Revue Neurologique[JOURNAL]
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200 papers
Macrophage inclusions in patients undergoing antisense oligonucleotide therapy for ALS or SMA: A retrospective and transversal study.
Demeret R, Vieles Marais D, Treiner E … +6 more , Acket B, Fabry V, Levade T, Nogueira L, Pages JC, Cintas P
Rev Neurol (Paris) · 2026 Jul · PMID 42399152 · Publisher ↗
BACKGROUND: Intrathecal antisense oligonucleotides (ASOs) have revolutionized the management of genetic motor neuron diseases. Nusinersen is approved for spinal muscular atrophy (SMA) caused by SMN1 mutations, and tofers...
BACKGROUND: Intrathecal antisense oligonucleotides (ASOs) have revolutionized the management of genetic motor neuron diseases. Nusinersen is approved for spinal muscular atrophy (SMA) caused by SMN1 mutations, and tofersen for amyotrophic lateral sclerosis (ALS) linked to SOD1 mutations. Since their approval, some studies reported the presence of macrophagic inclusions in cerebrospinal fluid (CSF) of patients treated with ASOs, first in nusinersen-treated patients and more recently in those receiving tofersen. These findings remain poorly characterized, and their clinical significance is unclear. METHODS: We first conducted a retrospective study in 21 patients (132 CSF samples): six treated with tofersen (every 4 weeks) and 15 with nusinersen (every 4 months). CSF samples were analyzed for macrophagic inclusions, their time of onset, and persistence over time. To assess clinical and inflammatory correlates of macrophagic inclusions, we then performed an analysis of CSF inflammatory biomarkers and serum ferritin and neurofilament light chain tests in 18 of these patients still under treatment. RESULTS: In tofersen-treated patients, macrophagic inclusions were consistently observed and persisted over time, except in one case. In nusinersen-treated patients, inclusions were rare and transient. An inflammatory CSF profile was associated with the presence of inclusions, but their cellular nature remained undetermined. Notably, tofersen-treated patients with "tofersenophages" exhibited favorable clinical responses. DISCUSSION: Macrophagic inclusions appear more frequent in the CSF of tofersen-treated patients than previously reported. While their origin remains unclear, they seem linked to CSF inflammation without precluding a beneficial therapeutic response.
Geographic disparities in MRI features of ischemic stroke and small vessel disease: A comparative study between French Guiana and mainland France. Findings from the BECATOUR multicenter registry.
Maisonnier C, Cottier JP, Charbonnier G … +8 more , Monaya A, Rhein J, Gaudron M, Haba M, Deschamps N, Nasri A, de Toffol B, Maldonado IL
Rev Neurol (Paris) · 2026 Jun · PMID 42373365 · Publisher ↗
BACKGROUND: Stroke disproportionately affects French Guiana, which has the highest incidence and premature stroke mortality among French territories. Beyond traditional vascular risks, cerebral small vessel disease (CSVD...
BACKGROUND: Stroke disproportionately affects French Guiana, which has the highest incidence and premature stroke mortality among French territories. Beyond traditional vascular risks, cerebral small vessel disease (CSVD) may explain these disparities. This study compared MRI characteristics of acute ischemic stroke and CSVD burden in Cayenne with two mainland French centers (Besançon and Tours). METHODS: In line with the multicenter observational BECATOUR study, we analyzed ischemic stroke subtype, vascular occlusion, T2-FLAIR intravascular hyperintensities, and hemorrhagic markers. CSVD features (white matter hyperintensities, lacunes, microbleeds, enlarged perivascular spaces) were scored using validated scales. Global cardiovascular risk was estimated using a Framingham-based score. FINDINGS: Intravascular T2-FLAIR hyperintensities, reflecting collateral arterial circulation, were less frequent in Cayenne than in mainland France. Patients in Cayenne had a markedly higher CSVD burden (CSVD score >2 according to Lau et al.: 42.4%, 19.8% and 17.2% respectively in Cayenne, Besançon, and Tours, P=0.01), driven by more severe white matter hyperintensities and increased microbleeds. Among 257 patients, a cardiovascular risk >20% was associated with severe white matter hyperintensities and higher overall CSVD burden. In Cayenne, 20% of patients would have met criteria for mechanical thrombectomy, a procedure that is not locally available. INTERPRETATION: Stroke patients in French Guiana carry a substantially higher burden of CSVD, closely linked to modifiable cardiovascular risk. Combined with limited access to advanced acute stroke interventions, these findings highlight major structural health inequities. Strengthening prevention, early risk-factor management, and access to reperfusion therapies is essential to reduce the disproportionate stroke burden in French Guiana.
