among them, 148 validated relapses were observed in the group treated with biotin and 38 in the control group (p = 0.62). After adjustment based on the PS, the ARR was 0.044 ± 0.23 for the biotin-treated group and 0.028 ± 0.16 for the control group (p = 0.18). The more relapses there were before biotin, the higher the risk of relapse during treatment, independently from the use of HDB. While the number of relapses reported for patients with no previous inflammatory activity receiving biotin has gradually increased, the present retrospective study is adequately powered to exclude an elevated risk of relapse for patients with PMS treated with HDB. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (10.1007/s13311-020-00926-2) contains supplementary material, which is available to authorized users.

Published
2020

4. Progressive Multifocal Leukoencephalopathy Incidence and Risk Stratification Among Natalizumab Users in France

Author

Xavier Moisset, Elisabeth Maillart, Julie Pique, Ofsep Investigators, Pierre Labauge, Bruno Stankoff, Eric Thouvenot, Bertrand Bourre, Fabien Rollot, Hélène Zéphir, Nathalie Derache, Gilles Edan, Sandra Vukusic, Audrey Rico-Lamy, Christine Lebrun-Frenay, David-Axel Laplaud, Eric Berger, Nicolas Maubeuge, Ayman Tourbah, Sandrine Wiertlewski, A.-M. Guennoc, Céline Labeyrie, Patrick Vermersch, Olivier Heinzlef, Mathieu Vaillant, Karolina Hankiewicz, C. Bensa, Philippe Cabre, Jérôme De Seze, Aurélie Ruet, Thibault Moreau, Romain Casey, Ivania Patry, Romain Marignier, Abdullatif Al-Khedr, Abir Wahab, Alexis Montcuquet, Chantal Nifle, Jean-Philippe Camdessanché, Guillaume Mathey, David Brassat, Service de Neurologie [Lyon], CHU Lyon, Université Claude Bernard Lyon 1 (UCBL), Université de Lyon, Centre de recherche en neurosciences de Lyon (CRNL), Université de Lyon-Université de Lyon-Université Jean Monnet [Saint-Étienne] (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Hospices Civils de Lyon (HCL), Fondation Eugène Devic EDMUS, Maladies chroniques, santé perçue, et processus d'adaptation (APEMAC), Université de Lorraine (UL), Service de neurologie [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Service de neurologie [Rennes], Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES), Centre d'Investigation Clinique [Rennes] (CIC), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Hôpital Pontchaillou-Institut National de la Santé et de la Recherche Médicale (INSERM), Neurologie vasculaire, pathologie neuro-dégénérative et explorations fonctionnelles du système nerveux [Toulouse], Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-CHU Toulouse [Toulouse]-Hôpital Purpan [Toulouse], CHU Toulouse [Toulouse], Neuroinflammation: imagerie et thérapie de la sclérose en plaques, Université Bordeaux Segalen - Bordeaux 2-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Universitaire de Saint-Etienne (CHU de Saint-Etienne), Institute for Translational Research in Inflammation - U 1286 (INFINITE (Ex-Liric)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Département de neurologie [Montpellier], Université Montpellier 1 (UM1)-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Hôpital Gui de Chauliac [Montpellier]-Université de Montpellier (UM), CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), Department of Neurology, Centre Hospitalier Universitaire de Nice (CHU Nice), Service de Neurologie générale, vasculaire et dégénérative (CHU de Dijon), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Centre de Recherche en Transplantation et Immunologie (U1064 Inserm - CRTI), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN), Service de Neurologie [CHRU Besançon], Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Neuro-Dol (Neuro-Dol), Université d'Auvergne - Clermont-Ferrand I (UdA)-Institut National de la Santé et de la Recherche Médicale (INSERM), Hôpital de la Timone [CHU - APHM] (TIMONE), Aix Marseille Université (AMU), Institut du Cerveau et de la Moëlle Epinière = Brain and Spine Institute (ICM), Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Service de Neurologie [CHU Nice], Hôpital Pasteur [Nice] (CHU)-Centre Hospitalier Universitaire de Nice (CHU Nice), Centre Hospitalier Universitaire de Nîmes (CHU Nîmes), Institut de Génomique Fonctionnelle (IGF), Université de Montpellier (UM)-Université Montpellier 1 (UM1)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Montpellier 2 - Sciences et Techniques (UM2)-Centre National de la Recherche Scientifique (CNRS), CHI Poissy-Saint-Germain, CHU Amiens-Picardie, Service de Neurologie, Fondation Ophtalmologique Rothschild, Université Grenoble Alpes - UFR Médecine (UGA UFRM), Université Grenoble Alpes (UGA), CHU de la Martinique [Fort de France], Service de Neurologie [CHU Limoges], CHU Limoges, Service de neurologie [Mondor], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), CHU Saint-Etienne, Service de neurologie [Reims], Centre Hospitalier Universitaire de Reims (CHU Reims), Service de Neurologie [Tours], Hôpital Bretonneau-Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Service de neurologie [CH Saint Denis], Centre Hospitalier de Saint-Denis [Ile-de-France], Hôpital Sud Francilien Corbeil Essonne, Centre Hospitalier de Versailles André Mignot (CHV), Service de neurologie [Poitiers], Centre hospitalier universitaire de Poitiers (CHU Poitiers), Service de neurologie [Le Kremlin Bicêtre], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Bicêtre, OFSEP (Observatoire Français de la Sclérose en Plaques) is supported by grant ANR-10-COHO-002 from the French state and handled by the Agence Nationale de la Recherche, within the framework of the Investments for the Future program, by the Eugène Devic EDMUS Foundation Against Multiple Sclerosis, and by the ARSEP Foundation., ANR-10-COHO-0002,OFSEP,Observatoire Français de la Sclérose en Plaques(2010), Centre de recherche en neurosciences de Lyon - Lyon Neuroscience Research Center (CRNL), Université de Lyon-Université de Lyon-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Université de Rennes (UR), Université de Rennes (UR)-Hôpital Pontchaillou-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), Université Montpellier 1 (UM1)-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Hôpital Gui de Chauliac [CHU Montpellier], Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Université de Montpellier (UM), Institut du Cerveau = Paris Brain Institute (ICM), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM)-Centre National de la Recherche Scientifique (CNRS), Fondation Ophtalmologique Adolphe de Rothschild [Paris], Centre Hospitalier Régional Universitaire de Tours (CHRU Tours)-Hôpital Bretonneau, Hôpital Gui de Chauliac [Montpellier]-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Université Montpellier 1 (UM1)-Université de Montpellier (UM), Lipides - Nutrition - Cancer (U866) (LNC), Université de Bourgogne (UB)-Institut National de la Santé et de la Recherche Médicale (INSERM)-AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement-Ecole Nationale Supérieure de Biologie Appliquée à la Nutrition et à l'Alimentation de Dijon (ENSBANA), Cardiovasculaire, métabolisme, diabétologie et nutrition (CarMeN), Institut National de la Recherche Agronomique (INRA)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Hospices Civils de Lyon (HCL), Pierre Wertheimer, Département de Neurologie, Lille Inflammation Research International Center (LIRIC), Institut Pasteur de Lille, Réseau International des Instituts Pasteur (RIIP)-Réseau International des Instituts Pasteur (RIIP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service de neurologie [Besançon], Centre Hospitalier Régional Universitaire [Besançon] (CHRU Besançon)-Hôpital Jean Minjoz, Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [APHP]-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Régional Universitaire de Nîmes (CHRU Nîmes), Hôpital Bicêtre, Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Bicêtre, ANR-10-COHO-002-01/10-COHO-0002,OFSEP,Observatoire Français de la Sclérose en Plaques(2010), Vialaron, Sylvie, and Cohortes - Observatoire Français de la Sclérose en Plaques - - OFSEP2010 - ANR-10-COHO-0002 - COHO - VALID

Subjects
Adult, Male, Pediatrics, medicine.medical_specialty, Adolescent, Population, JC virus, Rate ratio, medicine.disease_cause, 03 medical and health sciences, symbols.namesake, Immunocompromised Host, Young Adult, 0302 clinical medicine, Natalizumab, Multiple Sclerosis, Relapsing-Remitting, Risk Factors, medicine, Humans, Immunologic Factors, 030212 general & internal medicine, Poisson regression, Registries, education, Original Investigation, education.field_of_study, business.industry, Progressive multifocal leukoencephalopathy, Incidence (epidemiology), Incidence, Leukoencephalopathy, Progressive Multifocal, medicine.disease, JC Virus, 3. Good health, Discontinuation, [SDV.SPEE] Life Sciences [q-bio]/Santé publique et épidémiologie, symbols, Female, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, Neurology (clinical), France, business, 030217 neurology & neurosurgery, medicine.drug
Abstract

International audience; Importance:Risk of developing progressive multifocal leukoencephalopathy (PML) is the major barrier to using natalizumab for patients with multiple sclerosis (MS). To date, the association of risk stratification with PML incidence has not been evaluated.Objective:To describe the temporal evolution of PML incidence in France before and after introduction of risk minimization recommendations in 2013.Design, Setting, and Participants:This observational study used data in the MS registry OFSEP (Observatoire Français de la Sclérose en Plaques) collected between April 15, 2007, and December 31, 2016, by participating MS expert centers and MS-dedicated networks of neurologists in France. Patients with an MS diagnosis according to current criteria, regardless of age, were eligible, and those exposed to at least 1 natalizumab infusion (n = 6318) were included in the at-risk population. A questionnaire was sent to all centers, asking for a description of their practice regarding PML risk stratification. Data were analyzed in July 2018.Exposures:Time from the first natalizumab infusion to the occurrence of PML, natalizumab discontinuation plus 6 months, or the last clinical evaluation.Main Outcomes and Measures:Incidence was the number of PML cases reported relative to the person-years exposed to natalizumab. A Poisson regression model for the 2007 to 2016 period estimated the annual variation in incidence and incidence rate ratio (IRR), adjusted for sex and age at treatment initiation and stratified by period (2007-2013 and 2013-2016).Results:In total, 6318 patients were exposed to natalizumab during the study period, of whom 4682 (74.1%) were female, with a mean (SD [range]) age at MS onset of 28.5 (9.1 [1.1-72.4]) years; 45 confirmed incident cases of PML were diagnosed in 22 414 person-years of exposure. The crude incidence rate for the whole 2007 to 2016 period was 2.00 (95% CI, 1.46-2.69) per 1000 patient-years. Incidence significantly increased by 45.3% (IRR, 1.45; 95% CI, 1.15-1.83; P = .001) each year before 2013 and decreased by 23.0% (IRR, 0.77; 95% CI, 0.61-0.97; P = .03) each year from 2013 to 2016.Conclusions and Relevance:The results of this study suggest, for the first time, a decrease in natalizumab-associated PML incidence since 2013 in France that may be associated with a generalized use of John Cunningham virus serologic test results; this finding appears to support the continuation and reinforcement of educational activities and risk-minimization strategies in the management of disease-modifying therapies for multiple sclerosis.

