Annals of Neurology最新文献

{"title":"Adult Neurogenesis in the Subventricular Zone of Patients with Huntington's and Parkinson's Diseases and following Long-Term Treatment with Deep Brain Stimulation","authors":"Marta Snapyan,&nbsp;Francis Desmeules,&nbsp;Jonathan Munro,&nbsp;Morgan Bérard,&nbsp;Stephan Saikali,&nbsp;Peter V. Gould,&nbsp;Maxime Richer,&nbsp;Emmanuelle Pourcher,&nbsp;Mélanie Langlois,&nbsp;Anne-Marie Dufresne,&nbsp;Michel Prud'homme,&nbsp;Léo Cantin,&nbsp;André Parent,&nbsp;Armen Saghatelyan,&nbsp;Martin Parent PhD","doi":"10.1002/ana.27181","DOIUrl":"10.1002/ana.27181","url":null,"abstract":"<div>\u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>Parkinson's and Huntington's diseases are characterized by progressive neuronal loss. Previous studies using human postmortem tissues have shown the impact of neurodegenerative disorders on adult neurogenesis. The extent to which adult neural stem cells are activated in the subventricular zone and whether therapeutic treatments such as deep brain stimulation promote adult neurogenesis remains unclear. The goal of the present study is to assess adult neural stem cells activation and neurogenesis in the subventricular zone of patients with Huntington's and Parkinson's diseases who were treated or not by deep brain stimulation.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Postmortem brain samples from Huntington's and Parkinson's disease patients who had received or not long-term deep brain stimulation of the subthalamic nucleus were used.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Our results indicate a significant increase in the thickness of the subventricular zone and in the density of proliferating cells and activated stem cells in the brain of Huntington's disease subjects and Parkinson's disease patients treated with deep brain stimulation. We also observed an increase in the density of immature neurons in the brain of these patients.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>Overall, our data indicate that long-term deep brain stimulation of the subthalamic nucleus promotes cell proliferation and neurogenesis in the subventricular zone that are reduced in Parkinson's disease. Taken together, our results also provide a detailed characterization of the cellular composition of the adult human subventricular zone and caudate nucleus in normal condition and in Parkinson's and Huntington's diseases and demonstrate the plasticity of these regions in response to neurodegeneration. ANN NEUROL 2025;97:894–906</p>\u0000 </section>\u0000 </div>","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":"97 5","pages":"894-906"},"PeriodicalIF":8.1,"publicationDate":"2025-01-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/ana.27181","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142996754","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Synaptic Density Reductions in MSA: A Potential Biomarker Identified Through [18F]SynVesT-1 PET Imaging","authors":"Jian Li MD,&nbsp;Daji Chen MM,&nbsp;Yongxiang Tang MD,&nbsp;Zhao Chen MM,&nbsp;Ming Zhou M.Parm,&nbsp;Linlin Wan MD,&nbsp;Ling Xiao MM,&nbsp;You Fu MM,&nbsp;Zhiyou He M.Eng,&nbsp;Zhichao Tang MD,&nbsp;Zhengqun Hu MM,&nbsp;Xinrong Yuan MM,&nbsp;Jinhui Yang MM,&nbsp;Sudan Zhu MM,&nbsp;Xuan Guo MM,&nbsp;Riwei Ouyang MD,&nbsp;Rong Qiu D.Eng,&nbsp;Beisha Tang MD,&nbsp;Jifeng Guo MD,&nbsp;Hong Jiang MD,&nbsp;Shuo Hu MD","doi":"10.1002/ana.27179","DOIUrl":"10.1002/ana.27179","url":null,"abstract":"<div>\u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>The objective of this study was to delineate synaptic density alterations in multiple system atrophy (MSA) and explore its potential role as a biomarker for MSA diagnosis and disease severity monitoring using [¹⁸F]SynVesT-1 positron emission tomography / computed tomography (PET CT).