Continuous subcutaneous perfusion of apomorphine in Parkinson's disease: Towards monotherapy?
Marques A, Lambert C, Derost P … +4 more , Beal C, Debilly B, Rieu I, Durif F
Rev Neurol (Paris) · 2026 Jun · PMID 42309957 · Publisher ↗
BACKGROUND: Continuous subcutaneous apomorphine infusion (CSAI) is an effective therapy for Parkinson's disease (PD) patients with motor complications. Whether CSAI should be used in association or in monotherapy to prev...
BACKGROUND: Continuous subcutaneous apomorphine infusion (CSAI) is an effective therapy for Parkinson's disease (PD) patients with motor complications. Whether CSAI should be used in association or in monotherapy to prevent any pulsatile stimulation due to oral treatments remains unclear. OBJECTIVE: This study aimed to evaluate the feasibility, efficacy, and tolerance of CSAI monotherapy compared to its use in combination therapy. METHOD: We retrospectively collected the clinical characteristics of PD patients treated with CSAI between 2007 and 2023, before the introduction of CSAI (M0) and after six months of CSAI (M6). We identified the proportion of patients who had CSAI on monotherapy at M6, and collected data on motor symptoms, side effects, and treatment doses in patients treated by monotherapy versus in association. RESULTS: Of 117 PD patients treated with CSAI between 2007 and 2023, 21 (17.9%; 95%CI: 11.5 to 26.1) were on monotherapy at M6. Monotherapy patients were more likely to be female and had lower baseline levodopa-equivalent doses. Dyskinesia duration at M6 was lower in this group (P=0.04) without significant differences in "off" duration, MDS-UPDRS III scores, or side effects compared to those receiving combination therapy. Patients with monotherapy were more frequently treated with continuous 24-hour CSAI and had higher daily apomorphine doses. CONCLUSION: CSAI monotherapy is a feasible option for PD patients requiring continuous dopaminergic stimulation, offering improved dyskinesia control with similar efficacy and tolerability to combination therapy. Further studies are needed to determine patient selection for CSAI monotherapy, the long-term benefits obtained and broaden its clinical application.
Neuro-Whipple presenting as autoimmune encephalitis.
Assaraj R, Al Chare I, Marois C … +3 more , Bousquet-Mélou C, Pourcher V, Degos B
Rev Neurol (Paris) · 2026 Jun · PMID 42297708 · Publisher ↗
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Multimodal assessment of minimally conscious state and cognitive motor dissociation in neurocritical care: A critical review.
Vitturi BK
Rev Neurol (Paris) · 2026 Jun · PMID 42276905 · Publisher ↗
Minimally conscious state (MCS) is characterized by inconsistent but clearly discernible clinical and behavioral evidence of consciousness. Cognitive motor dissociation (CMD) or covert consciousness refers to a condition...
Minimally conscious state (MCS) is characterized by inconsistent but clearly discernible clinical and behavioral evidence of consciousness. Cognitive motor dissociation (CMD) or covert consciousness refers to a condition characterized by a marked dissociation in which a preserved but unacknowledged (covert) cognitive capacity exists in some patients with severe brain injury who show absent or non-purposeful behavioral responses. Traditionally underrecognized by standard neurological examination, this condition may carry significant implications for prognostication and critical care decision-making. Early identification of preserved cognitive processing challenges reductionist views of coma and may help prevent premature withdrawal of life-sustaining therapies. This review synthesizes the principal clinical, electrophysiological, and neuroimaging tools capable of detecting CMD, including advanced bedside examination strategies, behavioral rating scales, structural and functional MRI, EEG reactivity and complexity metrics, machine-learning-based analyses, and multimodal approaches. Although novel technologies - such as task-based fMRI, TMS-EEG, and FDG-PET - offer promising means to infer preserved network connectivity, expert clinical examination and validated scales such as the Coma Recovery Scale-Revised (CRS-R) remain critically important and continue to be among the most widely supported diagnostic methods. Integrating repeated behavioral assessments with quantitative neurophysiological data yields a more comprehensive understanding of residual cognitive function, yet challenges persist, including high false-negative rates, susceptibility to sedation and arousal fluctuations, and limited standardization across modalities. Ethical considerations surrounding the diagnosis of CMD are equally significant, especially in relation to goals-of-care decisions, neuro-palliative strategies, and family communication. Ultimately, while the detection of CMD is essential to contemporary neurocritical practice, its practical implications for therapeutic decision-making remain incompletely defined, mandating individualized, cautious interpretation within a patient-centered clinical framework.
