Search results for: Emilien Delmont

Items from 1 to 20 out of 41 results

article

Electrophysiological features of the peripheral neuropathy in patients with pathologic biallelic RFC1 repeat expansions

Claudia Calezis, Nathalie Bonello‐Palot, Annie Verschueren, Jean‐Philippe Azulay, more

Introduction/Aims Cerebellar ataxia, neuropathy, vestibular areflexia syndrome (CANVAS) is caused by RFC1 expansions. Sensory neuronopathy, polyneuropathy, and involvement of motor, autonomic, and cranial nerves have all been described with RFC1 expansions. We aimed to describe the electrodiagnostic features of patients with RFC1 expansions through multimodal electrophysiological investigations....

article

Longitudinal follow‐up and prognostic factors in nitrous oxide‐induced neuropathy

Etienne Fortanier, Emilien Delmont, Giovanni Corazza, Ludivine Kouton, more

Journal of the Peripheral Nervous System > 29 > 2 > 252 - 261

Background and Aim Recreational use of nitrous oxide (N₂O) has been associated with the development of severe nitrous oxide‐induced neuropathy (N₂On). 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...

article

Factors affecting the topography of nitrous oxide‐induced neurological complications

Eva Sole Cruz, Etienne Fortanier, Frederic Hilezian, Adil Maarouf, more

European Journal of Neurology > 31 > 7 > n/a - n/a

Background The factors underlying the topography of nitrous oxide (N₂O)‐induced neurological complications are unknown. Methods We included all consecutive patients admitted to the university hospital of Marseille for N₂O‐induced neurological complications in a prospective observational study. Patients underwent neurological examination, spinal cord magnetic resonance imaging, and nerve conduction...

article

Successful autologous hematopoietic stem cell transplantation in a refractory anti‐Caspr1 antibody nodopathy

Vadim Afanasiev, Pinelopi Tsouni, Thierry Kuntzer, Anne Cairoli, more

Journal of the Peripheral Nervous System > 29 > 1 > 116 - 119

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...

article

Quantification of muscle involvement in familial amyloid polyneuropathy using MRI

Clémence Durelle, Emilien Delmont, Constance Michel, Amira Trabelsi, more

European Journal of Neurology > 30 > 10 > 3286 - 3295

Background and purpose Transthyretin familial amyloid polyneuropathy (TTR‐FAP) is a rare genetic disease with autosomal‐dominant inheritance. In this study, we aimed to quantify fatty infiltration (fat fraction [FF]) and magnetization transfer ratio (MTR) in individual muscles of patients with symptomatic and asymptomatic TTR‐FAP using magnetic resonance imaging. Secondarily, we aimed to assess correlations...

article

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

Luce Barbat du Closel, Nathalie Bonello‐Palot, Yann Péréon, Andoni Echaniz‐Laguna, more

European Journal of Neurology > 30 > 10 > 3265 - 3276

Background X‐Linked Charcot–Marie–Tooth disease type 1 (CMTX1) is characterized by gender differences in clinical severity. Women are usually clinically affected later and less severely than men. However, their clinical presentation appears to be heterogenous. Our aim was to extend the phenotypic description in a large series of women with CMTX1. Methods We retrospectively evaluated 263 patients...

article

Anti‐disialosyl‐immunoglobulin M chronic autoimmune neuropathies: a nationwide multicenter retrospective study

Claire Peillet, David Adams, Shahram Attarian, Françoise Bouhour, more

European Journal of Neurology > 29 > 12 > 3547 - 3555

Background and purpose In this retrospective study involving 14 university hospitals from France and Switzerland, the aim was to define the clinicopathological features of chronic neuropathies with anti‐disialosyl ganglioside immunoglobulin M (IgM) antibodies (CNDA). Results Fifty‐five patients with a polyneuropathy evolving for more than 2 months and with at least one anti‐disialosyl ganglioside...

article

Phenotypical variability and atypical presentations in a French cohort of Andersen–Tawil syndrome

Rocio Nur Villar‐Quiles, Damien Sternberg, Grégoire Tredez, Norma Beatriz Romero, more

European Journal of Neurology > 29 > 8 > 2398 - 2411

Background and purpose Andersen–Tawil syndrome (ATS) is a skeletal muscle channelopathy caused by KCNJ2 mutations, characterized by a clinical triad of periodic paralysis, cardiac arrhythmias and dysmorphism. The muscle phenotype, particularly the atypical forms with prominent permanent weakness or predominantly painful symptoms, remains incompletely characterized. Methods A retrospective clinical,...

