Revue Neurologique[JOURNAL]
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200 papers
Long-interval dosing of anti-CD20 therapy: Toward affordable treatment for multiple sclerosis.
Boutiere C, Saunier R, Rico A … +7 more , Perriguey M, Demortiere S, Hilezian F, Maarouf A, Roudot M, Pelletier J, Audoin B
Rev Neurol (Paris) · 2026 Mar · PMID 41547622 · Publisher ↗
Abstract loading — click title to view on PubMed.
Visual misperceptions in favour of living beings in Parkinson's disease: A psychophysical study.
Cleret de Langavant L, Mouton A, de Gardelle V … +4 more , Kouider S, Bachoud-Lévi AC, Fénelon G, Remy P
Rev Neurol (Paris) · 2026 Mar · PMID 41545271 · Publisher ↗
BACKGROUND: Parkinson's disease (PD) patients often report seeing persons or animals rather than objects but this phenomenon remains poorly understood. Here, we use three experimental tasks to confirm such observation an...
BACKGROUND: Parkinson's disease (PD) patients often report seeing persons or animals rather than objects but this phenomenon remains poorly understood. Here, we use three experimental tasks to confirm such observation and to explore its cognitive mechanisms. METHOD: Fourteen PD patients with visual hallucinations (PD-VH), 14 PD patients without visual hallucinations (PD-NVH) and 14 controls with similar cognitive performance were tested using ambiguous stimuli. Ambiguous stimuli were morphs in which visual features from faces and flowers were melted together (Experiments 1 and 2) and a black and white picture where a Dalmatian dog was hidden (Experiment 3). In Experiment 1, participants categorised ambiguous stimuli either as face or flower. In Experiment 2, they were shown an ambiguous stimulus, then a mask, and finally two ambiguous stimuli, one of which was identical to the first stimulus. In this discrimination task, participants chose which of the two last stimuli had been presented before. In Experiment 3, participants guessed the items hidden in the picture. We assessed group differences for categorisation with logistic modelling and computed sensitivity index and criterion psychophysical measures in Experiments 1 and 2. The ratio of living beings identified in the Dalmatian dog task was compared across groups. RESULTS: In the categorisation task, the PD-VH group tended to use a smaller proportion of visual features (point of subjective equality [PSE]=41.5%) to label a stimulus as Face compared to PD-NVH (51%) and controls (56.2%). In the discrimination task, criterion c was lower in the PD-VH group compared to controls (c: -0.16 vs. 0.27; P=0.005). In the Dalmatian dog task, the PD-VH group reported seeing livings beings more frequently than controls (P=0.040). A bias towards living beings was confirmed in the PD-VH group in the three tasks, and a bias toward non-living beings was measured in controls in the discrimination task. INTERPRETATION: Observing that controls exhibited bias toward non-living beings in the discrimination task, we suggest that impaired top-down control over perception processes explains the bias toward living beings in PD-VH visual misperceptions.
Predictors of quality of life and social participation in myotonic dystrophy type 1.
Davion JB, Tard C, Fragoso L … +3 more , Wilu-Wilu A, Defebvre L, Delbeuck X
Rev Neurol (Paris) · 2026 Mar · PMID 41469288 · Publisher ↗
INTRODUCTION: Myotonic dystrophy type 1 (DM1) is a genetic multisystemic disorder, affecting the muscles but also the brain and other organs, and impacting quality of life (QoL). Most of previous studies focused on healt...
INTRODUCTION: Myotonic dystrophy type 1 (DM1) is a genetic multisystemic disorder, affecting the muscles but also the brain and other organs, and impacting quality of life (QoL). Most of previous studies focused on health-related QoL and its predictors, which might restrict the possibility to observe the consequences of more general factors on QoL. METHODS: We studied QoL from a more global point of view in adult non-congenital DM1 patients included in the DM-VASCOG cohort using the World Health Organization Quality of Life Brief Version (WHOQOL-BREF) questionnaire, which discriminates four domains (physical health, psychological state, social relationships, and environment). Social participation was also evaluated using a questionnaire designed to assess the frequency of and the degree of satisfaction with the patient's involvement in social activities. Associations of these questionnaires with demographic, DM1-related, neuropsychological and behavioral measures were analyzed. RESULTS: Among our 122 DM1 patients, lower scores for the physical QoL were observed compared to other dimensions (P<0.001). QoL predictors were different among the physical (motor function, fatigue), psychological (anxiety, depression), social (education, depression) and environmental (anxiety, depression, fatigue) dimensions. Frequency of social participation in DM1 patients was associated with executive functions (Stroop test), while satisfaction with social participation was associated with depression and fatigue. CONCLUSION: The different dimensions of QoL and social participation in adult DM1 are associated with different modifiable factors. The effect on QoL of interventions focusing on these factors should be studied in future DM1 trials.