Development of a new episodic memory assessment tool (NEM): Preliminary data and clinical perspectives.
Launay A, Baudouin A, Gonthier C … +4 more , Brachet M, Matysiak A, Vanneste S, Taconnat L
Rev Neurol (Paris) · 2026 Jun · PMID 42276904 · Publisher ↗
The present study aimed to test the discriminating power, reliability, and validity of a new episodic memory assessment tool (NEM). NEM was designed to be more ecological than current tests of episodic memory, based on i...
The present study aimed to test the discriminating power, reliability, and validity of a new episodic memory assessment tool (NEM). NEM was designed to be more ecological than current tests of episodic memory, based on incidental encoding and a deepened assessment of recall. The encoded material is a short video involving a choice procedure to encourage participant immersion. The delayed recall phase is guided by a semi-directive interview. Preliminary data were collected from 199 cognitively unimpaired participants aged 18 to 91, as well as from 35 older adults with cognitive impairment (18 patients with mild cognitive impairment and 17 patients with Alzheimer's disease). The results show that NEM exhibits good internal consistency and inter-rater agreement. NEM performance is sensitive to the effects of age and can discriminate between pathological and normal ageing. NEM offers an alternative to existing tools for a more ecological assessment of episodic memory, enabling better investigation of the formation of episodic memories in everyday life.
Novel variants and rare clinical presentations in MFN2-related Charcot-Marie-Tooth disease: Insights from 10 families.
Gharebaghian H, Ravanbod M, Ghasemi A … +3 more , Okhovat AA, Nafissi S, Alavi A
Rev Neurol (Paris) · 2026 Jun · PMID 42236346 · Publisher ↗
BACKGROUND: Charcot-Marie-Tooth disease (CMT) is a genetically heterogeneous group of inherited neuropathies, and MFN2-related CMT is a common CMT subtype. In this study, we described 13 affected individuals from 10 unre...
BACKGROUND: Charcot-Marie-Tooth disease (CMT) is a genetically heterogeneous group of inherited neuropathies, and MFN2-related CMT is a common CMT subtype. In this study, we described 13 affected individuals from 10 unrelated Iranian families harboring MFN2 variants. METHODS AND RESULTS: A total of 10 families (13 individuals) affected with MFN2-related CMT were recruited from a large CMT cohort, after whole exome sequencing and subsequent co-segregation analysis. We identified 10 missense variants, including five novel ones: p.Arg364Leu, p.Arg663His, p.Thr130Asn, p.Arg476Gly, and p.Glu744Asp. Patients showed a wide range of features, with age at onset varying from 1 to 69 years. Typical CMT features such as distal limb weakness, foot deformities, and sensory impairments were accompanied by rare clinical findings including erectile dysfunction (3/8 males), cognitive dysfunction, and central nervous system (CNS) abnormalities apparent on brain magnetic resonance imaging (MRI). Notably, two individuals carried biallelic MFN2 variants displaying AR MFN2-related CMT: one compound heterozygote individual who presented with diaphragmatic weakness and vocal cord involvement, and another homozygote individual who manifested a relatively mild phenotype. Intrafamilial variability and reduced penetrance were also noted, with some heterozygote individuals remaining asymptomatic throughout their lives. CONCLUSIONS: Our findings not only expand the mutational spectrum of MFN2-related CMT, but also highlight its broader phenotypic heterogeneity. This study also underscores that rare manifestations such as cognitive impairment and autonomic symptoms may be under-recognized features.
Current management and treatment of patients with myasthenia gravis in France: A survey of the neurologist's perspective.
Cintas P, Salort-Campana E, Demeret S … +6 more , Stanbury T, Bouquillon B, Nadaj-Pakleza A, Gallard J, Solé G, Attarian S
Rev Neurol (Paris) · 2026 Jun · PMID 42236345 · Publisher ↗
Myasthenia gravis (MG) is a rare autoimmune disease in which immunoglobulin G autoantibodies targeting post-synaptic receptors at neuromuscular junction play a central role. Due to recent therapeutic developments, there...