article

Shoulder palsy following SARS‐CoV‐2 infection: two cases of typical Parsonage–Turner syndrome

Etienne Fortanier, Thomas Le Corroller, Marie Hocquart, Emilien Delmont, more

European Journal of Neurology > 29 > 8 > 2548 - 2550

Background and purpose Coronavirus disease 2019 (COVID‐19) is now known to cause neurological complications in both the central and the peripheral nervous system. Two new cases of typical neuralgic amyotrophy or Parsonage–Turner (PT) syndrome following coronavirus 2 infection (SARS‐CoV‐2) are reported here with explicit electrophysiological and imaging pathological features, underlining the possible...

article

Motor unit number index: A potential electrophysiological biomarker for pediatric spinal muscular atrophy

Christophe Boulay, Emilien Delmont, Frédérique Audic, Brigitte Chabrol, more

Muscle & Nerve > 64 > 4 > 445 - 453

Introduction/Aims In adult spinal muscular atrophy (SMA), the motor unit number index (MUNIX) has been shown to be an useful electrophysiological biomarker. This study evaluated the feasibility and the clinical relevance of using the MUNIX technique for patients with pediatric SMA (Ped‐SMA) and correlated MUNIX results with clinical scores. Methods Fourteen patients with type II Ped‐SMA (11 females;...

article

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

Fabien Hauw, Guillaume Fargeot, David Adams, Shahram Attarian, more

European Journal of Neurology > 28 > 9 > 2846 - 2854

Background and purpose Charcot–Marie–Tooth (CMT) disease, an untreatable hereditary polyneuropathy, may mimic chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), a treatable neuropathy. Methods 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...

article

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

Marie Subréville, Nathalie Bonello‐Palot, Douniazed Yahiaoui, Sadia Beloribi‐Djefaflia, more

European Journal of Neurology > 28 > 9 > 2913 - 2921

Background and purpose Preparations for clinical trials of unfolded protein response (UPR) inhibitors (such as Sephin1) that target the upregulated UPR in patients with Charcot–Marie–Tooth disease (CMT) carrying MPZ mutations are currently underway. The inclusion criteria for these trials are still being formulated. Our objective was to characterize the relation between genotypes and phenotypes in...

article

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

Butrint Aliu, Delphine Demeestere, Emilie Seydoux, José Boucraut, more

Journal of Neurochemistry > 154 > 5 > 486 - 501

Anti‐myelin‐associated glycoprotein (MAG) neuropathy is a disabling autoimmune peripheral neuropathy that is caused by circulating monoclonal IgM autoantibodies directed against the human natural killer‐1 (HNK‐1) epitope. This carbohydrate epitope is highly expressed on adhesion molecules such as MAG, a glycoprotein present in myelinated nerves. We previously showed the therapeutic potential of the...

article

Quantitative muscle MRI study of patients with sporadic inclusion body myositis

Behnaz Ansari, Emmanuelle Salort‐Campana, Augustin Ogier, Arnaud Le Troter PhD, more

Muscle & Nerve > 61 > 4 > 496 - 503

Background Fat infiltration in individual muscles of sporadic inclusion body myositis (sIBM) patients has rarely been assessed. Methods Sixteen sIBM patients were assessed using MRI of the thighs and lower legs (LL). The severity of fat infiltration, proximal‐to‐distal and side asymmetries, and the correlations with clinical and functional parameters were investigated. Results All the patients...

article

Comparison of MRI and motor evoked potential with triple stimulation technique for the detection of brachial plexus abnormalities in multifocal motor neuropathy

Giovanni Corazza, Thomas Le Corroller, Aude‐Marie Grapperon, Emmanuelle Salort‐Campana, more

Muscle & Nerve > 61 > 3 > 325 - 329

Background Conduction blocks (CB) are the diagnostic hallmark of multifocal motor neuropathy (MMN). Conventional nerve conduction studies cannot detect CB above Erb's point. Our purpose was to compare the performance of the motor evoked potential with triple stimulation technique (MEP‐TST) and MRI in the detection of abnormalities of the brachial plexus. Methods Examinations were performed on 26...