Gerstmann-Sträussler-Scheinker syndrome neuropathology in a Creutzfeldt-Jakob disease-like phenotype patient caused by a novel 6-OPRI sequence in the PRNP gene.
Sýkora M, Baranová S, Parobková E … +5 more , Moško T, Keller J, Holada K, Rusina R, Matěj R
Rev Neurol (Paris) · 2026 · PMID 41455648 · Publisher ↗
Gerstmann-Sträussler-Scheinker syndrome is an extremely rare hereditary human prion disease caused by distinct mutations in the prion protein-encoding gene and is frequently associated with a positive family history. The...
Gerstmann-Sträussler-Scheinker syndrome is an extremely rare hereditary human prion disease caused by distinct mutations in the prion protein-encoding gene and is frequently associated with a positive family history. The disease typically presents with progressive cerebellar symptoms such as gaze apraxia with limb ataxia and axial ataxia; thus, the diagnostic process is often challenging due to nonspecific clinical presentation. We present a case of a 73-year-old patient with no family history of dementia and cerebellar symptomatology during the course of rapidly progressing dementia. Owing to the clinical suspicion of prion disease, antemortem analysis of cerebrospinal fluid using a real-time quaking-induced conversion (RT-QuIC) assay was performed, with positive results. Postmortem histopathological examination confirmed a familiar form of human prion disease with concomitant asymptomatic tauopathy. An additional finding was a novel 6 octapeptide repeat insertion mutation in the prion gene. Familiar cases with an increasing number of repeated insertions seem to be associated with a longer overall disease course, milder clinical deterioration and often false-negative RT-QuIC results. The performance of RT-QuIC in inherited prion diseases may vary. Our case, involving a 6 octapeptide repeat insertion mutation, is particularly noteworthy due to the rapidly progressive clinical course and positive RT-QuIC results in both antemortem and postmortem tissue analyses.
Deep brain stimulation in patients with mixed movement disorders linked to ADCY5.
Retailleau E, Dorison N, Poulen G … +13 more , Roubertie A, Trouillard O, Baik J, Conabady E, François-Heude MC, Chauvet-Piat E, Spitz MA, Ravelli C, Vayssière P, Nilles C, Desjardins C, Dubacq C, Roze E
Rev Neurol (Paris) · 2026 Mar · PMID 41455647 · Publisher ↗
INTRODUCTION: Pathogenic variants in ADCY5 cause mixed hyperkinetic movement disorders (MxMD-ADCY5) that can be occasionally refractory to medical treatment. While deep brain stimulation of the globus pallidus internus (...
INTRODUCTION: Pathogenic variants in ADCY5 cause mixed hyperkinetic movement disorders (MxMD-ADCY5) that can be occasionally refractory to medical treatment. While deep brain stimulation of the globus pallidus internus (GPi-DBS) has been previously used, knowledge on its indication, efficacy and safety is poor and mainly based on anecdotal reports and short case series. METHODS: We retrospectively reviewed clinical, genetic, therapeutic, and surgical data from patients with ADCY5-related movement disorders who underwent GPi-DBS, operated in two French expert centres or previously published. RESULTS: We obtained data from 23 patients for analysis. There were two distinct indications for GPi-DBS. The first group consisted of patients with long-standing, pharmacoresistant hyperkinetic movements. The second group included patients with acute motor exacerbations - fulfilling criteria for status dystonicus - and conceptually aligned with the recently proposed framework of severe acute motor exacerbation (SAME). Overall, GPi-DBS was safe and led to mild-to-moderate improvement of the motor condition in the two groups of patients. CONCLUSION: GPi-DBS can be considered as a relevant therapeutic option for MxMD-ADCY5 in case of pharmacological treatment failure both in patients with chronic motor impairment and those with paroxysmal exacerbations meeting criteria for SAME. Given the response to DBS in the two groups of patients, it is plausible that MxMD-ADCY5 reflects basal ganglia dysfunction mediated by dysregulated cAMP signalling, and that DBS acts by restoring homeostatic inhibitory control in these circuits.
The role of kynurenine pathway metabolites in clinical features and chronic migraine.
