BACKGROUND: Long-term disability progression data from Canadian multiple sclerosis (MS) populations remain limited. We examined disability progression patterns and prognostic factors in a large Canadian MS registry while...BACKGROUND: Long-term disability progression data from Canadian multiple sclerosis (MS) populations remain limited. We examined disability progression patterns and prognostic factors in a large Canadian MS registry while assessing the robustness of findings across different analytical approaches. METHODS: We analyzed 2,769 patients from the CHUM MS registry (1976-2020) with relapsing-remitting, secondary-progressive MS (SPMS), or clinically isolated syndrome. Primary outcomes were time to EDSS milestones 3, 4, 6, 8, and to SPMS from disease onset. Cox hazards models examined associations with sex, age at onset, calendar period, relapse frequency, pregnancy, and treatment timing. Sensitivity analyses used additional severity adjustments and alternative time origins to distinguish robust biological associations from methodology-sensitive findings. RESULTS: The cohort included 2,101 women and 668 men with median age at onset of 29.9 years and median follow-up of 24.6 years. Median time from disease onset to EDSS 3 was 21.3 years (95% CI: 20.1-22.4), to EDSS 4 was 26.1 years (24.7-27.8), to EDSS 6 was 34.1 years (31.8-35.8), and to SPMS was 36.4 years (35.5-38.1). Male sex consistently predicted faster progression across all milestones (HR 1.50-1.71, P<0.001). Age≥30 years at onset was associated with faster progression to earlier milestones. Pregnancy after disease onset was associated with slower progression (HR 0.70-0.86). High relapse frequency and treatment-timing effects varied markedly depending on analytical approach. CONCLUSION: This cohort demonstrates progression times comparable to international registries. Male sex emerged as the most robust prognostic factor, while other associations showed varying methodological sensitivity, highlighting complexity in interpreting observational MS research.
INTRODUCTION: Movement disorders (MD) can result from various mechanisms: central nervous system infection (20%), trauma (15%), metabolic dysregulation (7%), vascular causes (22%), or inflammatory, degenerative, and tumo...INTRODUCTION: Movement disorders (MD) can result from various mechanisms: central nervous system infection (20%), trauma (15%), metabolic dysregulation (7%), vascular causes (22%), or inflammatory, degenerative, and tumoral diseases. MD leading to the diagnosis of primary central nervous system lymphoma (PCNSL) are rare but may be treatable, depending on how quickly the diagnosis is made. The objective of this study was to explore movement disorders in PCNSL and discuss the potential pathophysiological relationships between PCNSL and symptoms. METHODS: We conducted a systematic review from databases inception (PubMed/MEDLINE and Google Scholar) through July 2025. RESULTS: Forty-five cases were found in the literature. An additional case from our records was included, with detailed clinical and paraclinical examinations for illustrative purposes (a 77-year-old man who experienced subacute left hemichorea-hemiballism movements, leading to the discovery of a right-sided lesion involving deep brain structures). Globally, the main movement disorders included gait impairment/ataxia (48%), parkinsonism (20%), chorea/ballism (14%), and tremor (10%). The mean age at onset was 59 years (ranging from 24 to 81), and the sex ratio was 23 males to 23 females. CONCLUSION: PCNSL may present with unusual symptoms like movement disorders (which may be of various types). Early diagnosis could enable prompt initiation of urgent treatments.