Myasthenia gravis (MG) is a rare autoimmune disease in which immunoglobulin G autoantibodies targeting post-synaptic receptors at neuromuscular junction play a central role. Due to recent therapeutic developments, there is a need to update current guidelines. FILNEMUS (the French network for rare neuromuscular diseases) conducted a survey in neurologists treating patients with MG to better understand the current clinical and therapeutic management of MG and to identify difficulties and unmet needs. The 45-questions survey assessed various aspects of MG management: including characteristics of neurologists and their patients, the treatments prescribed, and potential areas to improve MG management. Between the 21st of April and the 22nd of August 2022, 2,535 neurologists (90.4%) of the 2,792 registered in France (in 2022) were asked to participate. Finally, 321 complete the survey and were analysed. These population constitute a representative population of neurologists with all French regions and types of medical institutions represented. The survey found that 73.2% of patients with MG are treated by neurologists working in hospitals. Regarding treatment, most neurologists (96.9%) prescribed a cholinesterase inhibitor as first-line treatment most often combined with a corticosteroid (69.1%) or with a non-steroidal immunosuppressant (66.0%). Neurologists working in reference hospital centres tended to prescribe more corticosteroids as first-line treatment for ocular MG and as early disease-modifying treatment for generalized MG. Neurologists identified several aspects of MG management that needed to be improved, including the diagnosis of atypical types of MG, the transfer of patient information from neurologists to general practitioners, the need to optimise existing therapies (improve tolerance and effectiveness), continual medical education for neurologists, as well as the need for improved therapeutic patient education. Our survey shows that evidence from the neurologists' perspective can provide valuable insight into the management of patients with MG and can identify unmet clinical and therapeutics needs.
Ambulatory introduction of foslevodopa/foscarbidopa in Parkinson's disease in private practice. Case series of the first 22 patients.
Bonnet C, Mesnage V, Sacko F … +2 more , Ziz C, Jaulent P
Rev Neurol (Paris) · 2026 Jun · PMID 42225510 · Publisher ↗
Since foslevodopa/foscarbidopa (fLD/fCD) is available, all reported initiations have occurred in hospital or ambulatory care settings within specialized movement disorder clinics. We describe the feasibility and tolerabi...
Since foslevodopa/foscarbidopa (fLD/fCD) is available, all reported initiations have occurred in hospital or ambulatory care settings within specialized movement disorder clinics. We describe the feasibility and tolerability of an ambulatory initiation of fLD/fCD in two private practice settings. Using the patient profiles defined in the French Delphi consensus statement for apomorphine-based device-aided therapy (DAT), we were able to identify two distinct treatment strategies, quality of life outcomes and adverse events. Patients with no history of cognitive impairment or hallucinations, a disease duration of less than 10±4 years, and H&Y<3, tolerated fLD/fCD therapy very well, despite relatively high infusion flow rates, with a substantial improvement in quality of life. Profiles 4-5 with a history of cognitive impairment or hallucinations, longer disease duration, more severe disease, tolerated fLD/fCD less well, had less clinical improvement, required moderate infusion flow rates, supplemented oral medication, and often clozapine treatment before and after fLD/fCD initiation. Our study provides clinically relevant insights into the feasibility of fLD/fCD initiation by practicing neurologist.
Six-month outcomes of subcutaneous foslevodopa/foscarbidopa according to initiation setting in advanced Parkinson's disease: A retrospective cohort study.
Desjardins C, de Saint Vaulry H, Rosset C … +3 more , Salardaine Q, Brandel JP, Baille G
Rev Neurol (Paris) · 2026 May · PMID 42203566 · Publisher ↗
The optimal setting for initiating subcutaneous foslevodopa/foscarbidopa (CSFLI) in advanced Parkinson's disease remains debated. This study aimed to compare six-month clinical outcomes according to initiation setting. W...