Hasırcı Bayır BR, Ulutaş C, Nazlı E … +3 more , Köklü H, Güler EM, Ağırcan D
Rev Neurol (Paris) · 2026 · PMID 41449003 · Publisher ↗
OBJECTIVE: Migraine is a prevalent neurological disorder, yet its pathophysiology remains incompletely understood. While the role of neuroinflammation and metabolic dysregulation in migraine pathogenesis is increasingly...
OBJECTIVE: Migraine is a prevalent neurological disorder, yet its pathophysiology remains incompletely understood. While the role of neuroinflammation and metabolic dysregulation in migraine pathogenesis is increasingly recognized, the kynurenine pathway has gained attention due to its involvement in tryptophan metabolism. Our study aims to evaluate the relationship between kynurenine metabolism, the clinical characteristics of migraine, and the process of migraine chronification. MATERIALS AND METHODS: This study was designed as a prospective, observational study and was conducted with a total of 81 participants including 27 with episodic migraines, 27 with chronic migraines, and 27 healthy controls, aged 18-50years. Data collected included age, sex, pain type and location of migraine, attack frequency, severity, disease duration, and body mass index. Blood samples taken during the interictal phase were analyzed for Tryptophan (TRP), L-kynurenine (KYN), 3-hydroxykynurenine (3-HK), and 3-hydroxyanthranilic acid dioxygenase (3-HAAO) levels using ELISA kits. RESULTS: In the comparison between healthy controls and migraine groups, age, sex, and body mass index were similar. However, the patient group exhibited significantly lower levels of Trp, KYN, 3-HK, and 3-HAAO compared to the control group (P<0.001 for each). Laboratory analysis revealed higher levels of HAAO, 3-HK, KYN, and Trp in the episodic migraine group than in the chronic migraine group (Pp=0.027, P<0.001, P<0.001, P=0.002, respectively). ROC analysis revealed that 3-HK was identified as an independent risk factor for CM (OR=0.403, P<0.001). Painful headache days, monthly attack frequency, MIDAS, and HIT-6 scores were negatively correlated with HAAO, 3-HK, KYN, and Trp levels (P<0.005 for each). CONCLUSIONS: The study suggests that all four molecules are potentially involved in migraine clinical features and its chronification. Their levels decrease as migraine attacks worsen due to their neuroprotective effects. Future studies should explore targeted therapies to modulate kynurenine metabolism to prevent chronic migraine.
Intranasal insulin for mild cognitive impairment and Alzheimer's disease: A systematic review and meta-analysis of randomized controlled trials.
Silva AMP, Gonçalves OR, Tudella GCN … +8 more , Nascimento MVS, Pereira MAOM, Nantes LAO, Fernandes JVA, Quiroga DG, Lima DVS, Franco ES, Maia MBS
Rev Neurol (Paris) · 2026 · PMID 41436338 · Publisher ↗
BACKGROUND: Central insulin resistance has been implicated in the pathophysiology of Alzheimer's disease (AD), supporting the hypothesis that, in some individuals, AD may represent "type 3 diabetes." Intranasal insulin h...
BACKGROUND: Central insulin resistance has been implicated in the pathophysiology of Alzheimer's disease (AD), supporting the hypothesis that, in some individuals, AD may represent "type 3 diabetes." Intranasal insulin has been proposed as a non-invasive approach to enhance brain insulin signaling while minimizing peripheral metabolic effects, but clinical evidence remains inconsistent. METHODS: We conducted a systematic review and meta-analysis of randomized controlled trials (RCTs) comparing intranasal insulin with placebo in patients with mild cognitive impairment (MCI) or mild-to-moderate AD. Primary outcomes included changes in cognitive performance (ADAS-Cog13, CDR-SB, DSRS, delayed recall) and functional ability (ADCS-ADL). Secondary outcomes included cerebrospinal fluid (CSF) biomarkers (Aβ42, total tau, phosphorylated tau) and safety endpoints. Literature searches were performed in PubMed, Embase, and Cochrane Central up to April 2025. Random-effects models were used to pool effect estimates, and risk of bias was assessed using the Cochrane RoB 2 tool. RESULTS: Five RCTs enrolling 540 participants met the inclusion criteria. Pooled analyses showed no significant differences between intranasal insulin and placebo for ADAS-Cog13 (mean difference: -1.09 [95% CI: -4.89; 2.71]), ADCS-ADL (mean difference 0.06 [-0.33; 0.45]), CDR-SB, DSRS, or delayed recall. No significant effects were observed on CSF Aβ42, total tau, or p-tau181. Gastrointestinal adverse events were more frequent with insulin (risk ratio: 1.57; [95% CI: 1.15; 2.14]), whereas cardiovascular events were less frequent (risk ratio: 0.30 [0.12; 0.79]). No differences were found for other safety outcomes, and discontinuation rates were comparable between groups. CONCLUSION: Intranasal insulin was generally well tolerated but did not produce meaningful improvements in cognitive, functional, or biomarker outcomes in patients with MCI or mild-to-moderate AD. Current evidence does not support its routine clinical use, and further trials with standardized dosing, longer follow-up, and biomarker-stratified designs are warranted to clarify its therapeutic potential.