BACKGROUND: Medial temporal lobe amnestic syndrome (MTLA) is classically considered a hallmark of Alzheimer's disease (AD). However, emerging evidence suggests etiological heterogeneity, challenging the assumption that M...BACKGROUND: Medial temporal lobe amnestic syndrome (MTLA) is classically considered a hallmark of Alzheimer's disease (AD). However, emerging evidence suggests etiological heterogeneity, challenging the assumption that MTLA universally reflects AD pathology. OBJECTIVE: To determine the prevalence of amyloid pathology in isolated MTLA, identify phenotypic and genetic risk factors, and characterize associated network vulnerabilities in amnestic mild cognitive impairment (aMCI). METHOD: This retrospective observational study analyzed 55 patients with isolated MTLA at the aMCI stage. Participants underwent neuropsychological testing, cerebrospinal fluid (CSF) biomarker analysis, amyloid PET, and 18FDG-PET. Patients were stratified by amyloid status (positive/negative) and compared for APOE genotype, clinical features, and metabolic patterns. Statistical analyses included the Kruskal-Wallis test for non-parametric group comparisons and chi-square tests for categorical genetic associations. RESULTS: Amyloid pathology was observed in only 67% (37/55) of MTLA patients, dissociating the syndrome from AD in one-third of cases. Amyloid-positive patients demonstrated a significantly higher APOE ɛ4 carrier rate compared to amyloid-negative peers (χ=7.02, df=2, P=0.030), while 18FDG-PET revealed inferotemporal hypometabolism in amyloid-positive cases, marking early decontextualized memory impairment. CONCLUSION: MTLA syndrome is not homogeneous on the biological level and amyloid pathology and APOE ɛ4 genotype stratify patients into distinct subgroups. Amyloid-positive cases demonstrate inferotemporal hypometabolism, suggesting AD-related network vulnerability. By contrast, amyloid-negative MTLA group shows no systemic brain network vulnerabilities, likely due to its heterogeneous etiological origins. These findings advocate for a precision medicine framework integrating biomarkers to guide therapeutic strategies, moving beyond syndromic diagnoses to target underlying mechanisms.
Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is a recently defined inflammatory demyelinating disease of the central nervous system, affecting both children and adults. MOGAD pathological...Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is a recently defined inflammatory demyelinating disease of the central nervous system, affecting both children and adults. MOGAD pathologically differs from multiple sclerosis and neuromyelitis optica spectrum disorder (NMOSD). In this review, observational studies highlight an often-monophasic disease without an evident progression course and suggest, for a better recovery, the need for early and active anti-inflammatory action during attacks. We aim to specify hallmarks for the risk of relapse and long-term prognosis. Innovative clinical, biological and imaging biomarkers have been tested. Questions to be resolved are the optimal management of each attack and the definition of the window of opportunity to treat, looking to modify the risk of a potential relapsing disease course, with cumulative disability.
INTRODUCTION: Functional neurological disorders (FND) are a frequent reason for consultation in neurology, characterized by symptoms that can impair various functions, without any identifiable lesion. Diagnosis plays a k...INTRODUCTION: Functional neurological disorders (FND) are a frequent reason for consultation in neurology, characterized by symptoms that can impair various functions, without any identifiable lesion. Diagnosis plays a key role in patient adherence to treatment and the improvement of outcomes. However, the diversity of terms used to describe FND and etiological uncertainties reflect a lack of consensus, which may affect communication between healthcare professionals and patients. This raises the question of how healthcare providers explain the diagnosis of FND. OBJECTIVE: To explore similarities and differences in the language used by physicians, physiotherapists, and psychologists to explain FND to patients. METHOD: An exploratory qualitative study was conducted using individual semi-structured interviews with physicians, physiotherapists, and psychologists who had encountered at least one case of FND in the course of their practice. RESULTS: FND are associated with heterogeneous conceptualizations among healthcare professionals, ranging from psychological to neurological approaches. Despite this diversity marked by uncertainty, recognizing symptoms while reassuring patients remains essential. Diagnosis disclosure constitutes a complex process for clinicians due to its potential impact on patients, requiring appropriate communication to foster therapeutic alliance. This step requires an interprofessional dynamic, which is sometimes hindered by communication challenges and inconsistent narratives that disrupt care pathways. In this context, developing a common language emerges as a key imperative. CONCLUSION: This study highlights the importance of establishing coordinated care pathways for FND patients, which rely on harmonized communication among healthcare practitioners. Although resources exist, their implementation appears to be limited.