The optimal setting for initiating subcutaneous foslevodopa/foscarbidopa (CSFLI) in advanced Parkinson's disease remains debated. This study aimed to compare six-month clinical outcomes according to initiation setting. We conducted a retrospective cohort study of consecutive patients with advanced Parkinson's disease started on CSFLI in either an outpatient or inpatient setting. Baseline demographics and clinical measures were recorded. Outcomes at six months included motor complications (MDS-UPDRS Part IV), motor severity (MDS-UPDRS Part III), non-motor impact and quality of life, treatment parameters and levodopa equivalent daily dose, treatment simplification to CSFLI monotherapy, and safety. Group comparisons used standard statistical tests with two-sided significance. Thirty-six patients were included: 21 outpatients (58.3%) and 15 inpatients (41.7%). Six-month motor and quality-of-life outcomes were broadly similar between groups. The inpatients group showed higher MDS-UPDRS Part IV scores at six months (10.3 vs. 7.7, P=0.039) despite comparable baseline Part IV values. Infusion parameters and CSFLI levodopa-equivalent dosing at six months were comparable between groups. CSFLI monotherapy was achieved in more than 80% of patients in both groups with minimal use of concomitant oral levodopa. Adverse events were similar, and no serious events occurred. Early CSFLI outcomes appear largely independent of initiation setting when protocols are structured and follow-up is rigorous. Higher six-month Part IV scores in inpatients likely reflect post-initiation factors rather than baseline severity. Outpatient initiation emerges as a viable, resource-sparing option. Prospective studies with standardized assessments are needed to test causality and refine patient selection.
Establishing age-stratified reference values for blood Neurofilament Light chain (NfL) using the Lumipulse® platform.
Hanin A, Bahroun S, Chiodega V … +6 more , Bonnefont-Rousselot D, Del Mar Amador M, Salachas F, Mariotto S, Dingeo G, Lamari F
Rev Neurol (Paris) · 2026 Jun · PMID 42168010 · Publisher ↗
OBJECTIVES: Neurofilament light chain (NfL) is a sensitive biomarker of neuroaxonal injury detectable in blood and cerebrospinal fluid. Its concentrations are strongly influenced by age, yet no age-specific reference val...
OBJECTIVES: Neurofilament light chain (NfL) is a sensitive biomarker of neuroaxonal injury detectable in blood and cerebrospinal fluid. Its concentrations are strongly influenced by age, yet no age-specific reference values have been established for the fully automated Lumipulse® platform, and no direct comparison between serum and plasma has been reported for this assay. METHODS: Matched serum and plasma samples from 49 participants were analyzed to assess matrix comparability using correlation, paired comparison, and equivalence testing within predefined ±15% bounds. Reference values were determined in 211 control adults aged 18-87years from two international centers. Age groups were derived using K-means clustering, and multivariable linear regression was applied to examine the effects of recruitment center, age, and sex. RESULTS: Serum and plasma NfL levels were highly correlated (Spearman's ρ=0.969, P<0.001). Although serum values were slightly higher than plasma (+3.07pg/mL, P=0.042), the difference was within equivalence margins (P=0.019), indicating that either matrix can be used without compromising clinical interpretation. NfL concentrations increased markedly with age (Spearman's ρ=0.721, c<0.001), with median values rising from 8.45pg/mL in individuals aged 18-34years to 23.4pg/mL in those aged ≥69years. Reference values were defined as the 97.5th percentile and were 18.0pg/mL for patients aged 18-50, 25.4pg/mL for those aged 51-68, and 43.5pg/mL for patients aged 69years or older. No significant effect of sex was observed after adjusting for age. CONCLUSIONS: This is the first study to establish serum-plasma comparability for NfL on the Lumipulse® platform and to provide age-stratified reference values in control adults, emphasizing the need for age-adjusted interpretation when distinguishing pathological elevations from normal age-related changes.
Primary Lateral Sclerosis French National Diagnostic and Care Protocol.
Corcia P, Bernard E, de la Cruz E … +13 more , Danel V, Soriani MH, Guy N, Esselin F, Bruneteau G, Pradat PF, Goutines V, Catherine J, Eyraud N, Cheve P, Fernandez A, Erazo D, Couratier P
Rev Neurol (Paris) · 2026 Jun · PMID 42168009 · Publisher ↗
Primary lateral sclerosis (PLS) is a rare neurodegenerative motor neuron disease characterized by progressive and selective involvement of the central motor neuron within the bulbar and spinal regions. It is estimated to...