Published
2020

5. Comparative effectiveness of teriflunomide vs dimethyl fumarate in multiple sclerosis

Author

Patrick Vermersch, Ivania Patry, Nicolas Maubeuge, Catherine Lubetzki, Ofsep Study Groups, David-Axel Laplaud, Bruno Stankoff, Bruno Brochet, Eric Thouvenot, Céline Labeyrie, Jean-Philippe Camdessanché, Olivier Gout, Laure Michel, Chantal Nifle, Abdullatif Al Khedr, Emmanuelle Leray, Gilles Edan, Marc Debouverie, Laetitia Barbin, David Brassat, Yohann Foucher, Thibault Moreau, Romain Casey, Jean Pelletier, Christine Lebrun-Frenay, Sandra Vukusic, Laurent Magy, Olivier Heinzlef, Philippe Cabre, Caroline Papeix, Ayman Tourbah, Gilles Defer, Abir Wahab, Pierre Clavelou, Eric Berger, Jérôme De Seze, A.-M. Guennoc, Fabien Rollot, Olivier Casez, Sandrine Wiertlewski, Thomas Debroucker, Pierre Labauge, Bertrand Bourre, Centre de Recherche en Transplantation et Immunologie (U1064 Inserm - CRTI), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN), Cardiovasculaire, métabolisme, diabétologie et nutrition (CarMeN), Institut National de la Recherche Agronomique (INRA)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Hospices Civils de Lyon (HCL)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre d’Investigation Clinique de Nantes (CIC Nantes), Université de Nantes (UN)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre hospitalier universitaire de Nantes (CHU Nantes), Maladies chroniques, santé perçue, et processus d'adaptation (APEMAC), Université de Lorraine (UL), Service de Neurologie [Lyon], CHU Lyon, Centre de Physiopathologie Toulouse Purpan (CPTP), Université Toulouse III - Paul Sabatier (UT3), Université de Toulouse (UT)-Université de Toulouse (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Service de Neurologie, Centre hospitalier universitaire de Nantes (CHU Nantes)-Hôpital Guillaume-et-René-Laennec [Saint-Herblain], Service de neurologie [Bordeaux], CHU Bordeaux [Bordeaux]-Groupe hospitalier Pellegrin, Centre de résonance magnétique biologique et médicale (CRMBM), Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)-Centre National de la Recherche Scientifique (CNRS), Inflammation: mécanismes et régulation et interactions avec la nutrition et les candidoses, Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille, Droit et Santé, Service de neurologie [Rennes], Université de Rennes (UR), Department of Neurology, Centre Hospitalier Universitaire de Nice (CHU Nice), Service de Neurologie [CHU Clermont-Ferrand], CHU Gabriel Montpied [Clermont-Ferrand], CHU Clermont-Ferrand-CHU Clermont-Ferrand-CHU Estaing [Clermont-Ferrand], CHU Clermont-Ferrand, Centre Hospitalier Universitaire de Nîmes (CHU Nîmes), Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), Service de neurologie [Reims], Centre Hospitalier Universitaire de Reims (CHU Reims), Institut du Cerveau = Paris Brain Institute (ICM), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), CHU de la Martinique [Fort de France], CHU Pitié-Salpêtrière [AP-HP], Public Health Agency of Canada, CHI Poissy-Saint-Germain, Service de Neurologie [CHU de Poissy], CHU De Poissy, Service de Neurologie générale, vasculaire et dégénérative (CHU de Dijon), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Centre de Recherches Critiques sur le Droit (CERCRID), Université Lumière - Lyon 2 (UL2)-Université Jean Monnet - Saint-Étienne (UJM)-Centre National de la Recherche Scientifique (CNRS), Fondation Ophtalmologique Adolphe de Rothschild [Paris], Département de neurologie [Montpellier], Université Montpellier 1 (UM1)-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Hôpital Gui de Chauliac [CHU Montpellier], Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Université de Montpellier (UM), Service de Neurologie [CHU Limoges], CHU Limoges, Service de Neurologie [CHU Caen], Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Tumorothèque de Caen Basse-Normandie (TCBN), Hôpital Bicêtre, Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Bicêtre, Lipides - Nutrition - Cancer (U866) (LNC), Université de Bourgogne (UB)-Institut National de la Santé et de la Recherche Médicale (INSERM)-AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement-Ecole Nationale Supérieure de Biologie Appliquée à la Nutrition et à l'Alimentation de Dijon (ENSBANA), École des Hautes Études en Santé Publique [EHESP] (EHESP), Aide à la Décision pour une Médecine Personnalisé - Laboratoire de Biostatistique, Epidémiologie et Recherche Clinique - EA 2415 (AIDMP), Université Montpellier 1 (UM1)-Université de Montpellier (UM), Supported by Fondation ARSEP, CHU Nantes (Appel d’Offre Interne), and Association ANTARES. The French Observatoire of Multiple Sclerosis is supported by a grant provided by the French State and handled by the Agence Nationale de la Recherche, within the framework of the Investments for the Future program, under the reference ANR-10-COHO-002. The study was supported by West Neurosciences Network and Société Francophone de Scl´erose en Plaques, Fondation ARSEP, and by Appel d’Offre Interne du CHU de Nantes., SFSEP and OFSEP groups : Fontaine B, Marignier R, Durand-Dubief F, Mathey G, Le Page E, Peaureaux-Averseng D, Ouallet JC, Ruet A, Collongues N, Hautecoeur P, Zephir H, Maillard E, Cohen M, Derache N, Branger P, Ayrignac X, Carra-Dalliere C, Fromont A, Chamard-Witkowski L, Taithe F, Moisset X, Audoin B, Rico-Lamy A, Castelnovo G, Giannesini C, Fagniez O, Bensa C, Gueguen A, Kasonde IT, De Vilmarest A, Montcuquet A, Vaillants M, Beltran S, Creange A, Ayache S, Abdellaoui M, Pottier C, Slesari I, Deburghraeve V, Neau JP, Servan J, Pico F, Henry C, Hankiewicz K., Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Hospices Civils de Lyon (HCL), Université de Nantes (UN)-Centre hospitalier universitaire de Nantes (CHU Nantes)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre de Physiopathologie Toulouse Purpan ex IFR 30 et IFR 150 (CPTP), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Centre National de la Recherche Scientifique (CNRS), Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES), Service de neurologie, Centre Hospitalier Régional Universitaire de Nîmes (CHRU Nîmes), CHU Saint-Etienne, Institut du Cerveau et de la Moëlle Epinière = Brain and Spine Institute (ICM), Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [APHP]-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Service de Neurologie [CHU Saint-Antoine], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Saint-Antoine [APHP], Département de Neurologie [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-IFR70-CHU Pitié-Salpêtrière [APHP], CHU Pitié-Salpêtrière [APHP], Centre National de la Recherche Scientifique (CNRS)-Université Jean Monnet [Saint-Étienne] (UJM)-Université Lumière - Lyon 2 (UL2), Fondation Ophtalmologique Rothschild, Université Montpellier 1 (UM1)-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Hôpital Gui de Chauliac [Montpellier]-Université de Montpellier (UM), Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Bicêtre, Université de Montpellier (UM)-Université Montpellier 1 (UM1), Le Bihan, Sylvie, Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service de Neurologie [CHU Pitié-Salpêtrière], IFR70-CHU Pitié-Salpêtrière [AP-HP], Hospices Civils de Lyon (HCL)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Institut National de la Recherche Agronomique (INRA), Assistance Publique - Hôpitaux de Marseille (APHM)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), Université de Lille, Droit et Santé-Institut National de la Santé et de la Recherche Médicale (INSERM), Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Hôpital Bicêtre-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris-Sud - Paris 11 (UP11), Neuro-Dol (Neuro-Dol), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Clermont Auvergne [2017-2020] (UCA [2017-2020]), CHU Estaing [Clermont-Ferrand], CHU Clermont-Ferrand-CHU Clermont-Ferrand-CHU Gabriel Montpied [Clermont-Ferrand], and Hôpital Gui de Chauliac [Montpellier]-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Université Montpellier 1 (UM1)-Université de Montpellier (UM)

Subjects
Adult, Male, medicine.medical_specialty, Toluidines, [SDV.NEU.NB]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]/Neurobiology, Hydroxybutyrates, Effectiveness, Dimethyl fumarate, Multiple sclerosis, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, Recurrence, Internal medicine, Teriflunomide, Nitriles, medicine, Humans, 030212 general & internal medicine, 10. No inequality, Adverse effect, ComputingMilieux_MISCELLANEOUS, Intention-to-treat analysis, Expanded Disability Status Scale, [SDV.MHEP] Life Sciences [q-bio]/Human health and pathology, business.industry, Fingolimod Hydrochloride, Odds ratio, Middle Aged, Discontinuation, Treatment Outcome, chemistry, Crotonates, Propensity score matching, Disease Progression, Female, Neurology (clinical), business, 030217 neurology & neurosurgery, Immunosuppressive Agents, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract

ObjectiveIn this study, we compared the effectiveness of teriflunomide (TRF) and dimethyl fumarate (DMF) on both clinical and MRI outcomes in patients followed prospectively in the Observatoire Français de la Sclérose en Plaques.MethodsA total of 1,770 patients with relapsing-remitting multiple sclerosis (RRMS) (713 on TRF and 1,057 on DMF) with an available baseline brain MRI were included in intention to treat. The 1- and 2-year postinitiation outcomes were relapses, increase of T2 lesions, increase in Expanded Disability Status Scale score, and reason for treatment discontinuation. Propensity scores (inverse probability weighting) and logistic regressions were estimated.ResultsThe confounder-adjusted proportions of patients were similar in TRF- compared to DMF-treated patients for relapses and disability progression after 1 and 2 years. However, the adjusted proportion of patients with at least one new T2 lesion after 2 years was lower in DMF compared to TRF (60.8% vs 72.2%, odds ratio [OR] 0.60, p < 0.001). Analyses of reasons for treatment withdrawal showed that lack of effectiveness was reported for 8.5% of DMF-treated patients vs 14.5% of TRF-treated patients (OR 0.54, p < 0.001), while adverse events accounted for 16% of TRF-treated patients and 21% of DMF-treated patients after 2 years (OR 1.39, p < 0.001).ConclusionsAfter 2 years of treatment, we found similar effectiveness of DMF and TRF in terms of clinical outcomes, but with better MRI-based outcomes for DMF-treated patients, resulting in a lower rate of treatment discontinuation due to lack of effectiveness.Classification of evidenceThis study provides Class III evidence that for patients with RRMS, TRF and DMF have similar clinical effectiveness after 2 years of treatment.

