Pub Date : 2026-01-01DOI: 10.1016/j.neurol.2025.12.002
J. Henri , S. Redon , A. Donnet
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. ≥ 8 days/month), HIT-6 (< 60, 60–64, ≥ 65), and MIGSEV grades (1–3).
Results
Median monthly frequency was 8 days. Patients with ≥ 8 days/month had significantly higher HIT-6 scores (P = 0.044). Among patients with < 8 days/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) ≥ 8 days/month, or (B) < 8 days/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.
{"title":"Refining the definition of severe migraine: Evidence from a prospective observational study","authors":"J. Henri , S. Redon , A. Donnet","doi":"10.1016/j.neurol.2025.12.002","DOIUrl":"10.1016/j.neurol.2025.12.002","url":null,"abstract":"<div><h3>Introduction</h3><div>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.</div></div><div><h3>Methods</h3><div>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. ≥<!--> <!-->8<!--> <!-->days/month), HIT-6 (<<!--> <!-->60, 60–64,<!--> <!-->≥<!--> <!-->65), and MIGSEV grades (1–3).</div></div><div><h3>Results</h3><div>Median monthly frequency was 8<!--> <!-->days. Patients with<!--> <!-->≥<!--> <!-->8<!--> <!-->days/month had significantly higher HIT-6 scores (<em>P</em> <!-->=<!--> <!-->0.044). Among patients with<!--> <!--><<!--> <!-->8<!--> <!-->days/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.</div></div><div><h3>Discussion</h3><div>A multidimensional definition of severe migraine is supported: (A)<!--> <!-->≥<!--> <!-->8<!--> <!-->days/month, or (B)<!--> <!--><<!--> <!-->8<!--> <!-->days/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.</div></div>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"182 1","pages":"Pages 92-96"},"PeriodicalIF":2.3,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145820586","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-01-01DOI: 10.1016/j.neurol.2025.10.005
N. Minier , V. Olié , M. Consigny , J. Coadic , E. Nowak , Y. Béjot , S. Timsit , A. Gabet
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.
{"title":"Estimation of initial stroke severity in hospital databases using NIHSS score from population-based stroke registries","authors":"N. Minier , V. Olié , M. Consigny , J. Coadic , E. Nowak , Y. Béjot , S. Timsit , A. Gabet","doi":"10.1016/j.neurol.2025.10.005","DOIUrl":"10.1016/j.neurol.2025.10.005","url":null,"abstract":"<div><h3>Background</h3><div>Clinical severity assessed by the National Institutes of Health Stroke Scale (NIHSS) score is not available in national hospital database.</div></div><div><h3>Methods</h3><div>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.</div></div><div><h3>Results</h3><div>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.</div></div><div><h3>Conclusion</h3><div>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.</div></div>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"182 1","pages":"Pages 49-58"},"PeriodicalIF":2.3,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146044088","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-12-29DOI: 10.1016/j.neurol.2025.11.008
J-B Davion, C Tard, L Fragoso, A Wilu-Wilu, L Defebvre, X Delbeuck
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.
{"title":"Predictors of quality of life and social participation in myotonic dystrophy type 1.","authors":"J-B Davion, C Tard, L Fragoso, A Wilu-Wilu, L Defebvre, X Delbeuck","doi":"10.1016/j.neurol.2025.11.008","DOIUrl":"https://doi.org/10.1016/j.neurol.2025.11.008","url":null,"abstract":"<p><strong>Introduction: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusion: </strong>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.</p>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":" ","pages":""},"PeriodicalIF":2.3,"publicationDate":"2025-12-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145865156","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-12-26DOI: 10.1016/j.neurol.2025.12.003
E Retailleau, N Dorison, G Poulen, A Roubertie, O Trouillard, J Baik, E Conabady, M-C François-Heude, E Chauvet-Piat, M-A Spitz, C Ravelli, P Vayssière, C Nilles, C Desjardins, C Dubacq, E Roze
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.