Primary lateral sclerosis (PLS) is a rare neurodegenerative motor neuron disease characterized by progressive and selective involvement of the central motor neuron within the bulbar and spinal regions. It is estimated to account for 1-5% of motor neuron diseases and typically presents in the fifth or sixth decade of life, with a slight male predominance. According to current consensus criteria, the diagnosis relies on the demonstration of progressive upper motor neuron dysfunction in the absence of lower motor neuron involvement, with persistence of isolated upper motor neuron signs for at least four years in order to exclude a slowly progressive upper motor neuron-predominant form of amyotrophic lateral sclerosis (ALS). The French Motor Neuron Disease Network (FILSLAN) developed a National Diagnostic and Care Protocol (PNDS) with the aim of standardizing diagnostic criteria, optimizing differential diagnosis, and providing evidence-based recommendations for therapeutic management and follow-up across the national territory. These recommendations were elaborated in accordance with the methodological framework of the French National Authority for Health for rare diseases. The protocol provides practical guidance for establishing PLS as a diagnosis of exclusion, distinguishing it from ALS and hereditary spastic paraplegias, and organizing appropriate clinical and paraclinical investigations. It also outlines indications for genetic testing in selected cases and defines a multidisciplinary management strategy centered on symptomatic treatment, early rehabilitation, respiratory and nutritional surveillance, and psychosocial support. Given the slower progression of PLS compared with ALS, biannual multidisciplinary follow-up is generally appropriate. This protocol aims to harmonize clinical practice and improve patient care while acknowledging the current absence of disease-modifying therapies.
Real-world utilization of acute treatments for cluster headache: Insights from the French National Open Health Data.
Arcani V, Bruyat A, Hache G … +2 more , Donnet A, Redon S
Rev Neurol (Paris) · 2026 Jun · PMID 42168008 · Publisher ↗
BACKGROUND: The utilization of open health databases offers a unique opportunity to analyze the dispensing of healthcare products within real-world populations. A recent study by Van Obberghen et al. (2025) was the first...
BACKGROUND: The utilization of open health databases offers a unique opportunity to analyze the dispensing of healthcare products within real-world populations. A recent study by Van Obberghen et al. (2025) was the first to leverage the French national database to examine consumption volumes for cluster headache (CH) treatments, with a specific focus on the sex ratio. Nonetheless, further investigation into additional variables - such as seasonality and geographical distribution - is warranted, given the well-established role of chronobiology in the pathophysiology of CH. OBJECTIVES: The primary objective of this study was to characterize the clinical expression of CH using real-world healthcare data, specifically assessing the influence of gender, age, and temporal-spatial factors. Additionally, we aimed to quantify the evolving economic burden of CH treatments at the national level. METHODS: A retrospective analysis was conducted using the French national health database (2014-2024). We extracted dispensing records for subcutaneous sumatriptan (SS) and oxygen therapy (OT), the two validated acute treatments for CH. These patterns were analyzed across demographic groups and administrative regions, with statistical correlations performed against climatic (sunshine, rainfall), demographic, and economic variables. RESULTS: Between 2014 and 2024, the total expenditure for OT in CH tripled, while SS costs increased only 1.6-fold. A consistent decrease in the male-to-female sex ratio was observed across all age groups for both treatments. This decline was statistically significant in all three age groups over 20 years of age for OT delivery, and in the two age groups between 20 and 59 years for SS delivery. A distinct peak in SS dispensing was observed in July; however, no statistically significant associations were found between SS dispensing and sunshine or rainfall. Younger men exhibited significantly higher consumption than older men, and overall consumption remained higher in men than in women. CONCLUSIONS: Our findings highlight evolving treatment patterns and a shifting sex ratio in the delivery of CH therapies among treated patients. While no clear environmental drivers of treatment use were identified, the study underscores the value of open health data in monitoring prescribing trends and evaluating the economic impact of CH therapies.
Level of consciousness, wakefulness and responsiveness in epileptic seizures.
Beniczky S, Ryvlin P, Kahane P … +1 more , Trinka E
Rev Neurol (Paris) · 2026 Jun · PMID 42140834 · Publisher ↗
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New tools to shape the future of MS and related disorders.
Louapre C
Rev Neurol (Paris) · 2026 May · PMID 42120114 · Publisher ↗
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7T magnetic resonance imaging of the human spinal cord in multiple sclerosis: Advances, insights and unmet needs.