Published
2019

6. Immunization and multiple sclerosis: Recommendations from the French Multiple Sclerosis Society

Author

C. Lebrun, S. Vukusic, V. Abadie, C. Achour, F. Ader, H. Alchaar, A. Alkhedr, F. Andreux, G. Androdias, R. Arjmand, B. Audoin, D. Audry, D. Aufauvre, C. Autreaux, X. Ayrignac, M. Bailbe, M. Benazet, C. Bensa, D. Bensmail, E. Berger, P. Bernady, Y. Bertagna, D. Biotti, A. Blanchard-Dauphin, J. Bonenfant, M. Bonnan, B. Bonnemain, F. Borgel, E. Botelho-Nevers, S. Boucly, B. Bourre, C. Boutière, P. Branger, D. Brassat, S. Bresch, V. Breuil, B. Brochet, H. Brugeilles, P. Bugnon, P. Cabre, J.-P. Camdessanché, C. Carra-Dalière, O. Casez, J.-M. Chamouard, B. Chassande, P. Chataignier, M. Chbicheb, A. Chenet, J. Ciron, P. Clavelou, M. Cohen, R. Colamarino, N. Collongues, I. Coman, P.-R. Corail, S. Courtois, M. Coustans, A. Creange, E. Creisson, N. Daluzeau, C. Davenas, J. De Seze, M. Debouverie, R. Depaz, N. Derache, L. Divio, X. Douay, C. Dulau, F. Durand-Dubief, G. Edan, Z. Elias, O. Fagniez, M. Faucher, J.-M. Faucheux, M. Fournier, A. Gagneux-Brunon, P. Gaida, P. Galli, P. Gallien, J. Gaudelus, D. Gault, A. Gayou, M. Genevray, A. Gentil, J. Gere, L. Gignoux, M. Giroux, P. Givron, O. Gout, J. Grimaud, A.-M. Guennoc, N. Hadhoum, P. Hautecoeur, O. Heinzlef, M. Jaeger, S. Jeannin, L. Kremer, A. Kwiatkowski, P. Labauge, C. Labeyrie, S. Lachaud, I. Laffont, C. Lanctin-Garcia, J. Lannoy, L. Lanotte, D. Laplaud, D. Latombe, M. Lauxerois, E. Le Page, C. Lebrun-Frenay, P. Lejeune, P. Lejoyeux, B. Lemonnier, E. Leray, C.-M. Loche, C. Louapre, C. Lubetzki, A. Maarouf, B. Mada, L. Magy, E. Maillart, E. Manchon, R. Marignier, P. Marque, G. Mathey, A. Maurousset, C. Mekies, M. Merienne, L. Michel, A.-M. Milor, X. Moisset, A. Montcuquet, T. Moreau, N. Morel, M. Moussa, J.-P. Naudillon, M. Normand, P. Olive, J.-C. Ouallet, O. Outteryck, C. Pacault, C. Papeix, I. Patry, D. Peaureaux, J. Pelletier, B. Pichon, S. Pittion, E. Planque, M.-C. Pouget, V. Pourcher, C. Radot, I. Robert, F. Rocher, A. Ruet, C. Saint-Val, J.-Y. Salle, A. Salmon, E. Sartori, S. Schaeffer, B. Stankhof, F. Taithe, E. Thouvenot, C. Tizon, A. Tourbah, P. Tourniaire, M. Vaillant, P. Vermersch, S. Vidil, A. Wahab, M.-H. Warter, S. Wiertlewski, B. Wiplosz, B. Wittwer, C. Zaenker, H. Zephir, Université Côte d'Azur (UCA), Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Hospices Civils de Lyon (HCL), Centre de recherche en neurosciences de Lyon (CRNL), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Université Jean Monnet [Saint-Étienne] (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Hosp Civils Lyon, Serv Malad Infect, Lyon, France, Biogéosciences [UMR 6282] [Dijon] (BGS), Centre National de la Recherche Scientifique (CNRS)-Université de Bourgogne (UB)-AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement, Centre de résonance magnétique biologique et médicale (CRMBM), Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)-Centre National de la Recherche Scientifique (CNRS), CHU Montpellier, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), CHU Pontchaillou [Rennes], CHU Saint-Etienne, Centre d'Exploration Métabolique par Résonance Magnétique [Hôpital de la Timone - AP-HM] (CEMEREM), Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)-Centre National de la Recherche Scientifique (CNRS)-Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)-Centre National de la Recherche Scientifique (CNRS)- Hôpital de la Timone [CHU - APHM] (TIMONE), Comité de Développement Horticole du Centre Val de Loire (CDHRC), Génétique, physiopathologie et ingénierie du tissu osseux (GéPITOS), Centre National de la Recherche Scientifique (CNRS)-Université Nice Sophia Antipolis (... - 2019) (UNS), COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-Université Côte d'Azur (UCA), Service d'Etudes du Comportement des Matériaux de Conditionnement (SECM), Commissariat à l'énergie atomique et aux énergies alternatives (CEA), Galaxies, Etoiles, Physique, Instrumentation (GEPI), Institut national des sciences de l'Univers (INSU - CNRS)-Observatoire de Paris, Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Université Paris Diderot - Paris 7 (UPD7)-Centre National de la Recherche Scientifique (CNRS), Service de Neurologie [Rennes] = Neurology [Rennes], Excitabilité nerveuse et thérapeutique (ENT), Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12)-EA 4391, Service de Physiologie Explorations Fonctionnelles-Hôpital Henri Mondor, Maladies chroniques, santé perçue, et processus d'adaptation (APEMAC), Université de Lorraine (UL), RMN et optique : De la mesure au biomarqueur, Centre de Recherche en Acquisition et Traitement de l'Image pour la Santé (CREATIS), Université Jean Monnet [Saint-Étienne] (UJM)-Hospices Civils de Lyon (HCL)-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Jean Monnet [Saint-Étienne] (UJM)-Hospices Civils de Lyon (HCL)-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM), Combustion Research Facility, Sandia National Laboratories - Corporation, Service de Pédiatrie [Jean Verdier], Université Paris 13 (UP13)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Jean Verdier [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Kuriwa Observatory, Fondation Ophtalmologique Adolphe de Rothschild [Paris], Laboratoire de Mécanique, Modélisation et Procédés Propres (M2P2), Centre National de la Recherche Scientifique (CNRS)-École Centrale de Marseille (ECM)-Aix Marseille Université (AMU), Institut de biologie et chimie des protéines [Lyon] (IBCP), Université de Lyon-Université de Lyon-Centre National de la Recherche Scientifique (CNRS), Euromov (EuroMov), Université de Montpellier (UM), Centre de Recherche en Transplantation et Immunologie (U1064 Inserm - CRTI), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN), Université Nice Sophia Antipolis - Faculté de Médecine (UNS UFR Médecine), Université Nice Sophia Antipolis (... - 2019) (UNS), COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-COMUE Université Côte d'Azur (2015-2019) (COMUE UCA), Recherche en Pharmaco-épidémiologie et Recours aux Soins (REPERES), Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-École des Hautes Études en Santé Publique [EHESP] (EHESP), École des Hautes Études en Santé Publique [EHESP] (EHESP), Département Méthodes quantitatives en santé publique (METIS), Institut du Cerveau et de la Moëlle Epinière = Brain and Spine Institute (ICM), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Biologie des Interactions Neurones / Glie, Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Neurologie [CHU Pitié-Salpêtrière], IFR70-CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service de Neurologie [CHU Clermont-Ferrand], CHU Gabriel Montpied [Clermont-Ferrand], CHU Clermont-Ferrand-CHU Clermont-Ferrand-CHU Estaing [Clermont-Ferrand], CHU Clermont-Ferrand, Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Laboratoire d'automatique et de génie des procédés (LAGEP), Université de Lyon-Université de Lyon-École Supérieure Chimie Physique Électronique de Lyon-Centre National de la Recherche Scientifique (CNRS), CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Haras National Suisse, Centre Hospitalier Universitaire de Reims (CHU Reims), Center for health studies, Service de neurologie [Nantes], Université de Nantes (UN)-Centre hospitalier universitaire de Nantes (CHU Nantes)-Hôpital Guillaume-et-René-Laennec [Saint-Herblain], Hospices Civils de Lyon, Centre de recherche en neurosciences de Lyon - Lyon Neuroscience Research Center (CRNL), Biogéosciences [UMR 6282] (BGS), Université de Bourgogne (UB)-Centre National de la Recherche Scientifique (CNRS), Hôpital de la Timone [CHU - APHM] (TIMONE)-Centre de résonance magnétique biologique et médicale (CRMBM), Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)-Centre National de la Recherche Scientifique (CNRS)-Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)-Centre National de la Recherche Scientifique (CNRS), Université Nice Sophia Antipolis (1965 - 2019) (UNS), COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-Centre National de la Recherche Scientifique (CNRS)-Université Côte d'Azur (UCA), Hôpital Henri Mondor-EA 4391, Service de Physiologie Explorations Fonctionnelles-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Université de Lyon-Université de Lyon-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Hospices Civils de Lyon (HCL)-Université Jean Monnet [Saint-Étienne] (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Hospices Civils de Lyon (HCL)-Université Jean Monnet [Saint-Étienne] (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Université de Lyon-Université de Lyon-École Supérieure de Chimie Physique Électronique de Lyon (CPE)-Centre National de la Recherche Scientifique (CNRS), Université de Lyon-Université de Lyon-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), Centre d'Exploration Métabolique par Résonance Magnétique [Hôpital de la Timone - APHM] (CEMEREM), Comité de développement horticole de la région Centre-Val-de-Loire (CDHR CENTRE-VAL-DE-LOIRE), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Université de Rennes (UR)-École des Hautes Études en Santé Publique [EHESP] (EHESP), Université de Bourgogne (UB)-AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement-Centre National de la Recherche Scientifique (CNRS), Assistance Publique - Hôpitaux de Marseille (APHM)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), Assistance Publique - Hôpitaux de Marseille (APHM)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS)-Assistance Publique - Hôpitaux de Marseille (APHM)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS)- Hôpital de la Timone [CHU - APHM] (TIMONE), Institut National des Sciences Appliquées (INSA)-Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université de Lyon-Université Claude Bernard Lyon 1 (UCBL), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris 13 (UP13)-Hôpital Jean Verdier [AP-HP], Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), CHU Estaing [Clermont-Ferrand], CHU Clermont-Ferrand-CHU Clermont-Ferrand-CHU Gabriel Montpied [Clermont-Ferrand], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), COMUE Université Côte d'Azur (2015 - 2019) (COMUE UCA)-COMUE Université Côte d'Azur (2015 - 2019) (COMUE UCA)-Université Côte d'Azur (UCA), Service de Neurologie [Rennes], Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Hôpital Pontchaillou-CHU Pontchaillou [Rennes], Institut d’Électronique, de Microélectronique et de Nanotechnologie (IEMN) - UMR 8520 (IEMN), Ecole Centrale de Lille-Institut supérieur de l'électronique et du numérique (ISEN)-Université de Valenciennes et du Hainaut-Cambrésis (UVHC)-Université de Lille-Centre National de la Recherche Scientifique (CNRS)-Université Polytechnique Hauts-de-France (UPHF), Aix Marseille Université (AMU)-École Centrale de Marseille (ECM)-Centre National de la Recherche Scientifique (CNRS), COMUE Université Côte d'Azur (2015 - 2019) (COMUE UCA)-COMUE Université Côte d'Azur (2015 - 2019) (COMUE UCA), Sorbonne Université-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Département de Neurologie [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-IFR70-CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université, Centre d'Investigation Clinique [Rennes] (CIC), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Hôpital Pontchaillou-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Neurologie, CHU Clermont-Ferrand, Hôpital Jean Minjoz, Centre Hospitalier Régional Universitaire [Besançon] (CHRU Besançon), Sorbonne Université-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Centre National de la Recherche Scientifique (CNRS), and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)

Subjects
medicine.medical_specialty, Vaccination schedule, MEDLINE, Scientific literature, Multiple sclerosis, 03 medical and health sciences, 0302 clinical medicine, Recurrence, Risk Factors, Health care, Medicine, Humans, 030212 general & internal medicine, Immunization Schedule, Societies, Medical, Vaccines, business.industry, Risk of infection, Prevention, Vaccination, medicine.disease, 3. Good health, Neurology, Immunization, Family medicine, Evidence-Based Practice, Practice Guidelines as Topic, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, Neurology (clinical), France, business, Infection, Vaccine, 030217 neurology & neurosurgery
Abstract

Objectives To establish recommendations on immunization for patients with multiple sclerosis (MS). Background Vaccines have been suspected in the past to trigger MS and relapses. With the extension of the immunoactive treatment arsenal, other concerns have been raised more recently about an increased risk of infection or a decreased effectiveness of immunization in immunosuppressed patients. Methods The French Group for Recommendations into Multiple Sclerosis (France4MS) performed a systematic search of papers in Medline and other university databases (January 1975–June 2018). The RAND/UCLA appropriateness method was chosen to review the scientific literature and to formalize the degree of agreement among experts on 5 clinical questions related to immunization and MS. Readers from the steering committee conducted a systematic analysis, wrote a critical synthesis and prepared a list of proposals that were evaluated by a rating group of 28 MS experts. The final version of the recommendations was finally reviewed by a reading group of 110 health care professionals and classified as appropriate, inappropriate or uncertain. Results Neurologists should verify the vaccination status as soon as MS is diagnosed and before disease-modifying treatments (DMTs) are introduced. The French vaccination schedule applies to MS patients and seasonal influenza vaccination is recommended. In the case of treatment-induced immunosuppression, MS patients should be informed about the risk of infection and the vaccination standards of the French High Council of Health should be applied. Live attenuated vaccines are contra-indicated in patients recently treated with immunosuppressive drugs, including corticosteroids; other vaccines can be proposed whatever the treatment, but their effectiveness may be partly reduced with some drugs. Conclusion Physicians and patients should be aware of the updated recommendations for immunizations of patients with MS.

