Revue neurologique最新文献

{"title":"Neural correlates of foreign accent syndrome: Is white matter the key to the mystery?","authors":"G Costentin, L Deheinzelin, L Zourdani, O Ozkul, A Triquenot, E Massardier, D Maltete, D Wallon, A Morin","doi":"10.1016/j.neurol.2025.08.003","DOIUrl":"https://doi.org/10.1016/j.neurol.2025.08.003","url":null,"abstract":"<p><strong>Background: </strong>Foreign accent syndrome is a rare entity with neural underpinnings that are not yet fully understood. Cases of neurogenic foreign accent syndrome due to right hemisphere lesion are very uncommon. We investigated the cortical and white matter lesions in a patient to better understand this syndrome.</p><p><strong>Case report: </strong>A 39-year-old French woman had a right middle cerebral artery stroke. This French native speaker was now perceived as speaking with an English accent, without signs of dysarthria or aphasia. Lesions involved right insula, pre-central gyrus and surrounding white matter tracts. After transformation in MNI-152 coordinate system, we compared the lesions in our patient with Tractotron software, a white matter tract tool providing a probability of fiber disconnection.</p><p><strong>Discussion: </strong>Speech evaluation suggested a disruption in the speech output motor system. We found lesions within this system, in anterior frontal and insular cortices, in accordance with previous descriptions. Besides structural grey matter damage, white matter also exhibited damage related to the stroke, particularly the frontal aslant tract. Very few studies have focused on the specific role of this tract in speech motor programming, but a role in speech production and stuttering have been described. Some cases of foreign accent syndrome appeared consecutively to right hemisphere lesions. There is growing evidence suggesting that right hemisphere lesions might lead to subclinical alteration of prosody contributing to the emergence of foreign accent syndrome.</p><p><strong>Conclusion: </strong>This study emphasizes the importance of analyzing subcortical areas and their associated tracts, which could play a crucial role in better understanding symptoms and anatomical correlation.</p>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":" ","pages":""},"PeriodicalIF":2.3,"publicationDate":"2025-10-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145318490","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}

{"title":"Predictive factors of epilepsy in a cohort of brain arteriovenous malformation patients with a 5-year follow-up.","authors":"B O Pirlog, M Porché, M Kyheng, J Labreuche, A Taleb, E Dubus, E Houdart, M Mazighi","doi":"10.1016/j.neurol.2025.09.007","DOIUrl":"https://doi.org/10.1016/j.neurol.2025.09.007","url":null,"abstract":"<p><strong>Background and aims: </strong>Data on factors associated with new-onset epilepsy in the follow-up of patients with brain arteriovenous malformations (BAVM) are scarce. We aimed to characterize the baseline patient and BAVM characteristics related to epilepsy and the predictive factors of new-onset epilepsy in BAVM patients during their follow-up.</p><p><strong>Patients and methods: </strong>We retrospectively analyzed 200 BAVM patients treated between 2000 and 2023. We used univariate and multivariate analyses to assess the association between baseline characteristics and seizures. The risk of developing seizures during follow-up was estimated using nonparametric survival analysis for interval-censored data.</p><p><strong>Results: </strong>In the present cohort, 90/200 patients (45%) had epilepsy at baseline. Patients with epilepsy were predominantly men (71.1%), and alcohol users (18.8%), and presented with BAVM located more often in the frontal lobe (55.6%) as compared to patients without epilepsy (40%, 7.4%, 23.6% respectively). Male gender was associated with higher prevalence of seizures at inclusion (OR 3.81 [95%CI, 1.77; 8.24]), while headaches (OR 0.19 [95% CI, 0.09; 0.41]), focal deficit (OR 0.22 [0.09; 0.49]) and BAVM occipital localization (OR 0.16 [95% CI, 0.05; 0.47]) remained associated with lower prevalence of epilepsy at inclusion. Among patients without epilepsy at baseline, 20.6% had new-onset epilepsy after five years. Baseline predictors of seizure occurrence included ruptured BAVM (HR 3.77 [95%CI 1.50; 9.44]) and surgery (HR 7.75, 95%CI, [2.04; 29.45]).</p><p><strong>Conclusions: </strong>For patients with newly diagnosed BAVM, male gender, and frontal topography were baseline characteristics associated with a higher risk of epilepsy. Among patients without epilepsy, ruptured BAVM and surgery at baseline were predictors of epilepsy at five years follow-up.</p>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":" ","pages":""},"PeriodicalIF":2.3,"publicationDate":"2025-10-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145313491","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}