{"title":"Deep brain stimulation in patients with mixed movement disorders linked to ADCY5.","authors":"E Retailleau, N Dorison, G Poulen, A Roubertie, O Trouillard, J Baik, E Conabady, M-C François-Heude, E Chauvet-Piat, M-A Spitz, C Ravelli, P Vayssière, C Nilles, C Desjardins, C Dubacq, E Roze","doi":"10.1016/j.neurol.2025.12.003","DOIUrl":"https://doi.org/10.1016/j.neurol.2025.12.003","url":null,"abstract":"<p><strong>Introduction: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusion: </strong>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.</p>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":" ","pages":""},"PeriodicalIF":2.3,"publicationDate":"2025-12-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145846846","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-12-01DOI: 10.1016/j.neurol.2025.09.004
J. Dumurgier , B. Défontaines , K. Gallouj , B. Garcin , A. Garnier-Crussard , J. Lagarde , J.-M. Pauly , A. Rollin Sillaire , I. Rouch-Leroyer , M. Sarazin , M. Verny , D. Wallon
Alzheimer's disease (AD) is the most common neurodegenerative disorder and the leading cause of major neurocognitive disorder in older adults. Its diagnosis has evolved from clinical to clinico-biological criteria, integrating biomarkers such as beta-amyloid and phosphorylated tau in cerebrospinal fluid or specific positron emission tomography (PET) imaging. Recent therapeutic advances, including anti-amyloid immunotherapies, highlight the need for early and accurate diagnosis. Clinical presentation is heterogeneous and may include amnestic or non-amnestic forms. Diagnosis should be suspected in patients with progressive cognitive decline and confirmed through neuropsychological assessment and biomarker testing. Blood-based biomarkers are promising but not yet validated for routine use. Magnetic resonance imaging (MRI) is recommended for all patients with recent cognitive decline. The role of general practitioners in early detection is critical. These recommendations, developed by the French federation of memory clinics, provide guidance on diagnosis stages, biomarker indications, first-line assessments, referral criteria, and communication of diagnosis. They aim to standardize clinical practice and support timely, individualized care.
{"title":"Diagnosis of Alzheimer's disease: Recommendations from the French Federation of Memory Clinics","authors":"J. Dumurgier , B. Défontaines , K. Gallouj , B. Garcin , A. Garnier-Crussard , J. Lagarde , J.-M. Pauly , A. Rollin Sillaire , I. Rouch-Leroyer , M. Sarazin , M. Verny , D. Wallon","doi":"10.1016/j.neurol.2025.09.004","DOIUrl":"10.1016/j.neurol.2025.09.004","url":null,"abstract":"<div><div>Alzheimer's disease (AD) is the most common neurodegenerative disorder and the leading cause of major neurocognitive disorder in older adults. Its diagnosis has evolved from clinical to clinico-biological criteria, integrating biomarkers such as beta-amyloid and phosphorylated tau in cerebrospinal fluid or specific positron emission tomography (PET) imaging. Recent therapeutic advances, including anti-amyloid immunotherapies, highlight the need for early and accurate diagnosis. Clinical presentation is heterogeneous and may include amnestic or non-amnestic forms. Diagnosis should be suspected in patients with progressive cognitive decline and confirmed through neuropsychological assessment and biomarker testing. Blood-based biomarkers are promising but not yet validated for routine use. Magnetic resonance imaging (MRI) is recommended for all patients with recent cognitive decline. The role of general practitioners in early detection is critical. These recommendations, developed by the French federation of memory clinics, provide guidance on diagnosis stages, biomarker indications, first-line assessments, referral criteria, and communication of diagnosis. They aim to standardize clinical practice and support timely, individualized care.</div></div>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"181 10","pages":"Pages 1021-1029"},"PeriodicalIF":2.3,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145207523","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-12-01DOI: 10.1016/j.neurol.2025.09.008
D. Sablot , S. Rivas Lamelo , P. Renou , N. Nasr , P. Lavallee , C. Plantard , K. Blanc-Lasserre , V. Domigo , I. Sibon , Y. Béjot , C. Cordonnier , S. Alamowitch
Introduction
Transient ischemic attacks (TIAs) are associated with a high risk of early ischemic stroke. Timely and organized care is essential to prevent recurrence, as recommended by national guidelines. This survey aimed to describe current TIA management practices in French Stroke Units (SUs) and identify gaps relative to national and international recommendations.
Method
A declarative survey was sent by email to 139 French SU managers. Six reminders were sent to non-responders and partial responders. A link to the survey was also available on the French Neurovascular Society website from March 18 to November 1, 2023.