Published
2019

7. Neuromyélite optique à anticorps anti-MOG : à propos d’un cas

Author

P.-J. Pisella, S Majzoub, A M Guennoc, Sophie Arsene, and T. Pichard

Subjects
03 medical and health sciences, Ophthalmology, 0302 clinical medicine, business.industry, Medicine, 030212 general & internal medicine, business, Molecular biology, 030217 neurology & neurosurgery
Published
2017

8. Co-occurrence of MS and ALS: a clue in favor of common pathophysiological findings?

Author

Philippe Corcia, Damien Le Port, Emmanuelle Le Page, Rudolph Hergesheimer, A.-M. Guennoc, Maud Pallix-Guyot, Mathieu Daryabin, Gilles Edan, Ayman Tourbah, and Stéphane Beltran

Subjects
0301 basic medicine, Adult, Male, Pediatrics, medicine.medical_specialty, Multiple Sclerosis, Disease, 03 medical and health sciences, 0302 clinical medicine, Epidemiology, medicine, Humans, Amyotrophic lateral sclerosis, Age of Onset, Chi-Square Distribution, business.industry, Multiple sclerosis, Amyotrophic Lateral Sclerosis, Middle Aged, medicine.disease, Databases, Bibliographic, Magnetic Resonance Imaging, Pathophysiology, 030104 developmental biology, Neurology, Cohort, Female, Neurology (clinical), Age of onset, business, 030217 neurology & neurosurgery
Abstract

Amyotrophic lateral sclerosis (ALS) and multiple sclerosis (MS) are two neurological disorders that seem, theoretically, completely divergent according to epidemiological, clinical, pathophysiological, and therapeutic data. However, some reports that have mentioned the occurrence of both conditions within the same patient underpin the suggestion that this co-occurrence might not be random. We report six co-occurrences of ALS and MS cases, focusing on epidemiological and clinical diseases findings. We then compare our cohort to those in the literature. Our cohort was composed of five females and one male. The age of onset for MS ranged from 27 to 54 years with either primary or secondary prominence while all being progressive. Both diseases occurred sequentially in all but one the cases. Concerning ALS, the age of onset ranged from 51 to 60 years and the site of onset was the legs in 5/6 cases. The disease lasted from four to 29 months. Although infrequent, this co-occurrence supports the hypothesis of common, pathophysiological mechanisms between ALS and MS. We discuss some arguments favoring a potential link between both conditions.

Published
2018

9. Congenital Trypanosomiasis in Child Born in France to African Mother

Author

Luc Paris, Alain Goudeau, Pere Perez-Simarro, Zoha Maakaroun-Vermesse, Louis Bernard, Philippe Lanotte, A.-M. Guennoc, Jacques Chandenier, Marie-Alix De Kyvon, Bertrand de Toffol, Gerardo Priotto, Guillaume Desoubeaux, Infectiologie et Santé Publique (UMR ISP), Institut National de la Recherche Agronomique (INRA)-Université de Tours, Service de Neurologie [Tours], Centre Hospitalier Régional Universitaire de Tours (CHRU Tours)-Hôpital Bretonneau, Service de parasitologie - mycologie [CHU Pitié-Salpétrière], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), CHU Trousseau [Tours], Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), CHU Tours, Service de Parasitologie - Mycologie - Médecine Tropicale, Centre Hospitalier Régional Universitaire de Tours (CHRU TOURS), Centre d’Etude des Pathologies Respiratoires (CEPR), UMR 1100 (CEPR), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Tours, Institut National de la Recherche Agronomique (INRA)-Université de Tours (UT), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Université de Tours (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM), Infectiologie Animale et Santé Publique - IASP (Nouzilly, France), Institut National de la Recherche Agronomique (INRA), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Pitié-Salpêtrière [APHP], and Université de Tours-Institut National de la Santé et de la Recherche Médicale (INSERM)

Subjects
0301 basic medicine, Pediatrics, Letter, Blood transfusion, Epidemiology, sleeping sickness, [SDV]Life Sciences [q-bio], medicine.medical_treatment, Choreiform movement, lcsh:Medicine, 0302 clinical medicine, [SDV.MHEP.MI]Life Sciences [q-bio]/Human health and pathology/Infectious diseases, Medicine, African trypanosomiasis, Trypanosoma brucei, tsetse fly, enfant, ComputingMilieux_MISCELLANEOUS, [SDV.MP.MYC]Life Sciences [q-bio]/Microbiology and Parasitology/Mycology, biology, Psychomotor retardation, 3. Good health, Infectious Diseases, Democratic Republic of the Congo, France, medicine.symptom, medicine.drug, Microbiology (medical), medicine.medical_specialty, autochthonous congenital human trypanosomiasis, 030106 microbiology, 030231 tropical medicine, Médecine humaine et pathologie, parasites, lcsh:Infectious and parasitic diseases, 03 medical and health sciences, Eflornithine, lcsh:RC109-216, [SDV.MP.PAR]Life Sciences [q-bio]/Microbiology and Parasitology/Parasitology, Letters to the Editor, Pregnancy, trypanosomiase, business.industry, lcsh:R, DRC, Tsetse fly, parasitic infection, Democratic Republic of Congo, biology.organism_classification, medicine.disease, Surgery, Human health and pathology, business, Congenital Trypanosomiasis in Child Born in France to African Mother, Trypanosomiasis
Abstract

To the Editor: Sleeping sickness, or human African trypanosomiasis, is a neglected tropical parasitic infection transmitted by the tsetse fly bite. In central and western Africa, trypanosomiasis is caused by the Trypanosoma brucei subspecies gambiense. Chronic symptoms of the disease include neurologic impairment and sleep disorders (1). Infected children and adults can exhibit other nonspecific symptoms (1,2) attributable to biologic inflammatory syndrome, usually accompanied by an increase of IgM. Sleeping sickness can be fatal if left untreated (3). Although the efforts of the World Health Organization (WHO), national control programs, and nongovernmental organizations such as Medecins Sans Frontieres have substantially reduced the global burden of human African trypanosomiasis, its current annual incidence is still estimated to be ≈10,000 new cases (1,4). Most cases occur during long stays in trypanosomiasis-endemic areas. Rare alternative routes of transmission are possible in nonendemic areas (e.g., through blood transfusion or organ transplantation) (5). We report the case of a 14-month-old boy infected with Tr. brucei through the transplacental route. The child was referred to a pediatric care unit because of psychomotor retardation and axial hypotonia. His mother was from the Democratic Republic of the Congo (DRC) and had arrived in France 3 years earlier. The pregnancy, which had been initiated and monitored in France, was normal through delivery. Nonetheless, the newborn was placed with a foster family because his mother had vigilance disorders, aphasia, fluctuating hemiparesia and tetraparesia, convulsions associated with choreiform movements, anxiety, and severe depression. The foster family reported that the child did not smile and had been unable to grasp objects in the last 8 months. Clinical examination showed z-scores of −2.25 SD and −1.38 SD for height and weight, respectively; multiple lymph node enlargement; and absence of tendon reflexes. Intermittent fever and dystonic movements were reported later during a subsequent hospitalization. The results from blood tests were compatible with chronic inflammatory syndrome (albumin 28 g/L; hypergammaglobulinemia with IgM 7.38 g/L and IgG 31.3 g/L; leukocytes 20.9 g/L; hemoglobin concentration 98 g/L). Cranial magnetic resonance imaging showed several lesions of the white matter, mostly in the left frontotemporal lobe. The boy’s cerebrospinal fluid (CSF) contained glucose at 2.5 mmol/L, protein at 0.88 g/L, and 125 leukocytes/μL (100% lymph cells). Microscopic observation of CSF and blood smear highlighted Tr. brucei trypomastigotes (Figure, panels A and B; Video). Figure A) Cytological slide prepared from cerebrospinal fluid (CSF) from a child with congenital trypanosomiasis who was born in France to an African mother (Gram staining, original magnification ×1,000). B) Blood ... Video (video link forthcoming) Direct observation of the cerebrospinal fluid from a child with congenital trypanosomiasis who was born in France to an African mother (fresh mount in a Malassez counting cell, original ... All the clinical and biologic findings were compatible with a diagnosis of autochthonous congenital trypanosomiasis in the meningo-encephalitic second stage. In addition to diffuse bilateral lesions of her white matter and biologic inflammatory syndrome, the mother also exhibited the same neurologic symptoms as the boy, at least since her last stay in DRC, a trypanosomiasis-endemic country that currently harbors ≈90% of new cases of African trypanosomiasis worldwide (6). She previously lived in Kinshasa and also reported a short visit into the bush in Angola. Until trypomastigotes were observed in her son’s CSF, the mother had not received a definitive diagnosis. She was thereafter invited for clinical and laboratory examination. Lumbar puncture and blood tests were then performed: Mott cells were present in her CSF, but no parasite was detected (Figure, panel C). Diagnosis of trypanosomiasis was subsequently supported by positive serologic test results (7). The 2 patients were administered 400 mg/kg eflornithine monotherapy (difluoromethylornithine) daily for 2 weeks (8,9). The clinical outcome was globally satisfactory for the mother. Her serologic tests remained positive 8 months after treatment, which is long but not unusual, and confirms that serologic testing is unreliable for posttherapeutic follow up (4,7). Magnetic resonance imaging showed a substantial decrease of her brain lesions. Her son still exhibited serious neurologic sequelae, although trypomastigotes quickly disappeared from his CSF after treatment. He still had a restricted grip, axial hypotonia, and peripheral hypertonus 2 years later. To date, he has limited contact with his environment and is not able to stand up or eat alone. Altogether, this case is exceptional for 2 reasons: 1) the diagnosis of human African trypanosomiasis in adults and children is very unusual in countries where the disease is not endemic (

Published
2016

10. Peripheral Myelin Protein 22 gene duplication with atypical presentations: A new example of the wide spectrum of Charcot-Marie-Tooth 1A disease

Author

Meriem Tazir, A.-M. Guennoc, Corinne Magdelaine, Laurence Richard, William Camu, Jean-Michel Vallat, Benoît Funalot, Philippe Corcia, Philippe Latour, Stéphane Mathis, Laurent Magy, and Julien Biberon

Subjects
Adult, congenital, hereditary, and neonatal diseases and abnormalities, Pathology, medicine.medical_specialty, Peripheral myelin, Recurrent nerve, Disease, Biology, Charcot-Marie-Tooth Disease, Gene Duplication, Peripheral myelin protein 22, Gene duplication, medicine, Humans, Child, Pathological, Genetics (clinical), Nerve biopsy, medicine.diagnostic_test, Middle Aged, Pedigree, Chromosome 17 (human), Neurology, Pediatrics, Perinatology and Child Health, Female, Neurology (clinical), Myelin Proteins
Abstract

Charcot-Marie-Tooth type 1A (CMT1A) and hereditary neuropathy with liability to pressure palsies (HNPP) are both autosomal-dominant disorders linked to peripheral myelin anomalies. CMT1A is associated with a Peripheral Myelin Protein 22 (PMP22) duplication, whereas HNPP is due to a PMP22 deletion on chromosome 17. In spite of this crucial difference, we report three observations of patients with the 1.4 megabase CMT1A duplication and atypical presentation (electrophysiological, clinical or pathological): a 10 year-old girl with tomaculous lesions on nerve biopsy; a 26 year-old woman with recurrent paresthesiae and block conduction on the electrophysiological study; a 46 year-old woman with transient recurrent nerve palsies mimicking HNPP. These observations highlight the wide spectrum of CMT1A and the overlap between CMT1A and HNPP (both linked to the PMP22 gene), and finally illustrate the complexity of the genotype-phenotype correlations in Charcot-Marie-Tooth diseases.