{"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":"https://doi.org/10.1016/j.neurol.2025.09.006","url":null,"abstract":"<p><strong>Background: </strong>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.</p><p><strong>Methods: </strong>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 48hours 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.</p><p><strong>Results: </strong>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 24-48hours 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).</p><p><strong>Conclusion: </strong>We did not find a significant difference in the postoperative rebleeding rate after ICH associated with CAA compared to other causes.</p>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":" ","pages":""},"PeriodicalIF":2.3,"publicationDate":"2025-10-15","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}

{"title":"Disease modifying treatment of radiologically isolated syndrome: A systematic review of the use, efficacy, effectiveness, and safety.","authors":"D Ripsman, H Tremlett, A Alzahrani, N Makhani","doi":"10.1016/j.neurol.2025.09.005","DOIUrl":"https://doi.org/10.1016/j.neurol.2025.09.005","url":null,"abstract":"<p><strong>Background: </strong>Radiologically isolated syndrome (RIS) is characterized by incidental brain lesions suggestive of demyelination without symptoms of multiple sclerosis (MS). We systematically assessed the use, benefits, and adverse effects of DMTs for RIS.</p><p><strong>Methods: </strong>MEDLINE, EMBASE, and Web of Science were searched to identify English language studies including individuals with RIS treated with a DMT. Extracted data included patient characteristics, clinical progression, and adverse events. We conducted a meta-analysis using inverse probability weighting. Risk of bias (RoB) assessments used Cochrane's RoB-2 tool and Newcastle-Ottawa cohort study scale.</p><p><strong>Results: </strong>A total of 1012 abstracts were screened: 20 studies were included consisting of 2 RCTs, 12 observational cohort studies, and 6 case reports. A total of 1401 individuals with RIS were included; 291 (21%) received a DMT. The two RCTs randomized people with RIS to teriflunomide or dimethyl fumarate versus placebo and followed patients for at least 96weeks. In all other studies, follow-up ranged from 2months to 18years; only 3 studies exceeded 5years. DMT treatment was associated with a lower risk of a clinical demyelinating event (4 studies with different DMTs, adjusted hazard ratio=0.37 95% confidence interval [CI]: 0.15-0.95, high certainty). There was a higher rate of adverse events in DMT treated patients with RIS versus placebo (risk ratio=1.44, 95% CI: 1.09-1.90, moderate certainty). RoB was low for both RCTs, but high for 83% (10/12) of cohort studies.</p><p><strong>Conclusions: </strong>DMTs reduced the risk of a clinical demyelinating event in individuals with RIS, albeit with more adverse events compared to placebo. However, no literature addressed longer-term benefits/adverse effects.</p>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":" ","pages":""},"PeriodicalIF":2.3,"publicationDate":"2025-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145308956","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}

{"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":"https://doi.org/10.1016/j.neurol.2025.09.004","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":" ","pages":""},"PeriodicalIF":2.3,"publicationDate":"2025-09-30","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}

{"title":"First-bite syndrome following triptan intake: A report of three cases.","authors":"V Fayolle, N Moreau, S Redon, G Demarquay","doi":"10.1016/j.neurol.2025.09.002","DOIUrl":"https://doi.org/10.1016/j.neurol.2025.09.002","url":null,"abstract":"","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":" ","pages":""},"PeriodicalIF":2.3,"publicationDate":"2025-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145200943","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}

{"title":"Chronic meningoencephalitis due to enterovirus A71 complicating rituximab therapy.","authors":"A David, M Claudé, G Martin de Frémont, G Trédez, C Henry","doi":"10.1016/j.neurol.2025.09.003","DOIUrl":"https://doi.org/10.1016/j.neurol.2025.09.003","url":null,"abstract":"<p><strong>Background: </strong>Rituximab and other anti-CD20 therapies are increasingly used in the treatment of autoimmune and hematologic disorders. These treatments are associated with persistent immune impairment, potentially leading to severe infections. We describe a resolutive case of proven chronic enterovirus A71 (EV A71) meningoencephalitis complicating rituximab maintenance therapy for non-Hodgkin lymphoma.</p><p><strong>Methods: </strong>This article combines an original case report and a literature review of cases of enteroviral meningoencephalitis complicating rituximab treatment.</p><p><strong>Results: </strong>A 38-year-old man was treated with rituximab and chemotherapy for a mantle cell lymphoma. During maintenance treatment with rituximab, he developed a \"hand-foot-mouth disease\", and one month later severe neurological deterioration including quadriparesis and major neurocognitive disorder leading to a diagnosis of chronic enteroviral meningoencephalitis. A treatment associating monthly intravenous immunoglobulins (IVIg) and fluoxetine was initiated two months after neurological symptoms onset, resulting in dramatic clinical improvement within six months. A brief literature review shows that a treatment with high-dose IVIg often results in clinical improvement. Fluoxetine was added in recent reports based on in vitro evidence of anti-viral activity against enteroviruses.</p><p><strong>Discussion: </strong>Enteroviral infection should be evoked in patients treated with rituximab presenting with an encephalitic symptomatology, and restoring humoral immunity with high-dose IVIg might improve their condition.</p>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":" ","pages":""},"PeriodicalIF":2.3,"publicationDate":"2025-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145200877","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}