Small vessel disease: Trigger or bystander of late-onset epilepsy of unknown origin?
Hologne E, Chen Y, Moulin S … +1 more , Tyvaert L
Rev Neurol (Paris) · 2026 · PMID 41436337 · Publisher ↗
Late-onset epilepsy of unknown origin (LOEU) is a prevalent and disabling condition. Emerging evidence suggests a potential link between LOEU and new-onset dementia. Cerebral small vessel disease (cSVD) is a common patho...
Late-onset epilepsy of unknown origin (LOEU) is a prevalent and disabling condition. Emerging evidence suggests a potential link between LOEU and new-onset dementia. Cerebral small vessel disease (cSVD) is a common pathology and a major risk factor for both stroke and dementia. cSVD has been hypothesized to contribute to the development of LOEU and cognitive decline through blood-brain barrier dysfunction. This review summarizes current data exploring the association between LOEU and cSVD, highlighting conflicting results, probably due to major methodological limitations. Furthermore, in individuals over 60 years of age conditions such as obstructive sleep apnea (OSA), amyloidopathy, and tauopathy are frequently observed and independently associated with both LOEU and cSVD. To date, no robust evidence has established cSVD as a causal factor of LOEU. The complex interplay of these conditions necessitates further investigation to quantify the contribution of each pathology to the development of LOEU. Future studies using rigorous methodologies are required to determine whether cSVD acts as a primary trigger or merely represents a bystander in LOEU.
Refining the definition of severe migraine: Evidence from a prospective observational study.
Henri J, Redon S, Donnet A
Rev Neurol (Paris) · 2026 · PMID 41436336 · Publisher ↗
INTRODUCTION: Migraine severity is often assessed by attack frequency, but this single dimension fails to reflect the full burden of disease. We aimed to validate a pragmatic multidimensional definition of severe migrain...
INTRODUCTION: Migraine severity is often assessed by attack frequency, but this single dimension fails to reflect the full burden of disease. We aimed to validate a pragmatic multidimensional definition of severe migraine in a tertiary care setting. METHODS: We conducted an observational study including 96 consecutive adult migraine patients diagnosed according to ICHD-3 criteria at a tertiary pain center (Marseille, January-April 2024). Data collected included migraine days/month, HIT-6, MIGSEV, and HAD scores. Patients were categorized by frequency (<8 vs. ≥8days/month), HIT-6 (<60, 60-64,≥65), and MIGSEV grades (1-3). RESULTS: Median monthly frequency was 8days. Patients with≥8days/month had significantly higher HIT-6 scores (P=0.044). Among patients with<8days/month, both HIT-6≥65 and MIGSEV grade 3 identified individuals with greater functional disability and higher depressive symptoms, comparable to those in the high-frequency group. No demographic or comorbidity variables significantly distinguished severe cases. DISCUSSION: A multidimensional definition of severe migraine is supported: (A)≥8days/month, or (B)<8days/month with HIT-6≥65 and/or MIGSEV grade 3. This definition integrates frequency, functional impact, and perceived severity, providing a simple and reproducible framework to identify high-burden patients. It may improve preventive treatment decisions, referral to specialized care, and harmonization of research inclusion criteria.
Meige syndrome, a cranio-cervical dystonia with a unique clinical phenotype.
Marion MH, Flowers JM, Hicklin LA
Rev Neurol (Paris) · 2025 Dec · PMID 41429678 · Publisher ↗
BACKGROUND: The eponym "Meige syndrome" and the term "cranio-cervical dystonia" have been used inter-changeably in the literature and recently the validity of this eponym has been debated. OBJECTIVES: To study the unique...