Published
2014

11. Les critères d’Awaji : les nouveaux critères diagnostiques de la sclérose latérale amyotrophique

Author

William Camu, A.-M. Guennoc, and P. Corcia

Subjects
Weakness, Pediatrics, medicine.medical_specialty, medicine.diagnostic_test, business.industry, Physical examination, Diagnostic marker, medicine.disease, Fasciculation, Clinical research, Neurology, Predictive value of tests, Medicine, Neurology (clinical), Symptom onset, medicine.symptom, Amyotrophic lateral sclerosis, business
Abstract

Amyotrophic lateral sclerosis is the most common motor neuron disorder in adults. Although the diagnosis appears obvious in theory, clinical practice shows the contrary as diagnosis is delayed in many patients; the average time between symptom onset and diagnosis can reach 12 months. The delay can be explained by the variability of the clinical presentation and by the absence of diagnostic markers. In order to standardize diagnosis for enrollment in clinical research, diagnostic criteria for ALS were created and revisited during the last 20 years. In 2006, the Awaji criteria for the diagnosis of ALS were proposed, adding two major points to the diagnostic criteria: electromyography is considered equivalent to clinical examination for the identification of LMN signs and fasciculation potentials resume their prominent place in the diagnosis. Comparisons of the accuracy of the revisited El Escorial and Awaji criteria support improved diagnostic sensitivity without any effect on specificity with the new classification. The only weakness of the new classification involves patients with UMN signs in one region and LMN in two regions; these patients were previously classified as laboratory-supported probable ALS and currently as possible ALS, a lower level of diagnostic certainty. In all other instances the accuracy appears to be improved by the Awaji criteria. Nevertheless, there is a body of evidence suggesting the need for a revision of these new criteria, giving more weight to clinical and complementary findings of UMN involvement. The need to diagnose and treat ALS quickly could be facilitated by the inclusion of complementary investigations that detect UMN signs.

Published
2013

12. Leucoencéphalopathies vasculaires chez des patients sans facteur de risque vasculaire ni mutation de NOTCH3 : description clinique et radiologique

Author

A.-M. Guennoc, Xavier Ayrignac, David Wallon, C. Lebrun-Fresnay, Pierre Labauge, and Eloi Magnin

Subjects
Gynecology, medicine.medical_specialty, Neurology, Multicenter study, business.industry, Cerebral microangiopathy, Disease progression, medicine, Neurology (clinical), business
Abstract

Resume Introduction La presence d’une leucoencephalopathie vasculaire sur l’IRM cerebrale traduit le plus souvent une microangiopathie cerebrale secondaire a la presence de facteurs de risque vasculaire essentiellement representes par l’âge et l’hypertension arterielle. Chez des sujets jeunes et sans facteur de risque vasculaire, une maladie de CADASIL doit etre recherchee. L’objectif de ce travail etait de preciser les caracteristiques cliniques et neuroradiologiques de huit patients sans mutation NOTCH3 dont la severite de la leucoencephalopathie vasculaire n’etait pas expliquee par les facteurs de risque vasculaire. Patients et methode Nous rapportons une serie multicentrique de huit patients. Les criteres d’inclusion etaient leucoencephalopathie stade 3 de la classification de Fazekas, âge inferieur a 70 ans, et absence de mutation du gene NOTCH3 . La presence de facteurs de risque vasculaire severes ou d’atherosclerose averee etait un critere d’exclusion. Les donnees cliniques, neuroradiologiques et les resultats des autres explorations vasculaires ont ete analyses. Resultats Huit patients (quatre femmes) ont ete inclus, cinq n’avaient aucun facteur de risque vasculaire. L’âge de debut moyen etait de 59,5 ans. A l’exception de deux malades presentant un accident ischemique de type lacunaire, le debut des symptomes etait progressif caracterise le plus souvent par une astasie-abasie. Six patients se sont aggraves, avec apparition progressive de troubles cognitifs et de la marche. Un d’entre eux notamment, devenu grabataire, est decede a l’âge de 66 ans de son atteinte neurologique, soit quatre ans apres le debut des symptomes. L’IRM cerebrale objectivait une atrophie precoce chez cinq malades sur huit ; l’atteinte des lobes temporaux (deux sur huit) et des capsules externes (cinq sur huit, asymetrique le plus souvent) etait peu marquee. Quatre patients n’avaient aucune localisation d’atherome. Seul un patient avait une microangiopathie retinienne. Une HTA a ete decouverte chez deux patients. Conclusion La decouverte d’une leucoencephalopathie vasculaire chez un sujet jeune sans facteur de risque vasculaire doit faire realiser un bilan complet a la recherche notamment d’une HTA meconnue afin d’instaurer un traitement correcteur. La presentation clinique est le plus souvent caracterisee par une astasie-abasie. Dans ce contexte, l’IRM cerebrale ne permettant pas de distinguer avec certitude une maladie de CADASIL d’une microangiopathie acquise, le sequencage de 23 exons du gene NOTCH3 doit etre realise.

Published
2013

13. Predictive value of motor evoked potentials in clinically isolated syndrome

Author

A.-M. Guennoc, P. Corcia, Julien Praline, Hélène Blasco, B. de Toffol, and Maud Pallix-Guyot

Subjects
medicine.medical_specialty, Clinically isolated syndrome, Multiple sclerosis, medicine.medical_treatment, Significant difference, General Medicine, Audiology, medicine.disease, Predictive value, Transcranial magnetic stimulation, Neurology, Internal medicine, medicine, Cardiology, Silent period, Optic neuritis, In patient, Neurology (clinical), Psychology
Abstract

Pallix-Guyot M, Guennoc A-M, Blasco H, de Toffol B, Corcia P, Praline J. Predictive value of motor evoked potentials in clinically isolated syndrome. Acta Neurol Scand: 2011: 124: 410–416. © 2011 John Wiley & Sons A/S. Objectives – To assess the predictive role of several measures obtained by transcranial magnetic stimulation (TMS) in patients with clinically isolated syndrome (CIS) for the risk of conversion to multiple sclerosis (MS) during the first 2 years. Materials and methods – We investigated 34 patients with CIS. After 2 years of follow-up and classification into two groups according to MS diagnosis, initial TMS measures were compared to determine their predictive values for conversion to MS. Results – Sixteen patients developed MS. We observed a significant difference between the two groups for contralateral silent period and no significant difference for the central motor conduction time, amplitude ratio, motor threshold, ipsilateral silent period, and the transcallosal conduction time. Conclusions – Contralateral silent period (SP) seems to be a valuable parameter to early distinguish patients who will develop MS or not. This result about SP during CIS has never been described until now. An increased contralateral silent period would predict a conversion to MS with a positive predictive value of 75%, but this result needs to be confirmed in larger groups.

Published
2011

14. Histoire naturelle des leucodystrophies avec mutation EIF2B : étude rétrospective multicentrique de 24 cas adultes

Author

Laetitia Horzinski, Jean-Christophe Antoine, Diana Rodriguez, J. de Seze, L. Peter, F. Blanc, Anne Fogli, Pierre Labauge, Xavier Ayrignac, Cyril Goizet, Sandra Vukusic, Frédéric Sedel, David-Axel Laplaud, Odile Boespflug-Tanguy, A.-M. Guennoc, François Mauguière, E. Sartori, Michael Obadia, Francoise Bouhour, Christian Denier, Cyrille B. Confavreux, and Clarisse Carra-Dalliere

Subjects
Gynecology, 0303 health sciences, medicine.medical_specialty, business.industry, Disease progression, 3. Good health, 03 medical and health sciences, 0302 clinical medicine, Neurology, Multicenter study, medicine, Neurology (clinical), business, 030217 neurology & neurosurgery, 030304 developmental biology
Abstract

Resume Introduction Le syndrome Childhood ataxia with diffuse central nervous system hypomyelination–vanishing white matter disease (CACH–VWM) est une leucodystrophie autosomique recessive decrite initialement chez l’enfant caracterisee par un syndrome cerebellospastique aggrave par des episodes de stress et associee a un aspect cavitaire diffus de la substance blanche a l’IRM. Des mutations des cinq genes du facteur d’initiation de la traduction proteique, eukaryotic initiation factor 2B (EIF2B), ont ete impliquees dans cette pathologie. Le spectre clinique s’est ensuite elargi allant de formes de debut congenital tres severe a des formes de debut adulte tres lentement progressive parfois associee a une insuffisance ovarienne, faisant emerger le concept d’EIF2Bpathies. Methodes Nous rapportons une serie retrospective multicentrique de 24 cas adultes d’EIF2Bpathies. Les donnees cliniques, moleculaires et IRM ont ete collectees. L’evolution clinique a ete objectivee par une reevaluation des patients grâce a des echelles cognitives et de handicap fonctionnel. Resultats L’âge moyen de debut de la maladie etait de 30 ans mais avec un sex-ratio desequilibre (homme/femme = 5/19). La presentation clinique associait, de maniere variable, une ataxie cerebelleuse, une paraparesie spastique, un declin cognitif, une epilepsie ou une insuffisance ovarienne. L’aggravation par des facteurs de stress avait emaille l’evolution chez 33 % des patients. Le pronostic etait severe puisque deux patientes decederent de la maladie ; parmi les survivants, 67 % presentaient une alteration cognitive et 36 % perdirent la marche. Le score EDSS moyen etait de 5,6. L’IRM montrait une atteinte extensive de la substance blanche dans tous les cas, affectant le corps calleux et associee a une atrophie cerebrale dans plus de 90 % des cas et avec un caractere cavitaire dans 83 % des cas. La mutation R113H dans le gene EIF2B5 a l’etat homozygote etait la plus frequemment retrouvee. Conclusion Une EIF2Bpathie doit etre suspectee chez l’adulte devant un tableau de leucopathie diffuse surtout s’il existe une insuffisance ovarienne, des lesions cavitaires ou lorsque l’evolution est emaillee d’aggravations brutales. En l’absence de ces criteres, le contraste initial entre l’atteinte extensive de la substance blanche a l’IRM et la symptomatologie clinique moderee est un bon argument pour demander une recherche de la mutation R113H d’EIF2B5.