Callot V, Destruel A, Demortière S … +1 more , Testud B
Rev Neurol (Paris) · 2026 May · PMID 42031644 · Publisher ↗
Magnetic resonance imaging (MRI) is central to the diagnosis and monitoring of multiple sclerosis (MS). While conventional MRI (1.5 or 3T) is sufficient in clinical practice for detecting and monitoring lesions, it fails...
Magnetic resonance imaging (MRI) is central to the diagnosis and monitoring of multiple sclerosis (MS). While conventional MRI (1.5 or 3T) is sufficient in clinical practice for detecting and monitoring lesions, it fails to provide a comprehensive and sensitive assessment of the pathology across the entire central nervous system. This limitation has fueled growing interest in the use of 7T MRI systems to gain deeper insight into MS pathophysiological mechanisms. 7T MRI of the brain has already been shown to be transformative , with improved detection of cortical lesions, central vein sign and paramagnetic rim. Although not yet as advanced as for the brain in MS, spinal cord 7T MRI is also increasingly demonstrating its potential. Key advances to date include assessement of increased lesion burden and characterization of refined tissue in both white and gray matter substructures with anatomical MRI, refined atrophy measurements with higher spatial resolutions, enhanced susceptibility-based contrasts, as well as subregional quantitative and functional MRI. Such methods are opening opportunities to facilitate neuroradiological assessment, to better describe gray matter pathological involvement, and to identify potential myelin-related or vascular biomarkers, that can be used for disability risk stratification, monitoring and therapeutic decision-making. Yet, beyond the mere availability of 7T MRI systems in clinical practice and across sites worldwide, 7T SC MRI remains limited by technical challenges, radio frequency coil availability, regulatory constraints, and the need for standardized protocols. This review summarizes the techniques, together with the main findings, that have been applied so far for the study of MS. Most of them being exploratory, we discuss their potential use in clinical practice, perspectives, challenges and remaining unmet needs.
The role of Epstein-Barr virus in multiple sclerosis: From pathogenesis to therapeutic potential.
Bellucci G, Mechelli R, Bigi R … +2 more , Ristori G, Salvetti M
Rev Neurol (Paris) · 2026 May · PMID 42025559 · Publisher ↗
A growing body of evidence positions Epstein-Barr virus (EBV) as a central agent in the etiopathogenesis of multiple sclerosis (MS). Compelling epidemiological studies now demonstrate that EBV infection precedes MS onset...
A growing body of evidence positions Epstein-Barr virus (EBV) as a central agent in the etiopathogenesis of multiple sclerosis (MS). Compelling epidemiological studies now demonstrate that EBV infection precedes MS onset and is a necessary precondition for disease development. This is supported by pathology findings revealing EBV-infected B cells within CNS lesions and immunogenetic data linking viral and human genetic susceptibility to MS risk. Mechanistically, EBV appears to act as an upstream trigger that reshapes B cell function, promotes molecular mimicry with CNS antigens, and drives compartmentalized neuroinflammation. In this Review, we synthesize epidemiological, pathological, immunogenetic, and clinical-therapeutic evidence to construct a coherent model of EBV-driven MS pathogenesis. We examine how current MS therapies intersect with EBV biology and discuss the challenges and opportunities in developing EBV-targeted strategies, including vaccines and antivirals, for disease prevention and early intervention. Finally, we highlight key unresolved questions and outline a translational research agenda aimed at intercepting MS through virologically informed approaches.
New immunotherapies for multiple sclerosis (MS): A comprehensive review.
Ayrignac X
Rev Neurol (Paris) · 2026 May · PMID 42014318 · Publisher ↗
Multiple sclerosis (MS) is the leading cause of non-traumatic disability in young adults, characterized by autoimmune demyelination and neurodegeneration. While high-efficacy therapies have transformed relapsing MS manag...
Multiple sclerosis (MS) is the leading cause of non-traumatic disability in young adults, characterized by autoimmune demyelination and neurodegeneration. While high-efficacy therapies have transformed relapsing MS management, disability progression remains an unmet need. Emerging evidence implicates compartmentalized central nervous system (CNS) inflammation, including microglial activation, in disease pathogenesis, necessitating novel immunotherapeutic strategies that target both peripheral and CNS-resident immune cells. This review synthesizes recent preclinical and clinical data on emerging MS immunotherapies offering promise for halting disability progression in MS. Their clinical integration will depend on balancing efficacy with safety, particularly in progressive phenotypes where therapeutic options remain limited.