BACKGROUND: The eponym "Meige syndrome" and the term "cranio-cervical dystonia" have been used inter-changeably in the literature and recently the validity of this eponym has been debated. OBJECTIVES: To study the uniqueness of the term "Meige syndrome", from a large cohort of patients with cranio-cervical dystonia. METHODS: We describe the 15years follow-up of a large cohort of 50 patients with Meige syndrome, seen in a neurology movement disorder clinic and in a multidisciplinary neuro-ear-nose-throat (ENT) clinic. Following the original description of Henry Meige (1866-1940), we have included patients with blepharospasm (BSP) at onset and anatomical spread of dystonia to the jaw, mouth floor and neck, to describe a clinical phenotype of Meige syndrome among the cranio-cervical dystonias. RESULTS: We report 2 groups: group 1, including 12 patients with cranial dystonia, with BSP spreading around the nose, the mouth, the masticatory muscles with opening or closing jaw dystonia; group 2, including 38 patients with cranio-cervical dystonia, with BSP spreading further to the mouth floor muscles and to the neck including cervical and laryngeal muscles. Among them, 20/38 patients had a clinical presentation dominated by ENT sphere symptoms. In 18/38 patients, the clinical picture is dominated by an anterocollis posture, progressing in the 7 most elderly patients to an extreme posture of chin on sternum. CONCLUSION: The eponym Meige syndrome should be reserved for a particular group of dystonic patients, unique in its clinical midline spasm phenotype, and in its rostro-caudal progression, starting with a BSP and resulting in a clinical spectrum with a continuum from cranial to cranio-cervical dystonia. Stormy dystonic episodes, often precipitated by stress, are important to recognise and can be a therapeutic challenge.
Short-term follow-up of anti-MAG neuropathy patients given bendamustine plus rituximab combination therapy.
Bezou N, Morel P, Hivert B … +3 more , Diab E, Merle P, Tard C
Rev Neurol (Paris) · 2025 Dec · PMID 41429677 · Publisher ↗
INTRODUCTION: Anti-myelin-associated glycoprotein (anti-MAG) IgM antibodies provoke demyelinating neuropathies, which can be particularly disabling. Conventionally, patients with anti-MAG neuropathies were treated with r...
INTRODUCTION: Anti-myelin-associated glycoprotein (anti-MAG) IgM antibodies provoke demyelinating neuropathies, which can be particularly disabling. Conventionally, patients with anti-MAG neuropathies were treated with rituximab alone. This study aimed to evaluate the clinical and electrophysiological efficacy of bendamustine-rituximab (BR) combination therapy for anti-MAG neuropathy patients. METHODS: We retrospectively consulted the medical records of 11 patients whose diagnosis of anti-MAG neuropathy was retained and who were given BR combination therapy at university and community hospitals in northern France between 2017 and 2022. We collected clinical scores and electrophysiological data before BR therapy and at six to eleven months after treatment end (mean=8.40months). RESULTS: After BR therapy, six of eleven patients (54%) showed an improvement in their overall neuropathy limitations scale (ONLS) score as did five of eleven (45%) in their peripheral neuropathy disability (PND) score, but these results did not reach statistical significance. For the six patients who had a more complete clinical revaluation based on the neuropathy impairment score (NIS), five improved (P=0.021). An improvement in the sensory NIS was observed in three of six patients (50%), while the motor NIS remained stable. Patients improving their PND score seemed to start from a higher baseline value. Five of eight (62%) patients with electrodiagnostic control data met Lunn and Nobile-Orazio's electrophysiological improvement criteria. The higher the baseline PND score, the less likely was improvement of electrodiagnostic parameters. No link between duration of disease progression before treatment and clinical response was found. CONCLUSION: This multicenter cohort of anti-MAG patients given BR combination therapy is, to our knowledge, the largest published to date. Although BR therapy seems to improve the clinical and electrophysiological outcome of anti-MAG patients, precise evaluation scales are nevertheless needed to highlight improvement. Sensitive scales such as the NIS should be used routinely for monitoring anti-MAG patients. Prospective studies with a larger number of patients would be useful to confirm the efficacy of BR therapy in these patients.
Trends in the real-world management of cluster headache patients treated by subcutaneous sumatriptan and/or oxygen in France - An analysis of the French National Social Security System Open Data over eleven years (2014-2024).
Van Obberghen EK, Fabre R, Bailly L … +1 more , Lanteri-Minet M
Rev Neurol (Paris) · 2026 · PMID 41407575 · Publisher ↗
UNLABELLED: The burden of cluster headache (CH) requires better knowledge of management to improve it. OBJECTIVES: To describe changes in the real-world management of CH treated with subcutaneous sumatriptan and/or oxyge...