Lecanemab, the first anti-amyloid therapy approved by the European Medicines Agency, has demonstrated a statistically and clinically significant but moderate slowing of decline in early Alzheimer's disease (AD). In contr...Lecanemab, the first anti-amyloid therapy approved by the European Medicines Agency, has demonstrated a statistically and clinically significant but moderate slowing of decline in early Alzheimer's disease (AD). In contrast, its long-term impact on the disease course is not formally established. Any benefit, therefore, needs to be carefully weighed against its adverse effects and practical constraints, which must be discussed with patients and caregivers within a shared decision-making process (SMD). However, applying SDM in this context is challenging due to treatment complexity, cognitive impairment and the involvement of care partners. Supported decision-making, which aims to assist individuals with decisional limitations in participating in important choices, has been promoted as a relevant approach. In this bicentric study conducted in France, we evaluated a simulated supported decision-making process in 25 patients with early AD who were eligible for lecanemab and faced the decision to choose anti-amyloid immunotherapy. We created a written decision aid using a consensus-based method to provide clear, accurate, and scientifically validated information to patients and care partners. After a one-week reflection period, both patients and care partners demonstrated a good overall understanding of the information provided, though care partners showed higher levels of comprehension and retention. The treatment was considered acceptable by most patients and their care partners. While patients ultimately remain the decision-makers, these findings highlight the central role of care partners in strengthening supported decision-making, notably through frameworks such as the French "trusted person" model.
Desnoyers M, Bonjour M, Metereau E
… +12 more, Danaila T, Laurencin C, Jaulent P, Jaulent A, Liu L, Duraffourg M, Polo G, Simon E, Ferry T, Valour F, Thobois S, Prange S
BACKGROUND: Hardware-related infections may follow primary deep brain stimulation (DBS) implantation or implantable pulse generator (IPG) replacement with challenging management in fluctuating Parkinson's disease (PD) pa...BACKGROUND: Hardware-related infections may follow primary deep brain stimulation (DBS) implantation or implantable pulse generator (IPG) replacement with challenging management in fluctuating Parkinson's disease (PD) patients. OBJECTIVES: We aimed to investigate the incidence, risk factors, management and outcome of DBS-related infections in PD. METHODS: We performed a ten-year retrospective cohort study including all consecutive PD patients who underwent primary DBS implantation surgery and IPG replacement respectively. Incidence and risk factors of infections were analyzed in both cohorts using survival analysis, and infectious and neurological management was reported. RESULTS: The incidence of DBS-related infections was 11.3 (95%CI 6.3-18.7) per 1000 person-year following primary DBS implantation (293 patients) and 20.7 (95%CI 10.7-36.2) per 1000 person-year following IPG replacement (217 procedures in 188 patients). Infections were associated with a shorter disease duration before primary DBS implantation, but longer disease, DBS duration and a greater number of procedures for IPG replacement. Following DBS implantation, surgical management consisted in partial (3/15, 20%) or total (8/15, 53%) hardware removal, with good outcome in 12 (80%) patients. Infections resulted in increased short-term neurological worsening and challenging therapeutic management for total hardware removal and advanced PD patients. CONCLUSIONS: We found low incidence of DBS-related infections following primary DBS implantation or IPG replacement in PD patients, depending on PD duration. Our study highlights that patient-specific and multidisciplinary management in expert center resulted in good outcome and high rates of reimplantation following primary implantation-related infection, whereas PD patients with IPG replacement-associated infection had more severe outcome and challenging management.
Stress exposure, whether acute or chronic, is now recognized to be a determinant of epileptogenic vulnerability. Psychological stress or trauma may not only precipitate seizures but also actively contribute to the develo...Stress exposure, whether acute or chronic, is now recognized to be a determinant of epileptogenic vulnerability. Psychological stress or trauma may not only precipitate seizures but also actively contribute to the development of epilepsy, a concept that in the clinical setting could be termed "psychoepileptogenesis". Recent evidence from both animal models and clinical studies supports the role of emotional stress in facilitating epileptogenesis, particularly within limbic structures such as the amygdala and hippocampus. In rodent models, chronic stress has been shown to lower seizure thresholds and promote epileptogenesis through mechanisms involving brain-derived neurotrophic factor (BDNF) and dysregulation of the hypothalamic-pituitary-adrenal (HPA) axis. Human studies reinforce these findings: individuals exposed to trauma or suffering from post-traumatic stress disorder (PTSD) exhibit an elevated risk of developing epilepsy, especially temporal lobe epilepsy (TLE), with structural and functional neuroimaging revealing changes in limbic and paralimbic circuits. These converging lines of evidence suggest that psychoepileptogenesis is a plausible, albeit complex, phenomenon. Further research is needed to identify biomarkers of vulnerability and evaluate whether early interventions targeting stress pathways might alter the course of epileptogenesis.