Published
2011
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15. Efficacité comparée du Teriflunomide et du Dimethyl-Fumarate : une étude observationnelle française multicentrique

Author

Fabien Rollot, Gilles Edan, Alain Créange, Ivania Patry, A. Al Khedr, T. De Broucker, C. Dupel-Pottier, Céline Labeyrie, Olivier Gout, David Brassat, Chantal Nifle, Emmanuelle Leray, Patrick Vermersch, A.-M. Guennoc, Marc Debouverie, Catherine Lubetzki, Pierre Clavelou, David-Axel Laplaud, Olivier Casez, Yohann Foucher, Jonathan Ciron, Laurent Magy, J. de Seze, Giovanni Castelnovo, Eric Berger, Romain Casey, Olivier Heinzlef, Jean Pelletier, Ayman Tourbah, Laetitia Barbin, Sandrine Wiertlewski, J.-P. Camdessanché, de la part de la Sfsep de l’OFSEP, Bruno Brochet, Thibault Moreau, B. Stankoff, Sandra Vukusic, Gilles-Louis Defer, Christine Lebrun-Frenay, Pierre Labauge, and Bertrand Bourre

Subjects
Epidemiology, Public Health, Environmental and Occupational Health
Abstract

Introduction Le Teriflunomide (TRF) et le Dimethyl-Fumarate (DMF) sont deux traitements approuves de 1re ligne pour la forme remittente de sclerose en plaques (SEP). Cependant, a ce jour, il n’existe aucun essai randomise ni aucune etude observationnelle comparant leur efficacite relative. Objectif Le but de cette etude est de comparer le TRF et le DMF sur des criteres d’efficacite clinique et IRM a partir des donnees observationnelles des 34 centres issus de la cohorte francaise OFSEP. Methodes Les patients SEP inclus dans l’etude avaient une forme remittente de la maladie, etaient âges de 18 a 65 ans, avaient un score EDSS entre 0 et 5,5 a l’inclusion et une IRM dans l’annee precedant l’initiation du traitement. Les donnees ont ete collectees prospectivement a partir de 585 patients traites par TRF et 890 par DMF. Le critere de jugement principal etait la proportion de patients ayant fait au moins une poussee dans l’annee suivant l’initiation du traitement par TRF ou DMF. Trois criteres de jugement secondaires ont aussi ete etudies : la proportion de patients avec au moins une nouvelle lesion T2, la proportion de patients avec au moins une lesion prenant le gadolinium (gado+) et la proportion de patients avec une augmentation du score EDSS apres un an de traitement. L’analyse statistique etait basee sur des scores de propension (IPTW) et une regression logistique. Resultats La comparaison des deux groupes (TRF et DMF) a l’initiation du traitement montre que les patients traites par DMF avaient une duree de maladie plus courte, une proportion augmentee de patients ayant fait une poussee ou plus dans l’annee ou les deux annees precedant la mise en route du traitement et une augmentation du pourcentage de patients avec au moins une lesion gado+. Cependant apres ajustement sur ces facteurs confondant, apres un an de traitement la proportion de patients ayant fait au moins une poussee etait similaire dans le groupe TRF et DMF (20,1 % versus 20,4 %). De la meme facon l’analyse des criteres secondaires IRM et EDSS revelait une efficacite similaire entre les deux groupes apres un an de traitement. Interpretation En France, les neurologues choisissent preferentiellement le DMF comme traitement en premiere intention pour les patients ayant une forme plus active de maladie. Cependant, apres un an de traitement et apres correction pour les facteurs confondants, l’efficacite du DMF et du TRF est similaire en termes de prevention des poussees et de survenue de nouvelle lesion T2 ou de lesion gado+.

Published
2018

16. Le syndrome du défilé thoraco-brachial : une complication post-opératoire inhabituelle

Author

M.-A. Barthez, B. de Courtivon, J. Laulan, A.-M. Guennoc, B. de Toffol, and P. Corcia

Subjects
Gynecology, medicine.medical_specialty, Neurology, Costoclavicular Syndrome, business.industry, Arterial disease, medicine, Postoperative complication, Neurology (clinical), Scoliosis, medicine.disease, business, Thoracic outlet syndrome
Abstract

Resume Introduction Le syndrome du defile thoraco-brachial correspond a une compression des structures vasculo-nerveuses du membre superieur au passage du defile des scalenes par une cote cervicale surnumeraire ou une apophyse transverse de la vertebre C7 proeminente. Les formes neurologiques pures de syndrome du defile thoraco-brachial sont rares et evoluent habituellement lentement et progressivement. Observation Une jeune fille, âgee de 12 ans, developpa dans les suites immediates d’une intervention chirurgicale pour une scoliose, des paresthesies et un deficit moteur de la main droite. L’examen clinique ainsi que l’examen electroneuromyographique etaient tous les deux caracteristiques d’un syndrome du defile thoraco-brachial. Conclusion Le syndrome du defile thoraco-brachial peut compliquer certaines chirurgies notamment lorsque les patients sont installes de facon prolongee en decubitus ventral, les epaules en abduction.

Published
2006

17. Neuromyopathie toxique induite par la ciclosporine

Author

Y. Lebranchu, A. Al-Najjar, P. Corcia, A.-M. Guennoc, A. M. Bergemer-Fouquet, B. de Toffol, and Alain Autret

Subjects
Neurology, Neurology (clinical)
Abstract

Resume Introduction La ciclosporine est un medicament immunosuppresseur majeur dans la prevention de rejet des greffes et dans certaines affections auto-immunes. La prescription de cette molecule est limitee par de nombreux effets parmi lesquels on note des cas de neuropathies et de myopathies, rapportees notamment lorsque la ciclosporine est associee a des medicaments inhibant la co-enzyme A reductase. Observation Nous rapportons le cas d’une patiente de 67 ans qui a developpe quelques mois apres une greffe renale un deficit sensitivo-moteur rapidement evolutif en rapport avec une neuromyopathie. Bien que la ciclosporine fut prescrite seule dans les taux therapeutiques, l’elimination des autres etiologies responsables d’un tel tableau conduisait a privilegier l’hypothese d’une toxicite induite exclusivement par la ciclosporine. Cette hypothese fut confirmee par l’amelioration de l’etat neurologique apres arret de cette prescription. Conclusion Cette observation souligne donc la neurotoxicite potentielle de la ciclosporine meme lorsqu’elle est prescrite en monotherapie aux taux therapeutiques.

Published
2005

18. Infections cérébrales de localisation cérébelleuse induites par le virus JC après traitement par natalizumab : variabilité clinique et radiologique

Author

A.-M. Guennoc, Jérôme De Seze, Irina Sarafiant, Laurent Kremer, Nicolas Collongues, and Stéphane Kremer

Subjects
Gynecology, medicine.medical_specialty, Neurology, business.industry, medicine, Neurology (clinical), business
Abstract

Introduction Les infections cerebrales a JC virus (JCV) apres traitement par natalizumab (NTZ) sont bien connues. Nous decrivons le cas de 3 patients avec presentation clinique et radiologique atypique limitee au cervelet. Observation Il s’agit de 3 patients traites par NTZ presentant un syndrome cerebelleux rapidement progressif. Cas 1 : homme de 36 ans traite depuis 6 ans par NTZ. Trois IRM successives montrent une atrophie cerebelleuse rapidement progressive, des anomalies de signal du cortex et du vermis cerebelleux sans rehaussement, evoquant une neuronopathie des cellules granulaires du cervelet induite par le JCV. Cas 2 : femme de 27 ans traitee par NTZ depuis 4 ans. L’IRM initiale retrouve une minime prise de contraste cerebelleuse aspecifique mais le controle realise apres degradation clinique retrouve l’apparition d’importantes anomalies de signal au niveau du tronc cerebral et des deux hemispheres cerebelleux associees a des prises de contraste et un effet de masse de la fosse posterieure evoquant une leucoencephalopathie multifocale progressive (LEMP). Cas 3 : femme traitee depuis 8 ans par NTZ. Les IRM retrouvent une lesion nodulaire du pedoncule cerebelleux prenant le contraste et dont la taille evolue progressivement compatible avec une LEMP. Dans les 3 cas, le diagnostic d’infection a JCV a pu etre confirme par la ponction lombaire. Discussion Ces 3 cas illustrent la variabilite clinique et surtout IRM des infections a JCV limitees au cervelet. Une attention particuliere doit pouvoir etre portee sur les anomalies localisees en fosse posterieure a l’IRM et le diagnostic de LEMP doit etre evoque malgre les atypies, en raison de l’importante variabilite des resultats d’imagerie. Conclusion Toute imagerie cerebelleuse atypique chez un patient sous NTZ doit faire evoquer et rechercher une LEMP, afin de demarrer une prise en charge precoce pour ameliorer le pronostic.

Published
2016

19. [Awaji criteria: new diagnostic criteria for amyotrophic lateral sclerosis]

Author

A-M, Guennoc, W, Camu, and P, Corcia

Subjects
Adult, Electromyography, Predictive Value of Tests, Amyotrophic Lateral Sclerosis, Diagnostic Techniques, Neurological, Humans, Fasciculation
Abstract

Amyotrophic lateral sclerosis is the most common motor neuron disorder in adults. Although the diagnosis appears obvious in theory, clinical practice shows the contrary as diagnosis is delayed in many patients; the average time between symptom onset and diagnosis can reach 12 months. The delay can be explained by the variability of the clinical presentation and by the absence of diagnostic markers. In order to standardize diagnosis for enrollment in clinical research, diagnostic criteria for ALS were created and revisited during the last 20 years. In 2006, the Awaji criteria for the diagnosis of ALS were proposed, adding two major points to the diagnostic criteria: electromyography is considered equivalent to clinical examination for the identification of LMN signs and fasciculation potentials resume their prominent place in the diagnosis. Comparisons of the accuracy of the revisited El Escorial and Awaji criteria support improved diagnostic sensitivity without any effect on specificity with the new classification. The only weakness of the new classification involves patients with UMN signs in one region and LMN in two regions; these patients were previously classified as laboratory-supported probable ALS and currently as possible ALS, a lower level of diagnostic certainty. In all other instances the accuracy appears to be improved by the Awaji criteria. Nevertheless, there is a body of evidence suggesting the need for a revision of these new criteria, giving more weight to clinical and complementary findings of UMN involvement. The need to diagnose and treat ALS quickly could be facilitated by the inclusion of complementary investigations that detect UMN signs.

Published
2012

20. [Vascular leukoencephalopathy in patients without vascular risk factor or NOTCH3 mutation: clinical and radiological findings]

Author

X, Ayrignac, E, Magnin, D, Wallon, A-M, Guennoc, C, Lebrun-Fresnay, and P, Labauge

Subjects
Male, Receptors, Notch, DNA Mutational Analysis, Hyperhomocysteinemia, Brain, Retinal Vessels, CADASIL, Comorbidity, Middle Aged, Intracranial Arteriosclerosis, Magnetic Resonance Imaging, Diagnosis, Differential, Radiography, Leukoencephalopathies, Risk Factors, Cerebral Small Vessel Diseases, Hypertension, Disease Progression, Humans, Female, France, Atrophy, Receptor, Notch3, Aged
Abstract

White matter lesions seen on MR scan reflect small vessel disease of the brain; increasing age and high blood pressure are the main risk factors. In young patients without vascular risk factors, screening for CADASIL mutation has to be done. Our aim was to describe clinical as well as radiological features of a series of patients without NOTCH3 mutation with severe vascular leukoencephalopathy not explained by the presence of vascular risk factors.Inclusion criteria were grade 3 leukoencephalopathy according to the Fazekas scale, age70years at onset, and negative screening for NOTCH3 gene. Patients with severe vascular risk factors or atherosclerosis were excluded. Clinical and MRI findings were analysed.Eight patients (four men) were included, five did not have any vascular risk factor. Mean age at onset was 59.5years. Initial symptoms were progressive in six cases of eight cases. They consisted of astasia-abasia and progressively worsened; of note one patient died 4years after disease onset. Cerebral MRI disclosed marked atrophy in five patients out of eight, temporal lobe (two out of eight) and external capsule (five out of eight) involvement was moderate. Four patients did not have any other atherosclerosis lesion. Seven out of eight had no retinal microangiopathy. High blood pressure was identified in two patients.The identification of vascular leukoencephalopathy in young patients without any vascular risk factors should lead the clinician to perform a complete work-up to search for treatable conditions including high blood pressure. Patients with vascular leukoencephalopathy usually present with astasia-abasia. In this context, cerebral MRI, cannot perfectly discriminate between patients with CADASIL from those with acquired small-vessel disease of the brain so that sequencing of NOTCH3 gene exons 2-24 is recommended.

