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26 results on '"Delmont, Emilien"'

4. Clinical and electrophysiological characteristics of women with X‐linked Charcot–Marie–Tooth disease

Author

Barbat du Closel, Luce, primary, Bonello‐Palot, Nathalie, additional, Péréon, Yann, additional, Echaniz‐Laguna, Andoni, additional, Camdessanche, Jean Philippe, additional, Nadaj‐Pakleza, Aleksandra, additional, Chanson, Jean‐Baptiste, additional, Frachet, Simon, additional, Magy, Laurent, additional, Cassereau, Julien, additional, Cintas, Pascal, additional, Choumert, Ariane, additional, Devic, Perrine, additional, Leonard Louis, Sarah, additional, Gravier Dumonceau, Robinson, additional, Delmont, Emilien, additional, Salort‐Campana, Emmanuelle, additional, Bouhour, Françoise, additional, Latour, Philippe, additional, Stojkovic, Tanya, additional, and Attarian, Shahram, additional

Published
2023
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6. Anti‐disialosyl‐immunoglobulin M chronic autoimmune neuropathies: a nationwide multicenter retrospective study

Author

Peillet, Claire, primary, Adams, David, additional, Attarian, Shahram, additional, Bouhour, Françoise, additional, Cauquil, Cécile, additional, Cassereau, Julien, additional, Chanson, Jean‐Baptiste, additional, Cintas, Pascal, additional, Creange, Alain, additional, Delmont, Emilien, additional, Fargeot, Guillaume, additional, Genestet, Steeve, additional, Gueguen, Antoine, additional, Kaminsky, Anne Laure, additional, Kuntzer, Thierry, additional, Labeyrie, Céline, additional, Michaud, Maud, additional, Pereon, Yann, additional, Puma, Angela, additional, Viala, Karine, additional, Chretien, Pascale, additional, Adam, Clovis, additional, and Echaniz‐Laguna, Andoni, additional

Published
2022
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8. Phenotypical variability and atypical presentations in a French cohort of Andersen–Tawil syndrome

Author

Villar‐Quiles, Rocio Nur, primary, Sternberg, Damien, additional, Tredez, Grégoire, additional, Beatriz Romero, Norma, additional, Evangelista, Teresinha, additional, Lafôret, Pascal, additional, Cintas, Pascal, additional, Sole, Guilhem, additional, Sacconi, Sabrina, additional, Bendahhou, Said, additional, Franques, Jérôme, additional, Cances, Claude, additional, Noury, JB, additional, Delmont, Emilien, additional, Blondy, Patricia, additional, Perrin, Laurence, additional, Hezode, Marianne, additional, Fournier, Emmanuel, additional, Fontaine, Bertrand, additional, Stojkovic, Tanya, additional, and Vicart, Savine, additional

Published
2022
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10. Genotype–phenotype correlation in French patients with myelin protein zero gene‐related inherited neuropathy

Author

Subréville, Marie, primary, Bonello‐Palot, Nathalie, additional, Yahiaoui, Douniazed, additional, Beloribi‐Djefaflia, Sadia, additional, Fernandes, Sara, additional, Stojkovic, Tanya, additional, Cassereau, Julien, additional, Péréon, Yann, additional, Echaniz‐Laguna, Andoni, additional, Violleau, Marie‐Hélène, additional, Soulages, Antoine, additional, Louis, Sarah Léonard, additional, Masingue, Marion, additional, Magot, Armelle, additional, Delmont, Emilien, additional, Sacconi, Sabrina, additional, Adams, David, additional, Labeyrie, Céline, additional, Genestet, Steeve, additional, Noury, Jean‐Baptiste, additional, Chanson, Jean‐Baptiste, additional, Lévy, Nicolas, additional, Juntas‐Morales, Raul, additional, Tard, Céline, additional, Sole, Guilhem, additional, and Attarian, Shahram, additional

Published
2021
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11. Charcot–Marie–Tooth disease misdiagnosed as chronic inflammatory demyelinating polyradiculoneuropathy: An international multicentric retrospective study

Author

Hauw, Fabien, primary, Fargeot, Guillaume, additional, Adams, David, additional, Attarian, Shahram, additional, Cauquil, Cécile, additional, Chanson, Jean‐Baptiste, additional, Créange, Alain, additional, Gendre, Thierry, additional, Deiva, Kumaran, additional, Delmont, Emilien, additional, Francou, Bruno, additional, Genestet, Steeve, additional, Kuntzer, Thierry, additional, Latour, Philippe, additional, Le Masson, Gwendal, additional, Magy, Laurent, additional, Nardin, Clotilde, additional, Ochsner, François, additional, Sole, Guilhem, additional, Stojkovic, Tanya, additional, Maisonobe, Thierry, additional, Tard, Céline, additional, Van den Berghe, Peter, additional, and Echaniz‐Laguna, Andoni, additional

Published
2021
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12. Charcot-Marie-Tooth disease misdiagnosed as chronic inflammatory demyelinating polyradiculoneuropathy: An international multicentric retrospective study.