Results
A total of 123 SUs (88.5%) responded. A TIA clinic was identified in 15 SU (12% of respondents). In the other 108 SUs, no specific written procedure (63%), or a written procedure was applied at the SU (32%), and the corresponding healthcare territory (5%). The median time from admission to extra- and intra-cervical vessel imaging was 6 hours (IQR: 3–24), but in 25% of SUs, it was not provided in the first 24 hours after hospitalization. The median times to transthoracic echocardiogram and transesophageal echocardiogram were 4 days (IQR: 2–7) and 7 days (IQR: 4–14), respectively.
Conclusions
This study shows that dedicated TIA clinics are uncommon in France, but they are associated with faster diagnostic work-ups and shorter hospital stays. Expanding such structured care models within SUs could enhance the timeliness, consistency, and quality of TIA management nationwide, ultimately reducing the risk of recurrent stroke.
{"title":"Transient ischemic attack care pathways in stroke units: Findings from a French nationwide survey","authors":"D. Sablot , S. Rivas Lamelo , P. Renou , N. Nasr , P. Lavallee , C. Plantard , K. Blanc-Lasserre , V. Domigo , I. Sibon , Y. Béjot , C. Cordonnier , S. Alamowitch","doi":"10.1016/j.neurol.2025.09.008","DOIUrl":"10.1016/j.neurol.2025.09.008","url":null,"abstract":"<div><h3>Introduction</h3><div>Transient ischemic attacks (TIAs) are associated with a high risk of early ischemic stroke. Timely and organized care is essential to prevent recurrence, as recommended by national guidelines. This survey aimed to describe current TIA management practices in French Stroke Units (SUs) and identify gaps relative to national and international recommendations.</div></div><div><h3>Method</h3><div>A declarative survey was sent by email to 139 French SU managers. Six reminders were sent to non-responders and partial responders. A link to the survey was also available on the French Neurovascular Society website from March 18 to November 1, 2023.</div></div><div><h3>Results</h3><div>A total of 123 SUs (88.5%) responded. A TIA clinic was identified in 15 SU (12% of respondents). In the other 108 SUs, no specific written procedure (63%), or a written procedure was applied at the SU (32%), and the corresponding healthcare territory (5%). The median time from admission to extra- and intra-cervical vessel imaging was 6<!--> <!-->hours (IQR: 3–24), but in 25% of SUs, it was not provided in the first 24<!--> <!-->hours after hospitalization. The median times to transthoracic echocardiogram and transesophageal echocardiogram were 4<!--> <!-->days (IQR: 2–7) and 7<!--> <!-->days (IQR: 4–14), respectively.</div></div><div><h3>Conclusions</h3><div>This study shows that dedicated TIA clinics are uncommon in France, but they are associated with faster diagnostic work-ups and shorter hospital stays. Expanding such structured care models within SUs could enhance the timeliness, consistency, and quality of TIA management nationwide, ultimately reducing the risk of recurrent stroke.</div></div>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"181 10","pages":"Pages 998-1007"},"PeriodicalIF":2.3,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145452898","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-12-01DOI: 10.1016/j.neurol.2025.09.006
K. Chikh , J. Burel , A. Nikiema , H. Bulteau , D. Maltete , D. Wallon , E. Gerardin , R. Aboukais , T. Gaberel , S. Derrey , L. Grangeon
Background
Surgery for lobar intracerebral hemorrhages (ICH) associated with cerebral amyloid angiopathy (CAA) is believed to carry a high risk of postoperative rebleeding. The diagnosis of CAA is increasing with an aging population and external validation of the Edinburgh criteria on computed tomography (CT) scans. The aim of this study was to assess the postoperative risk of CAA-related ICH compared to non-CAA-related ICH.
Methods
We included patients admitted between 2008 and 2022 for spontaneous lobar ICH who underwent surgery at three university hospitals. A single-blinded neuroradiologist analyzed the Edinburgh criteria on the initial CT scan before surgery and assessed rebleeding on a repeat CT scan performed within 48 hours after surgery. Patients were classified into the “CAA group” according to the Edinburgh or Boston criteria, and into the “non-CAA group” if they had another cause of ICH.