Published
2012

21. Predictive value of motor evoked potentials in clinically isolated syndrome

Author

M, Pallix-Guyot, A-M, Guennoc, H, Blasco, B, de Toffol, P, Corcia, and J, Praline

Subjects
Adult, Male, Young Adult, Multiple Sclerosis, Adolescent, Predictive Value of Tests, Disease Progression, Humans, Female, Middle Aged, Evoked Potentials, Motor, Transcranial Magnetic Stimulation, Demyelinating Diseases
Abstract

To assess the predictive role of several measures obtained by transcranial magnetic stimulation (TMS) in patients with clinically isolated syndrome (CIS) for the risk of conversion to multiple sclerosis (MS) during the first 2 years.We investigated 34 patients with CIS. After 2 years of follow-up and classification into two groups according to MS diagnosis, initial TMS measures were compared to determine their predictive values for conversion to MS.Sixteen patients developed MS. We observed a significant difference between the two groups for contralateral silent period and no significant difference for the central motor conduction time, amplitude ratio, motor threshold, ipsilateral silent period, and the transcallosal conduction time.Contralateral silent period (SP) seems to be a valuable parameter to early distinguish patients who will develop MS or not. This result about SP during CIS has never been described until now. An increased contralateral silent period would predict a conversion to MS with a positive predictive value of 75%, but this result needs to be confirmed in larger groups.

Published
2011

22. Brait-Fahn-Schwarz disease: The missing link between ALS and Parkinson's disease

Author

Bertr De Toffol, Paul H. Gordon, Jérémie Belin, A.-M. Guennoc, and Philippe Corcia

Subjects
Oncology, medicine.medical_specialty, Parkinson's disease, business.industry, Disease progression, Disease, medicine.disease, Neurology, Internal medicine, medicine, Radionuclide imaging, Neurology (clinical), Amyotrophic lateral sclerosis, business
Abstract

Dear Editor We recently evaluated a male with Brait-Fahn-Schwarz disease (1), the co-appearance of amyotrophic lateral sclerosis (ALS) and Parkinson's disease (PD). ALS is a syndrome of numerous ca...

Published
2014

23. [Natural history of adult-onset eIF2B-related disorders: a multicentric survey of 24 cases]

Author

C, Carra-Dalliere, L, Horzinski, X, Ayrignac, S, Vukusic, D, Rodriguez, F, Mauguiere, L, Peter, C, Goizet, F, Bouhour, C, Denier, C, Confavreux, M, Obadia, F, Blanc, J, de Seze, F, Sedel, A-M, Guennoc, E, Sartori, D, Laplaud, J-C, Antoine, A, Fogli, O, Boespflug-Tanguy, and P, Labauge

Subjects
Adult, Male, Adolescent, Data Collection, Middle Aged, Cohort Studies, Eukaryotic Initiation Factor-2B, Hereditary Central Nervous System Demyelinating Diseases, Young Adult, Mutation, Disease Progression, Humans, Female, Age of Onset, Child, Retrospective Studies
Abstract

The childhood ataxia with central nervous system hypomyelination-vanishing white matter syndrome (CACH-VWM) was first characterized in children (2-5 years) on clinical and MRI criteria: cerebellospastic signs associated with episodes of rapid deterioration following stress and extensive cavitatingleucoencephalopathy. Causative mutations were found in the five genes encoding the subunits of the eukaryotic initiation factor 2B (eIF2B), involved in protein synthesis and its regulation under cellular stresses. A broad clinical spectrum has been subsequently described from congenital to adult-onset forms leading to the concept of eIF2B-related disorders. Our aim was to describe clinical and brain magnetic resonance imaging characteristics, genetic findings and natural history of patients with adult-onset eIF2B-related disorders.The inclusion criteria were based on the presence of EIF2B mutations and a disease onset after the age of 16 years. One patient with an asymptomatic diagnosis was also included. Clinical and MRI findings were retrospectively recorded in all patients. This multicentric study included 24 patients from 22 families.A sex-ratio imbalance was noted (male/female=5/19). The mean age of onset was 30 years (range 12-62). Initial symptoms were neurologic (n=20), psychiatric (n=3) and ovarian failure (n=6). During follow-up (mean: 11 years, range 2-35 years), two patients died. Of the 22 survivors, 67% showed a decline in their cognitive functions and mean EDSS was 5.6 (range=0-9.5). One case remained asymptomatic. Stress worsened clinical symptoms in 33% of the patients. Magnetic resonance imaging findings consisted of cerebral atrophy (92%), extensive cystic leucoencephalopathy (83%), corpus callosum involvement (92%) and cerebellar (37%) T2-weighted hyperintensities. Most patients (83%) showed mutations in the EIF2B5 gene. The recurrent p.Arg113His-eIF2Be mutation was found at a homozygous state in 58% of the 24 eIF2B-mutated patients.eIF2B-related disorder is probably underestimated as an adult-onset inherited leucoencephalopathy. Cerebral atrophy is constant, whereas the typical vanishing of the white matter can be absent. Functional and cognitive prognosis remains severe. Molecular diagnosis is facilitated for these forms by screening for the recurrent p.Arg113His-eIF2Be mutation.

Published
2010

24. La maladie de Brait-Fahn-Schwarz : le chaînon manquant entre SLA et maladie de Parkinson

Author

Paul H. Gordon, Bertrand de Toffol, A.-M. Guennoc, Philippe Corcia, and Jeremie Belin

Subjects
Neurology, Neurology (clinical)
Abstract

Introduction Auparavant definie comme une affection isolee du motoneurone, la sclerose laterale amyotrophique (SLA) est maintenant consideree comme un syndrome avec un phenotype clinique et des bases neuropathologiques heterogenes. Observation Nous rapportons le cas d’un patient de 74 ans, ancien mecanicien, ayant presente une maladie de Brait-Fahn-Schwarz, une rare association d’une SLA et d’un syndrome parkinsonien, decrite pour la premiere fois en 1973. Cliniquement, ce patient a developpe un syndrome parkinsonien dopa-sensible ressemblant en tous points a une maladie de Parkinson idiopathique, puis plusieurs mois apres, une atteinte progressive des motoneurones central et peripherique interessant les quatre membres et les muscles bulbaires caracteristiques de SLA. Il n’y avait pas d’antecedents familiaux neurologiques. Un Dat-scan a confirme une denervation dopaminergique pre-synaptique bilaterale asymetrique, et l’atteinte du motoneurone peripherique a ete authentifiee a l’electroneuromyogramme. La ponction lombaire etait normale, les anticorps anti-neuronaux etaient negatifs, de meme que la recherche des metaux lourds. Aucune mutation n’a ete identifiee dans les quatre genes principaux lies a la SLA, incluant le gene C9ORF72. Le patient deceda de detresse respiratoire 19 mois apres le debut des symptomes. La famille a decline la realisation d’une autopsie. Discussion Cliniquement, l’atteinte du motoneurone et le syndrome parkinsonien peuvent etre difficiles a demeler : tous deux occasionnent un ralentissement, une rigidite et des chutes : il est donc possible que cette association soit sous-diagnostiquee. Plusieurs etudes neuropathologiques ou imageriques ont documente des atteintes du motoneurone dans des maladies de Parkinson ou des atteintes de la substantia nigra chez des patients atteints de SLA. Conclusion Ce cas elargit le spectre clinique de la SLA, et emet l’hypothese de bases neuropathologiques communes entre la SLA et les syndromes parkinsoniens degeneratifs, dont la maladie de Parkinson idiopathique.

Published
2015

25. Présentation SLA-like d’une myopathie nécrosante à anti-SRP

Author

A.-M. Guennoc, Julien Biberon, Jean-Michel Vallat, Philippe Corcia, Bertrand de Toffol, and Aude Maurousset

Subjects
Neurology, Neurology (clinical)
Abstract

Introduction Nous rapportons le cas d’un patient de 60 ans ayant presente une myopathie necrosante a anticorps anti-SRP dont la presentation clinique initiale evoquait une sclerose laterale amyotrophique (SLA). Observation Un homme de 60 ans se presenta pour tetraparesie d’apparition progressive sur 5 mois. Le testing moteur etait globalement a 3/5, une amyotrophie severe predominait en proximal. Les reflexes etaient vifs aux membres inferieurs malgre l’atrophie. Il existait des signes bulbaires avec troubles de deglutition, voix nasonnee et dysarthrie sans atrophie ni fasciculation de la langue. Il n’y avait aucun signe sensitif ni trouble sphincterien. De rares fasciculations etaient constatees sur les vastes internes. Une sclerose laterale amyotrophique (SLA) etait evoquee. A l’EMG, l’amplitude des potentiels moteurs etait diminuee. Il n’etait pas retrouve de fasciculation en detection. La myographie montrait un trace riche avec des potentiels d’unite motrice (PUM) polyphasiques d’amplitude normale. Le bilan biologique retrouvait une cytolyse predominant sur les ASAT (5N), et des CPK a 5070 UI/L faisant reconsiderer le diagnostic. La positivite des anticorps anti-SRP a 2 reprises sur immunodot et l’histologie necrosante peu inflammatoire au niveau du deltoide posait le diagnostic de myopathie necrosante a anticorps anti-SRP. Le patient fut traite par corticotherapie, immunoglobulines et methotrexate permettant une amelioration rapide des symptomes. Discussion Les myopathies necrosantes a anti-SRP sont amyotrophiantes, d’evolution subaigue, de pronostic reserve, mais potentiellement curables. L’amyotrophie avec ROT preserves, l’atteinte de la motricite bulbaire peuvent mimer une SLA. L’absence de critere EMG en faveur de celle-ci et l’elevation importante des CPK, doivent faire evoquer une myopathie necrosante. Le diagnostic est confirme par l’histologie et la positivite des anticorps specifiques. Conclusion L’augmentation importante des CPK est rare dans la SLA et doit faire reconsiderer le diagnostic. Elle doit faire envisager le cadre des myopathies necrosantes puisqu’il s’agit de pathologies accessibles a un traitement.

Published
2015

26. Genetic and temperature sex determination in the turbot Scophthalmus maximus

Author

Pierrick Haffray, Alexis Fostier, Yann Guiguen, E. Lebègue, S. Jeu, M. Guennoc, Jean-François Baroiller, France Turbot - Marinove, Station commune de Recherches en Ichtyophysiologie, Biodiversité et Environnement (SCRIBE), Institut National de la Recherche Agronomique (INRA), Centre de Coopération Internationale en Recherche Agronomique pour le Développement (Cirad), and Syndicat des Sélectionneurs Avicoles et Aquacoles Français (SYSAAF)

Subjects
Genetics, biology, Détermination du sexe, medicine.drug_class, Reproduction, [SDV]Life Sciences [q-bio], Alpha (ethology), Aquatic Science, Turbot, biology.organism_classification, Androgen, Body weight, Poisson, Température, Scophthalmus, Human fertilization, Animal science, Scophthalmus maximus, medicine, [INFO]Computer Science [cs], M12 - Production de l'aquaculture, Sex ratio, ComputingMilieux_MISCELLANEOUS
Abstract

Genetic (GSD) and temperature sex determination (TSD) have been investigated in the turbot, Scophthalmus maximus using sex inversed males (androgen treated males=ATM) and females (oestradiol treated female-s= OTF). ATM and OTF have been produced through feeding treatments using 17[alpha]-methyltestosterone and 17[beta]-oestradiol at a dosage of 3 or 5 mg/kg food starting from 35 days post fertilization (dpf) during periods of 500 or 700° days. 16 ATM and 6 OTF were mated with normal females or males. The 22 resulting progenies and 11 control families were reared at 17°C. For TSD evaluation, parts of 8 of these 33 families (2 from ATM and 6 from controls) have been reared at 15°C, 17°C or 24°C from 35 dpf to 100 dpf. Then all progenies (GSD and TSD) have been reared at 17°C until 11 months old (100 g body weight) for the sex ratio assessment. For 27 progenies reared at 17°C, sex ratios perfectly fit in with the hypothesis of a male homogamety ZZ/ZW system (X-square test; P

Published
2006

27. [Thoracic outlet syndrome: an unusual postoperative complication]

Author

P, Corcia, A-M, Guennoc, M-A, Barthez, B, de Courtivon, B, de Toffol, and J, Laulan

Subjects
Adult, Thoracic Outlet Syndrome, Postoperative Complications, Time Factors, Scoliosis, Posture, Neural Conduction, Prone Position, Humans, Female
Abstract

Introduction. Neurogenic Thoracic Outlet Syndrome (NTOS) is a chronic lower trunk brachial plexus entrapment caused by a cervical rib or a fibrous band. True NTOS is rare and progresses usually slowly. Case report. A 12-year-old girl complained of numbness and weakness of the right upper limb immediately after an orthopedic surgical procedure for scoliosis. Neurological and neurophysiological features were both consistent with a neurogenic thoracic outlet syndrome (NTOS).This observation illustrates the risk of NTOS after certain surgical procedures, especially when a prolonged prone position with abducted shoulders is required.