Author

UCL - SSS/IONS - Institute of NeuroScience, UCL - (SLuc) Service de neurologie, UCL - (SLuc) Centre de référence neuromusculaire, Hauw, Fabien, Fargeot, Guillaume, Adams, David, Attarian, Shahram, Cauquil, Cécile, Chanson, Jean-Baptiste, Créange, Alain, Gendre, Thierry, Deiva, Kumaran, Delmont, Emilien, Francou, Bruno, Genestet, Steeve, Kuntzer, Thierry, Latour, Philippe, Le Masson, Gwendal, Magy, Laurent, Nardin, Clotilde, Ochsner, François, Sole, Guilhem, Stojkovic, Tanya, Maisonobe, Thierry, Tard, Céline, Van den Bergh, Peter, Echaniz-Laguna, Andoni, UCL - SSS/IONS - Institute of NeuroScience, UCL - (SLuc) Service de neurologie, UCL - (SLuc) Centre de référence neuromusculaire, Hauw, Fabien, Fargeot, Guillaume, Adams, David, Attarian, Shahram, Cauquil, Cécile, Chanson, Jean-Baptiste, Créange, Alain, Gendre, Thierry, Deiva, Kumaran, Delmont, Emilien, Francou, Bruno, Genestet, Steeve, Kuntzer, Thierry, Latour, Philippe, Le Masson, Gwendal, Magy, Laurent, Nardin, Clotilde, Ochsner, François, Sole, Guilhem, Stojkovic, Tanya, Maisonobe, Thierry, Tard, Céline, Van den Bergh, Peter, and Echaniz-Laguna, Andoni

Abstract

Charcot-Marie-Tooth (CMT) disease, an untreatable hereditary polyneuropathy, may mimic chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), a treatable neuropathy. In this retrospective study, we analyzed the characteristics of CMT patients misdiagnosed as CIDP at 16 university hospitals in three countries, compared these patients with a reference group of CIDP patients, and estimated the cost of misdiagnosis. Among 1104 CIDP cases, we identified 35 CMT patients misdiagnosed as CIDP (3.2%). All were initially diagnosed with definite or probable CIDP (European Federation of Neurological Societies/Peripheral Nerve Society criteria), and mutations in PMP22, MPZ, and 10 other CMT genes were found in 34%, 31%, and 35% of cases, respectively. In comparison with a reference group of 35 CIDP patients, CMT patients were younger (median age at disease onset = 39 vs. 56 years) and more frequently had motor weakness at disease onset (80% vs. 29%), hearing loss (14% vs. 0%), normal brachial plexus imaging (70% vs. 40%), lower cerebrospinal fluid protein content (median = 0.5 vs. 0.8 g/L), and lower treatment response (20% vs. 69%). Treatment cost in these 35 misdiagnosed patients was estimated at 4.6 million euros (M€), whereas the cost of CMT genetic analysis in 1104 patients was estimated at 2.7 M€. In this study, 35 of 1104 (3.2%) patients initially diagnosed with CIDP had CMT. Importantly, the cost of treating these 35 misdiagnosed patients was significantly higher than the cost of performing CMT genetic analysis in 1104 patients (4.6 M€ vs. 2.7 M€), suggesting that CMT genetic investigations should be more widely used before diagnosing CIDP.

Published
2021

13. Selective inhibition of anti‐MAG IgM autoantibody binding to myelin by an antigen‐specific glycopolymer

Author

Aliu, Butrint, primary, Demeestere, Delphine, additional, Seydoux, Emilie, additional, Boucraut, José, additional, Delmont, Emilien, additional, Brodovitch, Alexandre, additional, Oberholzer, Thomas, additional, Attarian, Shahram, additional, Théaudin, Marie, additional, Tsouni, Pinelopi, additional, Kuntzer, Thierry, additional, Derfuss, Tobias, additional, Steck, Andreas J., additional, Ernst, Beat, additional, Herrendorff, Ruben, additional, and Hänggi, Pascal, additional

Published
2020
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14. Quantitative muscle MRI study of patients with sporadic inclusion body myositis

Author

Ansari, Behnaz, primary, Salort‐Campana, Emmanuelle, additional, Ogier, Augustin, additional, Le Troter PhD, Arnaud, additional, De Sainte Marie, Benjamin, additional, Guye, Maxime, additional, Delmont, Emilien, additional, Grapperon, Aude‐Marie, additional, Verschueren, Annie, additional, Bendahan, David, additional, and Attarian, Shahram, additional