Results
A total of 140 patients were included, with 23 in the CAA group, 93 in the non-CAA group, and 24 in the undetermined group. The postoperative rebleeding rate at 24–48 hours did not differ significantly between groups (13% in the CAA group vs. 15% in the non-CAA group, P > 0.99). The overall rate of rebleeding associated with clinical deterioration did not differ between groups (9% in the CAA group vs. 6% in the non-CAA group, P = 0.66). The overall mortality rate during the acute phase did not significantly differ between groups (4% in the CAA group vs. 12% in the non-CAA group, P = 0.46). The modified Rankin scale score three months after discharge ranged from 0 to 3 for 63% of CAA patients compared to 53% of non-CAA patients, with no significant difference (P = 0.59).
Conclusion
We did not find a significant difference in the postoperative rebleeding rate after ICH associated with CAA compared to other causes.
{"title":"Surgical outcome of cerebral amyloid angiopathy-related cerebral hemorrhage–A multicenter comparative study","authors":"K. Chikh , J. Burel , A. Nikiema , H. Bulteau , D. Maltete , D. Wallon , E. Gerardin , R. Aboukais , T. Gaberel , S. Derrey , L. Grangeon","doi":"10.1016/j.neurol.2025.09.006","DOIUrl":"10.1016/j.neurol.2025.09.006","url":null,"abstract":"<div><h3>Background</h3><div>Surgery for lobar intracerebral hemorrhages (ICH) associated with cerebral amyloid angiopathy (CAA) is believed to carry a high risk of postoperative rebleeding. The diagnosis of CAA is increasing with an aging population and external validation of the Edinburgh criteria on computed tomography (CT) scans. The aim of this study was to assess the postoperative risk of CAA-related ICH compared to non-CAA-related ICH.</div></div><div><h3>Methods</h3><div>We included patients admitted between 2008 and 2022 for spontaneous lobar ICH who underwent surgery at three university hospitals. A single-blinded neuroradiologist analyzed the Edinburgh criteria on the initial CT scan before surgery and assessed rebleeding on a repeat CT scan performed within 48<!--> <!-->hours after surgery. Patients were classified into the “CAA group” according to the Edinburgh or Boston criteria, and into the “non-CAA group” if they had another cause of ICH.</div></div><div><h3>Results</h3><div>A total of 140 patients were included, with 23 in the CAA group, 93 in the non-CAA group, and 24 in the undetermined group. The postoperative rebleeding rate at 24–48<!--> <!-->hours did not differ significantly between groups (13% in the CAA group vs. 15% in the non-CAA group, <em>P</em> <!-->><!--> <!-->0.99). The overall rate of rebleeding associated with clinical deterioration did not differ between groups (9% in the CAA group vs. 6% in the non-CAA group, <em>P</em> <!-->=<!--> <!-->0.66). The overall mortality rate during the acute phase did not significantly differ between groups (4% in the CAA group vs. 12% in the non-CAA group, <em>P</em> <!-->=<!--> <!-->0.46). The modified Rankin scale score three months after discharge ranged from 0 to 3 for 63% of CAA patients compared to 53% of non-CAA patients, with no significant difference (<em>P</em> <!-->=<!--> <!-->0.59).</div></div><div><h3>Conclusion</h3><div>We did not find a significant difference in the postoperative rebleeding rate after ICH associated with CAA compared to other causes.</div></div>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"181 10","pages":"Pages 981-990"},"PeriodicalIF":2.3,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145308979","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-12-01DOI: 10.1016/j.neurol.2025.10.001
M.-H. Marion , J.M. Flowers , L.A. Hicklin
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 15 years 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.
{"title":"Meige syndrome, a cranio-cervical dystonia with a unique clinical phenotype","authors":"M.-H. Marion , J.M. Flowers , L.A. Hicklin","doi":"10.1016/j.neurol.2025.10.001","DOIUrl":"10.1016/j.neurol.2025.10.001","url":null,"abstract":"<div><h3>Background</h3><div>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.</div></div><div><h3>Objectives</h3><div>To study the uniqueness of the term “Meige syndrome”, from a large cohort of patients with cranio-cervical dystonia.</div></div><div><h3>Methods</h3><div>We describe the 15<!--> <!-->years 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.</div></div><div><h3>Results</h3><div>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.</div></div><div><h3>Conclusion</h3><div>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.</div></div>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"181 10","pages":"Pages 1015-1020"},"PeriodicalIF":2.3,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145712352","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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