{"title":"Clinical presentation and natural history of RAVINE leukoencephalopathy in Reunion Island","authors":"M. Renouil ,&nbsp;I.I. Grigorashvili-Coin ,&nbsp;M.-L. Jacquemont ,&nbsp;A. Gelot ,&nbsp;V. Trommsdorff ,&nbsp;A. Pervillé ,&nbsp;F. Darcel ,&nbsp;M. Bintner ,&nbsp;A. Choumert","doi":"10.1016/j.neurol.2025.06.014","DOIUrl":"10.1016/j.neurol.2025.06.014","url":null,"abstract":"<div><h3>Introduction</h3><div>RAVINE leukoencephalopathy (RLE) is a hereditary autosomal recessive disease characterized by typical clinical and radiological signs that has so far been observed only in patients of Reunionese origin. The term RAVINE is a French acronym for the main clinical features of the disease: <em>Réunion, Anorexie, Vomissements Incoercibles, signes NEurologiques</em> (Reunion, Anorexia, Intractable Vomiting, NEurological signs). Patients with RLE carry the IVS1-1778A&gt;G mutation of the <em>SLC7A2</em> gene in the homozygous state. Here we present the first complete clinical description and natural history of RLE.</div></div><div><h3>Material and methods</h3><div>The medical records of all patients born to Reunionese parents and presenting with suspected RLE were reviewed. The diagnosis of RLE was confirmed by detection of the homozygous mutation IVS1-1178A&gt;G of the <em>SLC7A2</em> gene. The clinical and paraclinical data of patients with genetically confirmed RLE were retrospectively analyzed to determine the clinical presentation and natural history of the disease.</div></div><div><h3>Results</h3><div>Our retrospective analysis of the clinical and paraclinical data of 40 patients with genetically confirmed RLE distinguished 3 types of the disease based on the evolution of symptoms. Symptoms were classified into 4 stages of development: stage A, or digestive-like stage, characterized by digestive symptoms and to a lesser extent by neurological symptoms; stage B, or clinically latent stage; stage C, or exacerbation stage, marked by attacks of neurological symptoms; and stage D, or decline stage, characterized by loss of walking ability and progression towards death. Patients with type I RLE (37.5%), or monophasic RLE, experience only the severe stage A. Death occurs before the age of 28<!--> <!-->months in a very narrow time window (23.0<!--> <!-->±<!--> <!-->2.2<!--> <!-->months). Patients with type II RLE (16.7%), or biphasic RLE, move directly from stage A to stage D. Patients with type III RLE (45.8%), or multiphasic RLE, experience all 4 stages with varying degrees of symptom severity. This is the most frequent type of RLE. The joint occurrence of central apnea and vocal cord paralysis during stage A is indicative of unfavorable prognosis. Corticosteroid therapy seems to be effective during stage A and in some cases during stage C.</div></div><div><h3>Discussion</h3><div>This retrospective study provides the first complete clinical description and natural history of RLE. Three types of the disease were distinguished based on the evolution of symptoms. The diagnosis of RLE can be established outside of Reunion Island as many Reunionese couples have emigrated to other parts of the world.</div></div>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"181 8","pages":"Pages 775-789"},"PeriodicalIF":2.3,"publicationDate":"2025-09-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145134922","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}