Published
2020
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17. Hereditary neuropathy with liability to pressure palsy in patients under 30 years old: Neurophysiological data and proposed electrodiagnostic criteria

Author

Robert-Varvat, Florence, primary, Jousserand, Guillemette, additional, Bouhour, Françoise, additional, Vial, Christophe, additional, Cintas, Pascal, additional, Echaniz-Laguna, Andoni, additional, Delmont, Emilien, additional, Clavelou, Pierre, additional, Chauplannaz, Guy, additional, Jomir, Laurent, additional, Pereon, Yann, additional, Leonard-Louis, Sarah, additional, Manel, Veronique, additional, Antoine, Jean-Christophe, additional, Lacour, Arnaud, additional, and Camdessanche, Jean-Philippe, additional

Published
2017
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23. Molecular Imaging in CANVAS: A Contribution for Differential Diagnosis?

Author

Horowitz T, Guedj E, Eusebio A, Fluchère F, Azulay JP, Delmont E, and Grimaldi S

Subjects
Humans, Male, Diagnosis, Differential, Middle Aged, Female, Aged, Molecular Imaging methods, Multiple System Atrophy diagnostic imaging, Multiple System Atrophy metabolism, Multiple System Atrophy diagnosis, Positron-Emission Tomography methods, Fluorodeoxyglucose F18, Brain diagnostic imaging, Brain metabolism, Dopamine Plasma Membrane Transport Proteins metabolism, Machado-Joseph Disease diagnostic imaging, Machado-Joseph Disease diagnosis, Machado-Joseph Disease metabolism, 3-Iodobenzylguanidine, Tomography, Emission-Computed, Single-Photon methods
Abstract

Background: Phenotypes of CANVAS are increasingly diversified, including bradykinesia and dysautonomia, so that its primary differential diagnoses are multiple system atrophy-cerebellar type (MSA-c), and spinocerebellar ataxia type 3 (SCA3). This case series aims to highlight key molecular imaging findings in CANVAS., Cases: We report a case series of six patients with CANVAS who underwent nuclear medicine examinations in our center and 13 patients from the literature. These include 18 F-FDG brain positron emission tomography (PET), single photon emission computed tomography (SPECT) of dopamine transporter (DaT) activity, and 123 I-MIBG cardiac scintigraphy of noradrenergic transmission., Conclusions: In CANVAS, 18 F-FDG brain PET mainly shows cerebellar hypometabolism, with preserved brainstem and striatum metabolism, contrasting with SCA3 and MSA-c. Dopaminergic denervation on scintigraphy seems to be associated with clinical parkinsonism, ranging from normal to severely impaired DaT SPECT. Additionally, 123 I-MIBG cardiac scintigraphy might show denervation in CANVAS, similar to SCA3, but not in most MSA-c patients., (© 2024 The Authors. Movement Disorders Clinical Practice published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.)

Published
2024
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24. Longitudinal follow-up and prognostic factors in nitrous oxide-induced neuropathy.

Author

Fortanier E, Delmont E, Corazza G, Kouton L, Micallef J, and Attarian S

Subjects
Humans, Male, Female, Adult, Young Adult, Retrospective Studies, Prognosis, Longitudinal Studies, Follow-Up Studies, Adolescent, Neural Conduction physiology, Neural Conduction drug effects, Nitrous Oxide adverse effects, Nitrous Oxide administration & dosage, Peripheral Nervous System Diseases chemically induced, Peripheral Nervous System Diseases physiopathology, Peripheral Nervous System Diseases diagnosis
Abstract

Background and Aim: Recreational use of nitrous oxide (N 2 O) has been associated with the development of severe nitrous oxide-induced neuropathy (N 2 On). Follow-up of these patients poses challenges, and their clinical progression remains largely unknown. The identification of prognostic factors is made difficult by the lack of standardized longitudinal assessments in most studies. The objective was to document the course of neuropathy through systematic follow-up assessments in N 2 On patients to identify prognostic factors for persistent disability after 6 months., Methods: We gathered demographic, clinical, biological, and electrophysiological data from N 2 On patients hospitalized in the Referral center in Marseille, both at baseline and during a standardized follow-up assessment at 6 months., Results: We retrospectively included 26 N 2 On patients (mean age 22.6 ± 4.4). Significant improvements were observed in all main clinical scores including Rankin, ONLS, and MRC testing (p < .01). Electrophysiological studies (EDX) revealed a predominantly motor neuropathy with marked reduction in CMAP in the lower limbs at baseline, and no significant improvement in motor parameters (p = .543). Rankin score at 6 months correlated with the initial weekly N 2 O consumption (r = .43, p = .03) and the CMAP sum score in the lower limbs at the first EDX (r = -.47, p = .02). Patients with and without myelitis showed similar Rankin and ONLS score after 6 months., Interpretation: The clinical course generally improved favorably at 6 months with notable amelioration in the primary disability scores, sensory deficits, and ataxia. However, distal motor impairment associated with peripheral neuropathy persisted, with distal axonal loss emerging as the main prognostic factor for long-term disability in these young patients., (© 2024 Peripheral Nerve Society.)