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>In this prospective study, 60 patients with MSA (30 patients with MSA-parkinsonian [MSA-P] subtype and 30 patients with MSA-cerebellar [MSA-C] subtype), 30 patients with Parkinson's disease (PD), and 30 age-matched healthy controls (HCs) underwent [¹⁸F]SynVesT-1 PET/CT for synaptic density assessment. Visual, voxel, and volumetric region of interest (VOI) analyses were used to elucidate synaptic density patterns in the MSA brain and establish diagnostic criteria. The diagnostic performances of both visual and VOI-based diagnostics were evaluated using receiver operating characteristic (ROC) analysis. Spearman correlation analyses were conducted to investigate the relationship between brain synaptic density and disease severity</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Patients with MSA displayed extensive reductions in synaptic density throughout the brain, notably affecting both primary VOIs (the cerebellum and putamen) and secondary VOIs including the medulla oblongata, ventral tegmental area, and pons. Notably, patients with MSA-C exhibited a remarkable decrease in cerebellar synaptic density, whereas patients with MSA-P demonstrated significant synaptic loss within the posterior putamen. Compared with patients with PD, the patients with MSA show a more pronounced reduction in synaptic density in infratentorial brain regions. VOI-based diagnosis significantly outperformed visual analysis in diagnosing and differentiating MSA and its subtypes. Synaptic density in primary and multiple secondary VOIs correlated significantly with motor scales in patients with MSA.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>Our study identified widespread synaptic density reductions in MSA, particularly in the basal ganglia and infratentorial region, suggesting [¹⁸F]SynVesT-1 PET as a potential biomarker for diagnosing and evaluating the disease, and guiding synaptic restoration trials. ANN NEUROL 2025;97:879–893</p>\u0000 </section>\u0000 </div>","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":"97 5","pages":"879-893"},"PeriodicalIF":8.1,"publicationDate":"2025-01-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142996772","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Quantitative Pupillometry Predicts Neurologic Deterioration in Patients with Large Middle Cerebral Artery Stroke","authors":"Yili Du MS,&nbsp;Jack E. Pohlmann MS,&nbsp;Stefanos Chatzidakis MD,&nbsp;Benjamin Brush MD,&nbsp;Leigh Ann Malinger BS,&nbsp;Rebecca A. Stafford BA,&nbsp;Anna M. Cervantes-Arslanian MD,&nbsp;Emelia J. Benjamin MD, ScM,&nbsp;Emily J. Gilmore MD,&nbsp;Josée Dupuis PhD,&nbsp;David M. Greer MD, MA,&nbsp;Stelios M. Smirnakis MD, PhD,&nbsp;Shariq Mohammed PhD,&nbsp;Charlene J. Ong MD, MPHS","doi":"10.1002/ana.27178","DOIUrl":"10.1002/ana.27178","url":null,"abstract":"<div>\u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>This study assesses whether longitudinal quantitative pupillometry predicts neurological deterioration after large middle cerebral artery (MCA) stroke and determines how early changes are detectable.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This prospective, single-center observational cohort study included patients with large MCA stroke admitted to Boston Medical Center's intensive care unit (2019–2024). Associations between time-to-neurologic deterioration and quantitative pupillometry, including Neurological Pupil Index (NPi), were assessed using Cox proportional hazards models with time-dependent covariates adjusted for age, sex, and Alberta Stroke Program Early CT Score. Models using dilation velocity were compared with partial likelihood ratio tests. Pupillometric changes over 2-h intervals in the 12 h preceding deterioration were analyzed with linear mixed-effects modeling and Tukey's test. Matched referents (age, sex, stroke side, follow-up duration) were used for comparison. Optimal thresholds were identified using the Youden Index.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Among 71 patients (mean age 66.5 years; 59.2% women), 32 (45.1%) experienced deterioration. A 1-unit decrease in NPi was associated with a higher hazard of deterioration (hazard ratio 2.46; 95% confidence interval 1.68–3.61). Dilation velocity improved model performance compared to NPi alone. NPi was significantly lower at 0–2 h (3.81 vs. 4.38, p = 0.001) and 2–4 h (3.71 vs. 4.38, p &lt; 0.001) before deterioration compared to 10–12 h prior. Optimal thresholds were 4.01 for NPi, 0.49 mm/s for dilation velocity, and −0.15 change in NPi over 12 h.