{"title":"Investigating neuroanatomical correlates of neuropathic pain in multiple sclerosis: A pilot comparative study using advanced MRI techniques","authors":"G. Fillebeen ,&nbsp;M. Ocampo-Pineda ,&nbsp;E. Ruberte ,&nbsp;L. Melie-Garcia ,&nbsp;J. Kuhle ,&nbsp;F.T. Kurz ,&nbsp;K.-O. Lovblad ,&nbsp;P.H. Lalive ,&nbsp;A.M. Lascano","doi":"10.1016/j.neurol.2025.06.011","DOIUrl":"10.1016/j.neurol.2025.06.011","url":null,"abstract":"<div><h3>Background</h3><div>Previous studies exploring the anatomical correlates of pain in multiple sclerosis (MS) have relied on structural magnetic resonance imaging (MRI) and descriptive methodologies.</div></div><div><h3>Objective</h3><div>To establish radiological correlates of neuropathic pain in MS patients through the objective segmentation and analysis of brain MRI.</div></div><div><h3>Methods</h3><div>This exploratory pilot study included three distinct groups: MS patients with neuropathic pain (<em>n</em> <!-->=<!--> <!-->8), MS patients without pain (<em>n</em> <!-->=<!--> <!-->11), and individuals with small fiber neuropathy (SFN, <em>n</em> <!-->=<!--> <!-->6). Neuropathic pain was confirmed using laser-evoked potentials (LEPs), ensuring an objective assessment of pain function. All participants underwent brain MRI, with MS patients additionally undergoing spinal MRI. Brain region segmentation was conducted using two advanced automated tools: SAMSEG (Sequence Adaptive Multimodal SEGmentation) and SynthSEG. Pain-related brain regions, including the thalamus, brainstem, basal ganglia, prefrontal cortex, and somatosensory cortex, were analyzed and compared amongst the three groups.</div></div><div><h3>Results</h3><div>The volume of the right pallidum was significantly reduced in MS patients with pain compared to those without pain, as measured by SynthSeg but not with SAMSEG. Individual analysis of regions of interest showed significant results of diffusion tensor imaging analysis in the external capsule, internal capsule, posterior thalamic radiation, and superior longitudinal fasciculus. Quantitative analysis of spinal cord lesions revealed no significant differences between the groups.</div></div><div><h3>Conclusions</h3><div>These findings highlight a potential of advanced neuroimaging techniques to uncover brain-based correlates of neuropathic pain in MS, though further studies with larger sample sizes are warranted for validation.</div></div>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"181 8","pages":"Pages 765-774"},"PeriodicalIF":2.3,"publicationDate":"2025-08-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144967011","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}

{"title":"Transthyretin variant cerebral amyloid angiopathy fulfilling the modified Boston criteria: Retrospective data from the Bicêtre hospital cohort","authors":"J.-B. Brunet de Courssou ,&nbsp;L. Gorza ,&nbsp;M. Babin ,&nbsp;G. Nasser ,&nbsp;C. Ancelet ,&nbsp;C. Labeyrie ,&nbsp;C. Denier ,&nbsp;C. Cauquil","doi":"10.1016/j.neurol.2025.07.012","DOIUrl":"10.1016/j.neurol.2025.07.012","url":null,"abstract":"<div><h3>Background</h3><div>Hereditary transthyretin amyloidosis (ATTRv) is the most common hereditary amyloidosis, affecting mainly the peripheral nervous system and the heart. Central nervous system (CNS) involvement is regarded as rare, apart in some <em>TTR</em> variants, although leptomeningeal amyloid deposit had been described in the frequent Val30Met variant. Cerebral amyloid angiopathy (CAA) is a neurovascular disease characterized by amyloid deposits in brain vasculature, leading to ischemic and hemorrhagic events. The main cause is sporadic CAA with β-amyloid deposits but hereditary causes are possible.</div></div><div><h3>Methods</h3><div>We analyzed a cohort of ATTRv symptomatic patients treated at Bicêtre university hospital who underwent brain magnetic resonance imaging (MRI) regardless of indication.</div></div><div><h3>Results</h3><div>Twenty out of 64 ATTRv patients (31%) fulfilled radiological criteria of possible or probable CAA (suspected CAA) according to the modified Boston criteria with an unusually high 77% (10/13) frequency of leptomeningeal enhancement. Clinically, suspected CAA patients tended to have more focal central neurological symptoms, seizures and memory complaints than ATTRv patients without those MRI features.</div></div><div><h3>Conclusion</h3><div>ATTRv-related CAA should be considered in case of CAA with suggestive systemic features or familial history, as specific treatments and genetic counseling now exist for ATTRv. Conversely, CNS symptoms and brain MRI abnormalities should be sought in ATTRv patients.</div></div>","PeriodicalId":21321,"journal":{"name":"Revue neurologique","volume":"181 8","pages":"Pages 801-807"},"PeriodicalIF":2.3,"publicationDate":"2025-08-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144859467","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}