UNLABELLED: The burden of cluster headache (CH) requires better knowledge of management to improve it. OBJECTIVES: To describe changes in the real-world management of CH treated with subcutaneous sumatriptan and/or oxygen in France over the period 2014-2024. METHODS: This is an analysis of two open data databases from the French Social Health Insurance ('Open Medic' and 'Open LPP'), providing an annual estimate from 2014 to 2024 of the delivery of subcutaneous sumatriptan and/or oxygen, the number of beneficiaries of these treatments and their socio-demographic profile. RESULTS: Annual deliveries of subcutaneous sumatriptan increased from 286,999 boxes in 2014 to 454,275 boxes in 2024 (58.2% increase). Beneficiaries of subcutaneous sumatriptan increased from 13,638 individuals in 2014 to 19,109 individuals in 2024 (40.1% increase). Annual deliveries of package for the weekly use of oxygen therapy equipment increased from 224,143 in 2014 to 790,768 in 2024 (2.5 times more). Beneficiaries of oxygen for CH increased from 7493 individuals in 2014 to 22,346 individuals in 2024 (2 times more). Over the period 2014-2024, the male to female ratio decreased from 2.3/1 to 1.5/1 and from 1.5/1 to 0.8/1 for individuals receiving subcutaneous sumatriptan and individuals receiving oxygen respectively. CONCLUSIONS: The delivery of subcutaneous sumatriptan and oxygen increased from 2014 to 2024, reflecting an improvement in the management of cluster headache in France. Nevertheless, given the one year-prevalence of this disease and the number of people expected to suffer from it, the number of people benefiting from these two treatments in 2024 indicates that there are still unmet needs. This study confirms the increase in the number of women treated for cluster headache observed over the last twenty years.
The importance of studying the history of neurosciences.
Fénelon G
Rev Neurol (Paris) · 2026 · PMID 41407574 · Publisher ↗
Abstract loading — click title to view on PubMed.
Assessment of social cognition impairments in patients with amyotrophic lateral sclerosis: How can it be improved? A systematic review.
Vovard B, Faure-de Baets J, Codron P … +2 more , Allain P, Cassereau J
Rev Neurol (Paris) · 2026 · PMID 41402228 · Publisher ↗
OBJECTIVES: The aim of this review was to evaluate the current evidence on which part of social cognition is impaired in amyotrophic lateral sclerosis (ALS) patients among emotion recognition, affective empathy and Theor...
OBJECTIVES: The aim of this review was to evaluate the current evidence on which part of social cognition is impaired in amyotrophic lateral sclerosis (ALS) patients among emotion recognition, affective empathy and Theory of Mind (ToM) and to suggest improvements in social cognition testing protocols. METHODS: A systematic review was conducted according to PRISMA guidelines. We included controlled cross-sectional or longitudinal studies published in English before September 1, 2024, that assessed social cognition in non-demented ALS patients. Searches were performed in Medline, Embase, Web of Science, and PsycINFO using specific MeSH terms. Studies using social cognition tests as a primary or secondary outcome were included. RESULTS: Thirty-four studies were analyzed. Impairments in emotion recognition - especially for negative emotions - were frequently reported, even in cognitively preserved ALS patients. Results for cognitive ToM were mixed and may be confounded by executive dysfunction or test limitations. In contrast, affective ToM deficits were more consistently identified. However, no included study directly assessed affective empathy. Tests used in the reviewed studies were often overly specific and lacked ecological validity, which may explain inconsistent results across domains and weak correlations with imaging or executive function. CONCLUSION: Social cognition is increasingly recognized as a key non-motor domain affected in ALS. However, current assessment tools may lack the sensitivity and ecological validity needed to capture real-life deficits. The implementation of dynamic, multimodal assessments such as real-life social interaction tests or tests like the Movie Assessment for Social Cognition (MASC) could improve detection and guide clinical interventions but remain to be validated for ALS patients.
Impact of HIV on disability progression in multiple sclerosis: An observational retrospective matched cohort study.
Moulignier A, Guillaume J, Pourcher V … +3 more , Maillart E, Januel E, Papeix C
Rev Neurol (Paris) · 2026 · PMID 41402227 · Publisher ↗
BACKGROUND: The coexistence of HIV infection and multiple sclerosis (MS) is uncommon, and poorly characterized. Whether HIV-related immune modulation influences MS, progression remains uncertain. Despite theoretical immu...