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>Quantitative pupillometry predicts neurological deterioration in MCA stroke, with declines detectable up to 12 h prior. Dilation velocity shows promise as a novel biomarker. ANN NEUROL 2025;97:930–941</p>\u0000 </section>\u0000 </div>","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":"97 5","pages":"930-941"},"PeriodicalIF":8.1,"publicationDate":"2025-01-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142996763","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Immunotherapy-Resistant Neuropathic Pain and Fatigue Predict Quality-of-Life in Contactin-Associated Protein-Like 2 Antibody Disease","authors":"Bryan Ceronie,&nbsp;Christine Strippel,&nbsp;Christopher Uy,&nbsp;Sofija Paneva,&nbsp;Mateusz Makuch,&nbsp;Babak Soleimani,&nbsp;Sanchit Turaga,&nbsp;Sophie Binks,&nbsp;Sudarshini Ramanathan,&nbsp;Sophia Michael,&nbsp;James Varley,&nbsp;Ava Easton,&nbsp;Andreas Themistocleous,&nbsp;John Dawes,&nbsp;David L. Bennett,&nbsp;Anushka Irani,&nbsp;Adam E. Handel,&nbsp;Sarosh R. Irani","doi":"10.1002/ana.27177","DOIUrl":"10.1002/ana.27177","url":null,"abstract":"<p>The long-term clinical outcomes and associated prognostic factors in contactin-associated protein-like 2 (CASPR2)-antibody diseases are unknown. A total of 75 participants with CASPR2 antibodies were longitudinally assessed for disability, quality-of-life, and chronic pain. Although most symptoms improved within 6 months of treatment, neuropathic pain and fatigue were the most immunotherapy refractory, and persisted for up to 6 years. Furthermore, these two factors—but not CASPR2 antibody levels or subclasses—independently predicted worse disability and quality-of-life at 24 months. Quality-of-life varied widely for any given modified Rankin Scale score, indicating a divergence between patient and clinician assessed outcomes. Further work should study the relative importance of these measures, and the immunopathogenesis underlying intractable symptoms. ANN NEUROL 2025;97:521–528</p>","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":"97 3","pages":"521-528"},"PeriodicalIF":8.1,"publicationDate":"2025-01-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/ana.27177","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142996761","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Subventricular Zone Microstructure in Pediatric-Onset Multiple Sclerosis","authors":"Monica Margoni MD, PhD,&nbsp;Loredana Storelli MSc, PhD,&nbsp;Elisabetta Pagani MSc,&nbsp;Paolo Preziosa MD, PhD,&nbsp;Damiano Mistri MSc,&nbsp;Mor Gueye MD,&nbsp;Martina Rubin MD,&nbsp;Lucia Moiola MD, PhD,&nbsp;Massimo Filippi MD,&nbsp;Maria Assunta Rocca MD","doi":"10.1002/ana.27180","DOIUrl":"10.1002/ana.27180","url":null,"abstract":"<div>\u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>The aim of this study was to explore the microstructural dynamics of the subventricular zone (SVZ) with aging and their associations with clinical disability and brain structural damage in pediatric-onset multiple sclerosis (MS) patients.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>One-hundred and forty-one pediatric-onset MS patients (67 pediatric and 74 adults with pediatric-onset) and 233 healthy controls (HC) underwent neurological and 3.0 T MRI assessment. Fractional anisotropy (FA) and mean diffusivity (MD) were extracted from the SVZ and the thalamus (as control region).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>In HC, SVZ FA was higher until age 40 then declined, whereas MD was lower until age 35 before rising (false discovery rate <i>p</i> value [pFDR] ≤ 0.008). Thalamic FA was higher until age 30 and then declined, whereas MD was higher until age 50 (pFDR ≤ 0.007). Pediatric MS patients showed significantly higher SVZ FA than pediatric HC (pFDR &lt; 0.001), while adult patients showed no differences compared to adult HC (pFDR ≤ 0.724). Adult patients had lower thalamic FA and higher MD (pFDR &lt; 0.001). Adults had lower SVZ FA and MD, but higher thalamic MD compared to pediatric patients (pFDR &lt; 0.001). In pediatric MS, higher SVZ FA and MD were associated with higher white matter (WM) lesion volume (LV) and choroid plexus volume and lower brain and thalamic volumes (pFDR ≤ 0.047). In adult patients, higher SVZ MD associated with higher WM LV, lower brain volumes, and lower z-SDMT (pFDR≤0.019). Thalamic microstructural abnormalities were associated with more severe disability and brain damage in both groups (pFDR ≤ 0.018).