article

Relevance of anti-HNK1 antibodies in the management of anti-MAG neuropathies

Emilien Delmont, Shahram Attarian, Jean-Christophe Antoine, Stephane Paul, more

Journal of Neurology > 2019 > 266 > 8 > 1973-1979

Introduction In peripheral neuropathies with antibodies against Myelin Associated Glycoprotein (MAG), an IgM monoclonal gammopathy recognizes a specific epitope called Human Natural Killer 1 (HNK1) shared by NK lymphocytes and several components of the peripheral nerve myelin. Recently an ELISA test has been developed to detect antibodies against HNK1 epitope. Objectives were to determine the usefulness...

article

Efficacy and Safety of Octagam® in Patients With Chronic Inflammatory Demyelinating Polyneuropathy

Chafké Belmokhtar, Pierre Lozeron, David Adams, Jérôme Franques, more

Neurology and Therapy > 2019 > 8 > 1 > 69-78

Introduction Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a debilitating autoimmune neuropathy that is treated with intravenous immunoglobulin (IVIG). The aim of this retrospective study was to investigate the efficacy and safety of the sucrose-free IVIG Octagam® (Octapharma AG, Lachen, Switzerland) in patients with CIDP. Methods Data from 47 patients who received at least...

article

Upper limb onset of hereditary transthyretin amyloidosis is common in non‐endemic areas

M. Théaudin, P. Lozeron, V. Algalarrondo, C. Lacroix, more

European Journal of Neurology > 26 > 3 > 497 - e36

Background and purpose The aim is to describe an uncommon phenotype of hereditary ATTR neuropathy with upper limb onset. Methods The French TTR Familial Amyloid Polyneuropathy database was used for a retrospective evaluation of 32 consecutive patients with upper limb onset of the neuropathy (study group) and they were compared to 31 Portuguese early‐onset patients and 99 late‐onset patients without...

article

Guillain‐BarrÉ syndrome subtype diagnosis: A prospective multicentric European study

Peter Y. K. Van den Bergh, Françoise Piéret, John L. Woodard, Shahram Attarian, more

Muscle & Nerve > 58 > 1 > 23 - 28

Introduction: There is uncertainty as to whether the Guillain‐Barré syndrome (GBS) subtypes, acute inflammatory demyelinating polyradiculoneuropathy (AIDP) and acute motor axonal neuropathy (AMAN), can be diagnosed electrophysiologically. Methods: We prospectively included 58 GBS patients. Electrodiagnostic testing (EDX) was performed at means of 5 and 33 days after disease onset. Two traditional...

article

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

Florence Robert‐Varvat, Guillemette Jousserand, Françoise Bouhour, Christophe Vial, more

Muscle & Nerve > 57 > 2 > 217 - 221

Introduction: In young patients with mononeuropathy who lack family history and precipitating factors, hereditary neuropathy with liability to pressure palsy (HNPP) may be a possibility. Our objective is to propose neurophysiological criteria for HNPP in patients <30 years of age. Methods: We conducted a national multicenter retrospective clinical and neurophysiological study in patients under...

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INFONA - science communication portal

Search results for: Emilien Delmont

Electrophysiological features of the peripheral neuropathy in patients with pathologic biallelic RFC1 repeat expansions

Longitudinal follow‐up and prognostic factors in nitrous oxide‐induced neuropathy

Factors affecting the topography of nitrous oxide‐induced neurological complications

Successful autologous hematopoietic stem cell transplantation in a refractory anti‐Caspr1 antibody nodopathy

Quantification of muscle involvement in familial amyloid polyneuropathy using MRI

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

Anti‐disialosyl‐immunoglobulin M chronic autoimmune neuropathies: a nationwide multicenter retrospective study

Phenotypical variability and atypical presentations in a French cohort of Andersen–Tawil syndrome

Shoulder palsy following SARS‐CoV‐2 infection: two cases of typical Parsonage–Turner syndrome

Motor unit number index: A potential electrophysiological biomarker for pediatric spinal muscular atrophy

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

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

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

Quantitative muscle MRI study of patients with sporadic inclusion body myositis

Comparison of MRI and motor evoked potential with triple stimulation technique for the detection of brachial plexus abnormalities in multifocal motor neuropathy

Relevance of anti-HNK1 antibodies in the management of anti-MAG neuropathies

Efficacy and Safety of Octagam® in Patients With Chronic Inflammatory Demyelinating Polyneuropathy

Upper limb onset of hereditary transthyretin amyloidosis is common in non‐endemic areas

Guillain‐BarrÉ syndrome subtype diagnosis: A prospective multicentric European study

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

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