BACKGROUND: The coexistence of HIV infection and multiple sclerosis (MS) is uncommon, and poorly characterized. Whether HIV-related immune modulation influences MS, progression remains uncertain. Despite theoretical immunological interactions, real-world data are scarce. OBJECTIVES: To compare long-term disability outcomes in persons living with HIV (PLWHIVs) with MS (MS-PLWHIVs) versus matched HIV-negative MS controls (MS-controls). METHODS: This observational retrospective matched cohort study used databases from two French tertiary MS centers (1991-2021). Each MS-PLWHIV was matched with up to five MS-controls for MS subtype (relapsing-remitting or primary-progressive), sex, age at first MS attack (±5 years), and first-to-second attack interval (±5 years). The primary outcome was time to reach sustained EDSS-6 (cane use for≥12 months). Time-to-event analyses used Kaplan-Meier estimates and Cox regression adjusted for matching variables. RESULTS: We identified 16 MS-PLWHIVs and 75 matched MS-controls, with comparable baseline MS characteristics and a median follow-up>20 years. MS-PLWHIVs received significantly fewer MS disease-modifying therapies (MS-DMTs) (median: 0 [0-1] vs. 2 [1-3.5], P<0.001), and only 19% were still treated at study end versus 75% of controls. Notwithstanding this treatment gap, MS-PLWHIVs reached EDSS-6 a median of 8 years later than controls (22.8 vs. 14.5 years, P=0.05). However, the risk of reaching EDSS-6 did not differ significantly (adjusted HR: 1.60 [95% CI: 0.6-4.2], P=0.6). One-third of patients in each group converted to secondary progressive MS. CONCLUSION: Despite fewer MS-DMTs, MS-PLWHIVs did not show worse disability progression and even appeared to progress more slowly. While HIV-related immune modulation could contribute, this remains speculative. These findings, though limited by small sample size and retrospective design, provide rare long-term data on this understudied comorbidity.
Fertility ratio of functional/dissociative seizures compared to other neuropsychiatric disorders.
Nishi A
Rev Neurol (Paris) · 2026 · PMID 41402226 · Publisher ↗
Abstract loading — click title to view on PubMed.
Real-world comparison of treatments with antibodies targeting the CGRP pathway or botulinum toxin type A for resistant migraine.
Gardon L, Guy D, Pereira B … +6 more , Sickout-Arondo S, Mongaret C, Delage N, Picard P, Condé S, Moisset X
Rev Neurol (Paris) · 2026 · PMID 41387033 · Publisher ↗
BACKGROUND: Migraine, particularly in its chronic and treatment-resistant forms, imposes a significant burden on patients. Monoclonal antibodies targeting the calcitonin gene-related peptide (CGRP) pathway (anti-CGRP mAb...
BACKGROUND: Migraine, particularly in its chronic and treatment-resistant forms, imposes a significant burden on patients. Monoclonal antibodies targeting the calcitonin gene-related peptide (CGRP) pathway (anti-CGRP mAbs) and onabotulinum toxin type A (BTX-A) have emerged as key preventive treatments for resistant migraine. However, direct comparisons between these therapies remain limited. OBJECTIVES: This study aimed to compare the effectiveness of anti-CGRP mAbs and BTX-A in patients with resistant migraine in a real-world setting at a single tertiary care center in France. METHODS: A retrospective cohort study was conducted, including 93 patients treated with either anti-CGRP mAbs (n=50) or BTX-A (n=43) for six months. Propensity score matching and inverse probability of treatment weighting (IPTW) were applied to balance baseline differences between groups. The primary outcome was the proportion of patients achieving a ≥50% reduction in monthly headache days. RESULTS: After six months, 43% of patients in the anti-CGRP mAbs group and 18% in the BTX-A group achieved a ≥50% reduction in monthly headache days (P=0.003). Using a less stringent 30% threshold, response rates were 54% for anti-CGRP mAbs and 36% for BTX-A (P=0.06). Similar results were observed when the analysis was restricted to chronic migraine patients (n=79). The reduction in monthly headache days was significantly greater in the anti-CGRP mAbs group at both three months (P=0.015) and six months (P=0.022). CONCLUSION: Anti-CGRP mAbs demonstrated superior efficacy compared to BTX-A in resistant migraine patients.
Artificial intelligence in presymptomatic neurological diseases: Bridging normal variation and prodromal signatures.
Soulier T, Burgos N, Hassanaly R … +12 more , Pitombeira M, Solal M, Roy H, Hamzaoui M, Yazdan-Panah A, de Paula Faria D, Louapre C, Bodini B, Bottlaender M, Ayache N, Colliot O, Stankoff B
Rev Neurol (Paris) · 2025 Nov · PMID 41238324 · Publisher ↗
Presymptomatic neurological diseases are marked by early pathological changes that occur before overt clinical symptoms. These stages, which include prodromes such as REM sleep behavior disorder in Parkinson's or mild co...