OBJECTIVE: To develop standardized benchmarks for evaluating endovascular treatment of ischemic stroke by analyzing treatment efficacy and safety. This allows the comparison of new techniques and devices with solid stand...OBJECTIVE: To develop standardized benchmarks for evaluating endovascular treatment of ischemic stroke by analyzing treatment efficacy and safety. This allows the comparison of new techniques and devices with solid standards and quality control. MATERIALS AND METHODS: We conducted a systematic review and meta-analysis of randomized controlled trials from the MEDLINE, OVID, and Cochrane databases from January 2015 (MRCLEAN trial published date) to June 2025, focusing on predefined clinical outcomes. After applying the inclusion and exclusion criteria, 35 studies were included from the initial search of 1949 (1.8%). The benchmarks selected were modified treatment for ischemic cerebral infarction (mTICI)≥2b, modified Rankin Scale (mRS)≤2, symptomatic hemorrhagic transformation rate, and death rate. According to concept of confidence of interval (CI) 95%, everything that falls outside the border is defined as statistically significant. RESULTS: The I rages from 61.6% to 93.86%. The mTICI≥2b rate was 75.5% (95% CI: 75.7 to 81.5), the mRS≤2 was 43.5% (95% CI: 40.0 to 47.2), with a symptomatic hemorrhagic transformation rate of 5.4% (95% CI: 4.5 to 6.4), and a death rate of 18.4% (95% CI: 16.2 to 20.8) at three months. Subgroup analysis suggest trend in favour of higher mTICI≥2b rate intra-arterial thrombolysis+MT vs. MT. The mRS≤2 rate was lower in the large core trial and higher in the intra-arterial thrombolysis+MT. CONCLUSION: The derived benchmarks served as reference standards for assessing new endovascular treatments and quality control. This methodology enhances the quality of evidence, aids in clinical decision-making, and fosters advancements in treatment technologies.
BACKGROUND: Since 2005, the standard treatment for newly diagnosed glioblastoma involves radiotherapy combined with concomitant and adjuvant temozolomide (TMZ), as per the Stupp protocol. A known long-term complication o...BACKGROUND: Since 2005, the standard treatment for newly diagnosed glioblastoma involves radiotherapy combined with concomitant and adjuvant temozolomide (TMZ), as per the Stupp protocol. A known long-term complication of radiotherapy is Radiation-Induced Leukoencephalopathy (RIL), characterized by diffuse FLAIR-hyperintensities and cortico-subcortical brain atrophy, often leading to cognitive, balance and urinary impairments. While RIL has been typically described years after treatment in long survivors, there is little information regarding early-onset RIL (<6 months) and its potential risk factors. OBJECTIVE: This ancillary study aims to assess the incidence of early-onset RIL in glioblastoma patients treated within the ASTER phase III trial. Additionally, we explored potential risk factors, including genetic susceptibility, and evaluate whether Losartan, a PPARγ agonist, reduces early RIL incidence. METHODS: We conducted a retrospective analysis of data from the ASTER trial, a randomized, placebo-controlled phase III study evaluating the effects of Losartan on corticosteroid needs in glioblastoma patients receiving standard radiotherapy with concomitant TMZ. Patients were included if they had MRI scans available at baseline and six months post-treatment, with no evidence of tumor progression. Early-onset RIL was defined as an increase of at least two points on the simplified Scheltens rating scale, with associated cortical-subcortical atrophy. Genetic testing focused on the PPARγ germline SNP rs2120825. Statistical analyses included univariate comparisons of clinical and genetic variables. RESULTS: Among the 31 patients included, 29% (n=9) developed early-onset RIL. No significant associations were found between early RIL and known risk factors such as smoking (P=0.43), hypertension (P=0.12), age (P=0.067), or PPARγ TG polymorphism (P=0.68). Similarly, Losartan administration (100mg/day) showed no significant effect on prevention of early RIL (P=0.72). The limited power of the study may explain the lack of significant findings. CONCLUSION: This study reports a high incidence (29%) of early-onset RIL in glioblastoma patients treated with the Stupp protocol, highlighting the question of individual sensitivity to this severe side effect. While previous research suggests a role for PPARγ in radiation-induced toxicity, this study was underpowered to confirm whether Losartan, as PPARγ agonist, could offer protection against early-onset RIL. The lack of significant results underscores the need for larger, prospective trials to further investigate these factors and develop targeted preventive strategies.