Treatment pathway, healthcare resource utilization and direct cost of ALS in France: A nationwide claims database study.
Desnuelle C, Couratier P, Corcia P … +5 more , Duburcq A, Torreton E, Baffert S, Nevoret C, Turgeman S
Rev Neurol (Paris) · 2026 Jun · PMID 42000269 · Publisher ↗
OBJECTIVE: Amyotrophic lateral sclerosis (ALS) is a severe, progressive disease, associated with high clinical burden. The aim of this study was to estimate ALS-related healthcare resource utilization (HCRU), associated...
OBJECTIVE: Amyotrophic lateral sclerosis (ALS) is a severe, progressive disease, associated with high clinical burden. The aim of this study was to estimate ALS-related healthcare resource utilization (HCRU), associated direct costs and their determinants in France. METHODS: A retrospective cohort study was conducted among newly diagnosed patients with ALS identified between 2012 and 2022 (11 years) in the French National Health Data System (SNDS) through a validated algorithm. This incident population was compared with non-ALS controls (1:2) matched on age, sex, and region. Direct all-cause healthcare reimbursable costs were estimated. Survival, HCRU and direct costs were analyzed over the first five years after diagnostic. RESULTS: A total of 16,814 newly diagnosed ALS patients were identified who could be matched with 33,628 non-ALS controls. The median age was 68.0 year and 55.3% were males. Over the first year after diagnosis, the direct all-cause medical cost per patient was €19,497 of which 47.2% was related to inpatient care, compared to €4,921 for controls which led to an ALS-attributable cost of €14,474 per patient per year. ALS patients had significantly (P<0.0001) higher HCRU than controls in all items of inpatient and outpatient care but especially for utilization of medical devices, frequencies of nurse and physiotherapist visits and acute care hospitalizations. The annual direct cost per patient who survived the successive annual period after diagnosis increased during the second, third and fourth year to €22,358, €22,276 and €21,372 respectively and then declined in year 5 to €19,720. These results largely underestimated the real cost of the management of ALS by not considering the out-of-pocket expenses associated with informal care and home renovation as well as productivity loss. CONCLUSIONS: Patients with ALS had higher HCRU and direct medical cost, compared with controls. The economic burden of ALS was substantial even when restricted to the medical costs covered by the public health insurance system. There is an important need for novel therapies that might lower disease progression in early disease stages.
Cell therapy in multiple sclerosis: An overview.
Michel L
Rev Neurol (Paris) · 2026 May · PMID 41997827 · Publisher ↗
Despite major advances in therapy for multiple sclerosis (MS) patients, substantial unmet needs remain, particularly regarding the prevention of disability progression and the treatment of progressive and aggressive form...
Despite major advances in therapy for multiple sclerosis (MS) patients, substantial unmet needs remain, particularly regarding the prevention of disability progression and the treatment of progressive and aggressive forms of the disease. While early use of high-efficacy therapies has improved inflammatory disease control, their impact on long-term neurodegeneration is limited, and therapeutic options for progressive MS remain scarce. Autologous hematopoietic stem cell transplantation (AHSCT) has emerged as a highly effective escalation strategy for selected patients with aggressive, inflammatory MS. Randomized trials and large observational cohorts demonstrate sustained suppression of inflammatory activity and high rates of no evidence of disease activity, with markedly improved safety profiles over time. Current consensus recommendations support AHSCT for highly active MS refractory to high-efficacy DMTs, while its use in non-inflammatory progressive MS remains debatable. Chimeric antigen receptor (CAR) T-cell therapies represent a novel approach targeting compartmentalized B-cell - driven pathology. Early clinical reports using CD19- and BCMA-directed CAR-T cells in progressive MS demonstrate CNS penetration, manageable toxicity, and preliminary signals of clinical improvement, warranting further investigation in controlled trials. Mesenchymal stromal cells (MSCs) offer immunomodulatory and neurotrophic properties with a favorable safety profile. Clinical studies suggest potential benefits, particularly with intrathecal administration, though efficacy remains inconsistent due to heterogeneous study designs, patient populations, and outcome measures. Overall, cell-based therapies hold promise for addressing unmet needs in MS, but robust randomized trials, optimized delivery strategies, and appropriate patient selection are essential to define their therapeutic role.
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