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>Our findings suggest that microstructural changes in the SVZ occur early in pediatric MS and are associated with brain structural damage but not with clinical impairment. ANN NEUROL 2025;97:979–992</p>\u0000 </section>\u0000 </div>","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":"97 5","pages":"979-992"},"PeriodicalIF":8.1,"publicationDate":"2025-01-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/ana.27180","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142996769","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical Relevance of ‘Cap’ and ‘Track’ Development after Recent Small Subcortical Infarct","authors":"Yajun Cheng PhD,&nbsp;Carmen Arteaga-Reyes MD,&nbsp;Una Clancy PhD,&nbsp;Daniela Jaime Garcia MSc,&nbsp;Maria Del C. Valdés Hernández PhD,&nbsp;Michael J. Thrippleton PhD,&nbsp;Michael S. Stringer PhD,&nbsp;Gordon W. Blair MRCP,&nbsp;Stewart Wiseman PhD,&nbsp;Francesca M. Chappell PhD,&nbsp;Junfang Zhang PhD,&nbsp;Xiaodi Liu PhD,&nbsp;Angela C.C. Jochems PhD,&nbsp;Susana Muñoz Maniega PhD,&nbsp;Eleni Sakka MSc,&nbsp;Mark E. Bastin DPhil,&nbsp;Rosalind Brown PhD,&nbsp;Caroline M.J. Loos PhD,&nbsp;Stephen D.J. Makin PhD,&nbsp;Ming Liu PhD,&nbsp;Bo Wu PhD,&nbsp;Fergus N. Doubal PhD,&nbsp;Joanna M. Wardlaw MD, FRCR, FMedSci,&nbsp;MSS-2, MSS-3 and the INVESTIGATE-SVDs Study Group","doi":"10.1002/ana.27182","DOIUrl":"10.1002/ana.27182","url":null,"abstract":"<div>\u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>After a recent small subcortical infarct (RSSI), some patients develop perilesional or remote hyperintensities (‘caps/tracks’) to the index infarct on T2/FLAIR MRI. However, their clinical relevance remains unclear. We investigated the clinicoradiological correlates of ‘caps/tracks’, and their impact on long-term outcomes following RSSI.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We identified participants with lacunar stroke and MRI-confirmed RSSI from 3 prospective studies. At baseline, we collected risk factors, RSSI characteristics, small vessel disease (SVD) features, and microstructural integrity on diffusion imaging. Over 1-year, we repeated MRI and recorded ‘caps/tracks’ blinded to other data. We evaluated predictors of ‘caps/tracks’, and their association with 1-year functional (modified Rankin Scale score ≥2), mobility (Timed Up-and-Go), cognitive outcomes (Montreal Cognitive Assessment [MoCA] score &lt;26), and recurrent cerebrovascular events (stroke/transient ischemic attack/incident infarct) using multivariable regression.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Among 185 participants, 93 (50.3%) developed ‘caps/tracks’ first detected at median 198 days after stroke. ‘Caps/tracks’ were independently predicted by baseline factors: larger RSSI, RSSI located in white matter, higher SVD score, and higher mean diffusivity in normal-appearing white matter (odds ratio [OR] [95% confidence interval {CI}], 1.15 [1.07–1.25], 6.01 [2.80–13.57], 1.77 [1.31–2.44], 1.42 [1.01–2.03]). At 1 year, ‘cap/track’ formation was associated with worse functional outcome (OR: 3.17, 95% CI: 1.28–8.22), slower gait speed (β: 0.13, 95% CI: 0.01–0.25), and recurrent cerebrovascular events (hazard ratio [HR]: 2.05, 95% CI: 1.05–4.02), but not with cognitive impairment.