Published
2006

28. [Cyclosporin-induced toxic neuromyopathy]

Author

A-M, Guennoc, P, Corcia, A, Al-Najjar, A-M, Bergemer-Fouquet, Y, Lebranchu, B, de Toffol, and A, Autret

Subjects
Graft Rejection, Nerve Fibers, Electromyography, Cyclosporine, Neural Conduction, Humans, Coenzyme A, Female, Neuromuscular Diseases, Middle Aged, Kidney Transplantation, Immunosuppressive Agents
Abstract

Cyclosporine is an immunosuppressive treatment whose side effects limit its usefulness. Among neurological side effects, neuropathies or myopathies have been reported, specially inpatients given combinations of cyclosporine with co-enzyme A reductase inhibitors.We report here the case of a 67-year-old woman who developed few months after a kidney graft sensorimotor disorders which progressed rapidly. Since all etiologies of such a disorder were ruled out, the hypothesis of toxicity exclusively induced by cyclosporine was suggested and confirmed by the improvement observed after its withdrawal.This observation highlights the fact that cyclosporine may induce neuromyopathies even when given alone at the therapeutic dosage.

Published
2005

30. [Demyelinating neuropathy and Sjögren's syndrome: a diagnostic pitfall]

Author

A M, Guennoc, P, Corcia, T, Maisonobe, T, Lefrancq, B, de Toffol, and A, Autret

Subjects
Diagnosis, Differential, Male, Sjogren's Syndrome, Brain, Humans, Peripheral Nervous System Diseases, Aged, Demyelinating Diseases
Abstract

Neuropathies induced by Sjögren's syndrome (SS) are usually axonal. Nevertheless some demyelinating neuropathies have been described in patients with SS. To date, the relationship between demyelinating neuropathies and SS remains imprecise.A 75 year-old man presented with a chronic history of sensory disturbances linked to demyelinating neuropathy. Electroneuromyography revealed a demyelinating neuropathy and complementary tests revealed both Sjögren's syndrome (SS) and HMSN IA.We suggested that an inherited affection might be researched before considering that demyelinating neuropathy might be a form of peripheral nervous system involvement in SS.

Published
2004

31. Neurofascin is a glial receptor for the paranodin/Caspr-contactin axonal complex at the axoglial junction

Author

Catherine Lubetzki, Catherine Faivre-Sarrailh, Natalia Denisenko-Nehrbass, Frank J. Gunn-Moore, A.-M. Guennoc, G. Barbin, Steven Tait, Peter J. Brophy, Jean-Antoine Girault, and Perrine Charles

Subjects
Nervous system, Macromolecular Substances, NFASC, Cell Adhesion Molecules, Neuronal, Receptors, Cell Surface, CHO Cells, Biology, Transfection, Models, Biological, Nerve Fibers, Myelinated, General Biochemistry, Genetics and Molecular Biology, 03 medical and health sciences, 0302 clinical medicine, Contactins, Paranodal junction, Cricetinae, medicine, Cell Adhesion, Animals, Protein Isoforms, Nerve Growth Factors, Axon, Cell adhesion, 030304 developmental biology, 0303 health sciences, Membrane Glycoproteins, Agricultural and Biological Sciences(all), Cell adhesion molecule, Biochemistry, Genetics and Molecular Biology(all), Neuropeptides, Brain, Axolemma, Axons, Coculture Techniques, Cell biology, Protein Structure, Tertiary, Rats, medicine.anatomical_structure, nervous system, General Agricultural and Biological Sciences, Cell Adhesion Molecules, Neuroglia, 030217 neurology & neurosurgery, Protein Binding
Abstract

In myelinated fibers of the vertebrate nervous system, glial-ensheathing cells interact with axons at specialized adhesive junctions, the paranodal septate-like junctions [1]. The axonal proteins paranodin/Caspr and contactin form a cis complex in the axolemma at the axoglial adhesion zone, and both are required to stabilize the junction [2, 3]. There has been intense speculation that an oligodendroglial isoform of the cell adhesion molecule neurofascin, NF155, expressed at the paranodal loop [4, 5] might be the glial receptor for the paranodin/Caspr-contactin complex, particularly since paranodin/Caspr and NF155 colocalize to ectopic sites in the CNS of the dysmyelinated mouse Shiverer mutant [5]. We report that the extracellular domain of NF155 binds specifically to transfected cells expressing the paranodin/Caspr-contactin complex at the cell surface. This region of NF155 also binds the paranodin/Caspr-contactin complex from brain lysates in vitro. In support of the functional significance of this interaction, NF155 antibodies and the extracellular domain of NF155 inhibit myelination in myelinating cocultures, presumably by blocking the adhesive relationship between the axon and glial cell. These results demonstrate that the paranodin/Caspr-contactin complex interacts biochemically with NF155 and that this interaction is likely to be biologically relevant at the axoglial junction.

Published
2002

34. [Primary lateral sclerosis with breast cancer, a potential paraneoplastic neurological syndrome]

Author

P, Corcia, J, Honnorat, A M, Guennoc, B, de Toffol, and A, Autret

Subjects
Tamoxifen, Brain, Humans, Antineoplastic Agents, Breast Neoplasms, Female, Motor Neuron Disease, Combined Modality Therapy, Magnetic Resonance Imaging, Aged, Paraneoplastic Syndromes, Nervous System
Abstract

Few reports indicate that motor neuron diseases may have paraneoplastic origin. A 70 year-old woman suffering from progressive upper motor neuron disease is presented. Laboratory, radiological and neurophysiologic studies were compatible with primary lateral sclerosis. Six years later a routine screening led to the discovery of a breast cancer, suggesting that the upper motor neuron syndrome could be paraneoplastic. So, in female patients with primary lateral sclerosis, a mammography should be recommended to search for breast cancer.

Published
2000

35. Induction and synchronisation of spawning in cultivated turbot (Scophthalmus maximus L.) broodstock by implantation of a sustained-release GnRH-a pellet

Author

M. Guennoc, C Mugnier, Alexis Fostier, Bernard Breton, E. Lebègue, Station commune de Recherches en Ichtyophysiologie, Biodiversité et Environnement (SCRIBE), and Institut National de la Recherche Agronomique (INRA)

Subjects
medicine.medical_specialty, media_common.quotation_subject, Broodstock, Aquatic Science, OEUFS DE POISSON, 03 medical and health sciences, SYNCHRONISATION, Animal science, Human fertilization, Internal medicine, medicine, 14. Life underwater, Ovulation, ComputingMilieux_MISCELLANEOUS, 030304 developmental biology, media_common, 0303 health sciences, biology, Hatching, PLEURONECTIDAE SCOPHTHALMUS MAXIMUS, Scophthalmidae, 04 agricultural and veterinary sciences, biology.organism_classification, Scophthalmus, Turbot, Endocrinology, [SDV.SA.STP]Life Sciences [q-bio]/Agricultural sciences/Sciences and technics of fishery, 040102 fisheries, 0401 agriculture, forestry, and fisheries, Reproduction
Abstract

Cultivated turbot were successfully spawned using sustained-release pellets containing a gonadotropin-releasing hormone analogue (GnRH-a), [ d -Ala 6 –Pro 9 -Net]-luteinising hormone releasing hormone. Significant synchronisation of females was obtained, reducing the spawning season by about half. The number of spawning females was increased, and all sexually mature females ovulated. The delay between ovulations decreased from more than 2 days in controls to about 36 h in GnRH-a treated fish. The mean batch volume increased from 144 to 214 ml, but total volume of eggs for the whole spawning season did not change. At a production scale, fertilisation rate was significantly lower in the GnRH-a group than in the control (76.6 and 89.5%, respectively). Hatching rates were not significantly different between experimental groups.

Published
2000

36. [Acute demyelinating motor neuropathy: an atypical form of the Guillain-Barre syndrome?]

Author

P, Corcia, A, Beaume, A M, Guennoc, B, de Toffol, J L, Preud'homme, and A, Autret

Subjects
Adult, Electrophysiology, Male, Neurology, Acute Disease, Campylobacter Infections, Immunization, Passive, Polyradiculoneuropathy, Humans, Motor Neuron Disease, Demyelinating Diseases
Abstract

A 33-year-old man presented an acute motor demyelinating neuropathy following Campylobacter jejuni enteritis. The patient was improved with an IgIV treatment. Clinical features and course time were compatible with the diagnosis of a Guillain-Barré syndrome. The electrophysiologic studies were in favor of multifocal motor neuropathy with conduction blocks. We discuss the nosologic group of this neuropathy.

Published
1999

37. Bilateral hand amyotrophy with PMP-22 gene deletion

Author

A.-M. Guennoc, M. C. Malinge, B. de Toffol, P. Corcia, A. Gochard, and Julien Praline

Subjects
Genetics, Pathology, medicine.medical_specialty, business.industry, Middle Aged, Motor neuron, Gene deletion, Hand, Amyotrophy, medicine.disease, Phenotype, medicine.anatomical_structure, Bilateral hand weakness, Neurology, Demyelinating neuropathy, Humans, Medicine, Female, Medical history, Neurology (clinical), Hereditary Sensory and Motor Neuropathy, business, Gene, Gene Deletion, Myelin Proteins
Abstract

Hereditary neuropathy with liability to pressure palsies (HNPP) phenotypes are heterogeneous. We report the case of a 52-year-old woman without medical history, who complained of bilateral hand weakness suggestive first of a motor neuron disorder. The presence of a diffuse predominant distal demyelinating neuropathy suggested a deletion of PMP-22 gene, which was confirmed by genetic analysis. This case report underlines a novel phenotype related to the deletion of PMP-22 gene.

Published
2007

38. Improvement of a CIDP associated with hepatitis C virus infection using antiviral therapy

Author

P. Corcia, D. Barbereau, B. de Toffol, Y. Bacq, and A.-M. Guennoc

Subjects
Male, medicine.medical_specialty, viruses, Hepatitis C virus, Chronic inflammatory demyelinating polyneuropathy, Interferon alpha-2, medicine.disease_cause, Antiviral Agents, Gastroenterology, Virus, Polyethylene Glycols, chemistry.chemical_compound, Pharmacotherapy, Internal medicine, Ribavirin, Humans, Medicine, business.industry, Remission Induction, Interferon-alpha, virus diseases, Polyradiculoneuropathy, Hepatitis C, Hepatitis C, Chronic, Middle Aged, medicine.disease, Recombinant Proteins, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating, chemistry, Immunology, Drug Therapy, Combination, Neurology (clinical), Viral disease, business
Abstract

A 57-year-old man with chronic inflammatory demyelinating polyneuropathy associated with hepatitis C virus infection was treated successfully with the combination of peginterferon-alpha-2b and ribavirin. Viral eradication was confirmed during the 4th week of treatment and was followed 3 weeks later by neurologic improvement. The patient resumed normal activity 1 year after the therapy was completed.

Published
2004

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