BACKGROUND AND OBJECTIVES: The Montreal Cognitive Assessment has been recommended by the Movement Disorder Society (MDS) for screening cognitive disorders in Parkinson's disease (PD), although validations of language var...BACKGROUND AND OBJECTIVES: The Montreal Cognitive Assessment has been recommended by the Movement Disorder Society (MDS) for screening cognitive disorders in Parkinson's disease (PD), although validations of language variants are required. This study aimed to determine the reliability and validity of the Vietnamese Montreal Cognitive Assessment (MoCA-V) in Vietnamese PD patients. METHODS: PD patients from a movement disorder clinic at a tertiary hospital in Vietnam were recruited. Participants underwent the MoCA-V and Mini-Mental State Examination (MMSE) assessments. Patients were classified into three cognitive groups: dementia, mild cognitive impairment (MCI), and normal cognition (NC), based on the MDS diagnostic criteria. The reliability of the MoCA-V was determined through internal consistency and test-retest reliability. Validity was assessed through concurrent validity, convergent validity, and criterion validity. Exploratory analysis of diagnostic accuracy was performed as a secondary objective. RESULTS: Among 40 PD patients (dementia: 37.5%, MCI: 52.5%, NC: 10.0%), the MoCA-V demonstrated good internal consistency (Cronbach's alpha=0.83; 95% confidence interval [0.74-0.90]) and high test-retest reliability (total score's intraclass correlation coefficient=0.84 [0.69-0.92]; P<0.001). The total score showed strong correlations with the patient's cognitive status (Kendall's tau-b=0.63; P<0.001) and the MMSE total score (Spearman's rho=0.86; P<0.001), indicating high concurrent and convergent validity. The scale's criterion validity in discriminating cognitive impairment (including dementia and MCI) from NC and dementia from non-dementia was high (area under the curve [AUC]=0.98) and moderate (AUC=0.88), respectively. Notably, the MoCA-V demonstrated superior sensitivity compared to the MMSE in differentiating cognitive impairment from NC. CONCLUSIONS: The MoCA-V is a reliable and valid instrument for cognitive assessment in Vietnamese PD patients across all PD stages, with superior sensitivity to the MMSE for detection of cognitive impairment.
BACKGROUND: To analyze the clinical, radiological, therapeutic, and clinical outcomes of the leukodystrophy-like phenotype in neuropsychiatric systemic lupus erythematosus (NPSLE). METHODS: We conducted this systematic r...BACKGROUND: To analyze the clinical, radiological, therapeutic, and clinical outcomes of the leukodystrophy-like phenotype in neuropsychiatric systemic lupus erythematosus (NPSLE). METHODS: We conducted this systematic review following the Preferred Report Items for Systematic Review and Meta-analysis (PRISMA). We searched Pubmed, Embase, and Web of Science databases for articles published until May 31, 2025, using the terms "systemic lupus erythematosus" AND ("diffuse white matter lesions" OR "leukoencephalopathy" OR "leukodystrophies"). Additionally, we report five patients with leukodystrophy-like phenotype in early-onset NPSLE. RESULTS: Thirty-three cases were reviewed. The mean age was 36.9±14.9years, and 28 (84.8%) were female patients. A previous diagnosis of SLE was present in 66% of cases. The main neurological symptoms included headache (33.3%), seizures (15.1%), and consciousness disturbances (15.1%). Among the 17 patients with cerebrospinal fluid (CSF) abnormalities, elevated protein levels were observed in 11 (40.7%) cases, and pleocytosis in 6 (22.2%). MRI findings were reported in 31 patients, typically showing cerebral white matter lesions characterized by hyperintense areas with T2-weighted or fluid-attenuated inversion recovery (FLAIR) sequences. Most patients were treated with high-dose corticosteroid pulse therapy (22/32), while others received cyclophosphamide pulses (18/32), therapeutic plasma exchange (PLEX) (4/32), or rituximab (6/32). Overall, the therapeutic response was satisfactory, with clinical improvement in 23 out of 33 patients. CONCLUSION: In light of the severe clinical presentation of the leukodystrophy-like phenotype in NPSLE, early diagnosis and aggressive treatment are crucial for successful outcomes, as suggested by our review, which reported clinical improvement in 70% of patients. Future prospective studies are needed to confirm these findings.