Presymptomatic neurological diseases are marked by early pathological changes that occur before overt clinical symptoms. These stages, which include prodromes such as REM sleep behavior disorder in Parkinson's or mild cognitive impairment in Alzheimer's, offer critical opportunities for early intervention. However, their detection remains challenging due to the subtlety of changes and the overlap with normal interindividual variability. Artificial intelligence (AI), especially machine learning (ML) and deep learning (DL), offers new tools to uncover hidden signatures in complex biomedical data. First, we explore how supervised ML models can detect known prodromal patterns across diverse modalities, including EEG, cognitive scores, and structural imaging. Depending on the input, various model types - such as tree-based algorithms for structured data and convolutional or transformer networks for images and signals - can extract predictive features of early neurodegeneration. These approaches have demonstrated success in identifying at-risk individuals before clinical thresholds are reached. Yet, detecting only known patterns limits the scope of early intervention. Many individuals who will go on to develop neurological disease may not yet exhibit any recognized prodromal syndrome. Bridging this gap requires moving beyond predefined labels toward models capable of identifying subtle, unknown anomalies in individuals still considered healthy. Second, we address the detection of latent anomalies among individuals not yet considered at risk without identifiable known prodromal patterns. By mining clinical records, free-text medical notes, and population-level health databases (e.g., UK Biobank, EDS-AP-HP), and by analyzing sensor data from smartphones or wearables, AI can flag deviations from healthy patterns long before symptom onset or formal diagnosis. This approach holds promise for scalable, low-burden, ecological screening. Finally, we introduce the concept of pseudo-healthy twins - synthetic, personalized baselines generated from structural data such as MRI, to improve anomaly detection. These models predict a patient's expected healthy signal in another modality, such as PET, enabling the subtraction of normal anatomical and physiological variability to isolate disease-specific effects. Generative models like GANs and VAEs have shown promise in producing these cross-modal references, enhancing early anomaly detection in diseases like Alzheimer's and multiple sclerosis. Together, these approaches show how AI can bridge the gap between normal variation and early pathology, enabling more sensitive, personalized, and population-scalable detection of presymptomatic neurological disease.
Digital health in presymptomatic diseases.
Cohen M
Rev Neurol (Paris) · 2025 Nov · PMID 41238323 · Publisher ↗
Most of widely available consumer devices like smartphones and tablets are equipped with various sensors that allow for detection of subtle and undetectable neurological impairment of various neurological functions like...
Most of widely available consumer devices like smartphones and tablets are equipped with various sensors that allow for detection of subtle and undetectable neurological impairment of various neurological functions like motricity, coordination, cognition, visual function or eye movements. This opens the perspective of earlier diagnosis of neurological diseases, even at the preclinical stage, which could allow for earlier therapeutic intervention and improved long term outcomes. In this article, we review how technology can enhance the clinician's examination skills and the current level of evidence in the field of preclinical diseases detection, in diseases like Parkinson's disease, Alzheimer's disease or multiple sclerosis. Many studies reported subtle impairment regarding fine motricity, eye movements, cognition, voice features and speech. We will also discuss current limitations regarding scientific evidence and practical implementation in the daily practice, as well as future perspectives.
Late-onset epilepsy as a prodromal symptom.
Dupont S
Rev Neurol (Paris) · 2025 Nov · PMID 41238322 · Publisher ↗
Late-onset epilepsy (LOE) of unknown etiology accounts for 15-30% of all LOE cases. A critical question is whether these unexplained seizures represent a prodromal manifestation of an underlying neurological disorder - m...
Late-onset epilepsy (LOE) of unknown etiology accounts for 15-30% of all LOE cases. A critical question is whether these unexplained seizures represent a prodromal manifestation of an underlying neurological disorder - most notably, stroke or Alzheimer's disease (AD). Growing evidence suggests that seizures may be an early sign of subclinical cerebrovascular disease, serving as a warning signal for future stroke, or an early symptom of a neurodegenerative disorder, particularly AD, reflecting initial pathological changes such as amyloid-β and tau deposition. An alternative hypothesis proposes that shared risk factors - especially cardiovascular ones - underlie all three conditions: LOE, stroke, and AD. As a result, it is recommended that patients with LOE of unknown etiology undergo comprehensive cardiovascular evaluation and receive appropriate management of any identified risk factors. However, it remains unclear whether this approach is sufficient to prevent future strokes. Cognitive assessment is also essential in these patients. In this context, prodromal seizures may provide an opportunity to identify individuals suitable for early, targeted interventions aimed at slowing neurodegeneration.
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