Published
2024
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25. Successful autologous hematopoietic stem cell transplantation in a refractory anti-Caspr1 antibody nodopathy.

Author

Afanasiev V, Tsouni P, Kuntzer T, Cairoli A, Delmont E, Vallat JM, Devaux J, and Théaudin M

Subjects
Female, Humans, Child, Preschool, Middle Aged, Rituximab therapeutic use, Axons pathology, Immunoglobulin G, Autoantibodies, Hematopoietic Stem Cell Transplantation, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating therapy
Abstract

Aim: Autoimmune nodopathies have specific clinicopathologic features, antibodies directed against nodal proteins (neurofascin 186) or paranodal proteins (neurofascin 155, contactin 1, contactin-associated protein 1 (Caspr1)), and usually have a poor response to first-line therapies for chronic inflammatory demyelinating polyradiculoneuropathy. Anti-Caspr1 nodopathy treated with autologous hematopoietic stem cell transplantation (AHSCT) has not been previously reported., Methods: We report the first case of an anti-Caspr1 antibody-positive nodopathy refractory to high-intensity immunosuppressive treatment, including rituximab, that responded dramatically to AHSCT., Results: A 53-year-old woman presented with a rapidly progressive generalized ataxic, painful motor, and inflammatory neuropathy supported by neurophysiologic and MRI studies. Initial tests for antibodies to nodal/paranodal proteins were negative. She was treated with multiple courses of intravenous immunoglobulin and methylprednisolone, plasma exchange, rituximab, and cyclophosphamide without significant clinical benefit. Repeated testing for antibodies to nodal/paranodal proteins yielded a positive result for anti-Caspr1/IgG4 isotype antibodies. Given the poor response to multiple high intensity treatments and the relatively young age of the patient, we decided to perform AHSCT at 30 months post-onset. Immediately after AHSCT, she stopped all immunomodulatory or immunosuppressive therapy. The Overall Neuropathy Limitation Score improved from 8/12 to 4/12 at 6 months post-AHSCT. At 3 months post-AHSCT, IgG4 against Caspr1 was negative and no reactivity against paranodes could be detected., Conclusion: We report a particularly severe anti-Caspr1 antibody autoimmune nodopathy that responded dramatically to AHSCT. Although the rarity of the disease limits the possibility of larger studies, AHSCT may be a valuable therapy in treatment-refractory cases., (© 2023 Peripheral Nerve Society.)

Published
2024
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26. A clinical pattern-based etiological diagnostic strategy for sensory neuronopathies: a French collaborative study.

Author

Camdessanché JP, Jousserand G, Franques J, Pouget J, Delmont E, Créange A, Kuntzer T, Maisonobe T, Abba K, and Antoine JC

Subjects
Adult, Aged, Female, France epidemiology, Humans, Male, Middle Aged, Paraneoplastic Polyneuropathy diagnosis, Paraneoplastic Polyneuropathy epidemiology, Paraneoplastic Polyneuropathy etiology, Polyneuropathies epidemiology, Cooperative Behavior, Polyneuropathies diagnosis, Polyneuropathies etiology
Abstract

Sensory neuronopathies (SNNs) encompass paraneoplastic, infectious, dysimmune, toxic, inherited, and idiopathic disorders. Recently described diagnostic criteria allow SNN to be differentiated from other forms of sensory neuropathy, but there is no validated strategy based on routine clinical investigations for the etiological diagnosis of SNN. In a multicenter study, the clinical, biological, and electrophysiological characteristics of 148 patients with SNN were analyzed. Multiple correspondence analysis and logistic regression were used to identify patterns differentiating between forms of SNNs with different etiologies. Models were constructed using a study population of 88 patients and checked using a test population of 60 cases. Four patterns were identified. Pattern A, with an acute or subacute onset in the four limbs or arms, early pain, and frequently affecting males over 60 years of age, identified mainly paraneoplastic, toxic, and infectious SNN. Pattern B identified patients with progressive SNN and was divided into patterns C and D, the former corresponding to patients with inherited or slowly progressive idiopathic SNN with severe ataxia and electrophysiological abnormalities and the latter to patients with idiopathic, dysimmune, and sometimes paraneoplastic SNN with a more rapid course than in pattern C. The diagnostic strategy based on these patterns correctly identified 84/88 and 58/60 patients in the study and test populations, respectively., (© 2012 Peripheral Nerve Society.)

Published
2012
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