BACKGROUND: Medication overuse headache (MOH) is a common and disabling condition associated with the excessive use of acute headache treatments. This study aimed to investigate MOH management by neurologists in France....BACKGROUND: Medication overuse headache (MOH) is a common and disabling condition associated with the excessive use of acute headache treatments. This study aimed to investigate MOH management by neurologists in France. METHODS: A vignette-based study was conducted among 107 neurologists (60 from private practice, 47 from hospitals or both hospital and private practice) in France. Participants evaluated four randomized clinical vignettes simulating real-life MOH cases and selected from five treatment strategies: (1) drug withdrawal and immediate initiation of preventive treatment, (2) initiation of preventive treatment without drug withdrawal, (3) initiation of preventive treatment with titration followed by drug withdrawal, (4) drug withdrawal alone, and (5) referral to a specialized centre. RESULTS: The most common strategies of MOH management involved combining drug withdrawal with preventive treatment: 44.1% of cases received preventive treatment with titration followed by drug withdrawal, and 42.5% received immediate preventive treatment alongside drug withdrawal. Most neurologists (82.6%) recommended outpatient care, while hospital-based neurologists were more likely to suggest inpatient treatment. A progressive drug withdrawal protocol was preferred by 73.4%, with the goal of limiting drug use to fewer than 8 days per month in 81.3% of cases. Preventive treatments were most commonly antidepressants (52.2%), followed by beta-blockers (24.4%) and antiepileptics (14.9%). Educational advice, non-pharmacological treatments, and psychological care were frequently recommended as complementary approaches. No significant associations were found between clinical parameters (e.g., drug type, comorbidities) and treatment choices. CONCLUSIONS: This study with an innovative approach provides valuable insight into the current management practices for MOH among neurologists in France, revealing alignment with established guidelines outlined by "La Société française d'études des migraines et céphalées (SFEMC)" guidelines, being withdrawal coupled with preventative treatment.
BACKGROUND: Clinical severity assessed by the National Institutes of Health Stroke Scale (NIHSS) score is not available in national hospital database. METHODS: Data from the French National Health Data System (SNDS) in t...BACKGROUND: Clinical severity assessed by the National Institutes of Health Stroke Scale (NIHSS) score is not available in national hospital database. METHODS: Data from the French National Health Data System (SNDS) in the region of the Brest Stroke Registry (BSR), were used to calculate a probability of death among hospitalized ischemic strokes (IS) and intracerebral hemorrhages (ICH), relying on multivariable logistic regressions using available stroke/patient characteristics. Data from the BSR, have been used to derive equations putting in relation initial NIHSS and probability of death. These equations were used to estimate initial NIHSS based on probability of death. Data from the Dijon Stroke Registry were used to assess the concordance between predicted and observed initial stroke severity levels outside of the geographical area of its training dataset. RESULTS: In the years 2012-2019, the BSR reported 5883 IS and 816 ICH among people aged 16 or above, while 5623 IS and 787 ICH could be identified in the SNDS. Ten-day mortality was found to be the best proxy for initial stroke severity. In the Dijon Stroke Registry, among 1,254 IS, our algorithm predicted 53.0% events of minor severity (initial NIHSS≤4), 38.6% events of intermediate severity (5-20), and 8.5% events of high severity (≥ 21), compared to known prevalence of 53.0%, 38.0%, and 9.0%, respectively. No reliable predictions could be made for ICH. CONCLUSION: The possible estimation of prevalence of initial stroke severity levels with satisfying performances in healthcare database is likely to improve epidemiological surveillance of this disease at national and local level.