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>‘Caps/tracks’ after RSSI are associated with worse clinical outcomes, and may reflect vulnerability to progressive SVD-related injury. Reducing ‘caps/tracks’ may offer early efficacy markers in trials aiming to improve outcome after lacunar stroke. ANN NEUROL 2025;97:942–955</p>\u0000 </section>\u0000 </div>","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":"97 5","pages":"942-955"},"PeriodicalIF":8.1,"publicationDate":"2025-01-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/ana.27182","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142996755","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Annals of Neurology: Volume 97, Number 2, February 2025","authors":"","doi":"10.1002/ana.26975","DOIUrl":"https://doi.org/10.1002/ana.26975","url":null,"abstract":"","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":"97 2","pages":"C1"},"PeriodicalIF":8.1,"publicationDate":"2025-01-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/ana.26975","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143116111","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Diagnosing Epilepsy with Normal Interictal EEG Using Dynamic Network Models","authors":"Patrick Myers,&nbsp;Kristin M. Gunnarsdottir PhD,&nbsp;Adam Li PhD,&nbsp;Vlad Razskazovskiy MD,&nbsp;Jeff Craley PhD,&nbsp;Alana Chandler,&nbsp;Dale Wyeth,&nbsp;Edmund Wyeth,&nbsp;Kareem A. Zaghloul MD, PhD,&nbsp;Sara K. Inati MD,&nbsp;Jennifer L. Hopp MD,&nbsp;Babitha Haridas MBBS,&nbsp;Jorge Gonzalez-Martinez MD, PhD,&nbsp;Anto Bagíc MD, PhD,&nbsp;Joon-yi Kang MD,&nbsp;Michael R. Sperling MD,&nbsp;Niravkumar Barot MD,&nbsp;Sridevi V. Sarma PhD,&nbsp;Khalil S. Husari MD","doi":"10.1002/ana.27168","DOIUrl":"10.1002/ana.27168","url":null,"abstract":"<div>\u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>Whereas a scalp electroencephalogram (EEG) is important for diagnosing epilepsy, a single routine EEG is limited in its diagnostic value. Only a small percentage of routine EEGs show interictal epileptiform discharges (IEDs) and overall misdiagnosis rates of epilepsy are 20% to 30%. We aim to demonstrate how network properties in EEG recordings can be used to improve the speed and accuracy differentiating epilepsy from mimics, such as functional seizures – even in the absence of IEDs.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>In this multicenter study, we analyzed routine scalp EEGs from 218 patients with suspected epilepsy and normal initial EEGs. The patients’ diagnoses were later confirmed based on an epilepsy monitoring unit (EMU) admission. About 46% ultimately being diagnosed with epilepsy and 54% with non-epileptic conditions. A logistic regression model was trained using spectral and network-derived EEG features to differentiate between epilepsy and non-epilepsy. Of the 218 patients, 90% were used for training and 10% were held out for testing. Within the training set, 10-fold cross validation was performed. The resulting tool was named “EpiScalp.”</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>EpiScalp achieved an area under the curve (AUC) of 0.940, an accuracy of 0.904, a sensitivity of 0.835, and a specificity of 0.963 in classifying patients as having epilepsy or not.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>EpiScalp provides an accurate diagnostic aid from a single initial EEG recording, even in more challenging epilepsy cases with normal initial EEGs. This may represent a paradigm shift in epilepsy diagnosis by deriving an objective measure of epilepsy likelihood from previously uninformative EEGs. ANN NEUROL 2025;97:907–918</p>\u0000 </section>\u0000 </div>","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":"97 5","pages":"907-918"},"PeriodicalIF":8.1,"publicationDate":"2025-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/ana.27168","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142996758","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"ANA Podcasts and Webinars: Minimally Invasive Epilepsy Surgery","authors":"Ioannis Karakis MD, PhD, MSc,&nbsp;Adeline L. Goss MD,&nbsp;Jessica W. Templer MD,&nbsp;Michelle C. Johansen MD, PhD,&nbsp;Jon T. Willie MD, PhD, FAANS","doi":"10.1002/ana.27183","DOIUrl":"https://doi.org/10.1002/ana.27183","url":null,"abstract":"","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":"97 3","pages":"407-408"},"PeriodicalIF":8.1,"publicationDate":"2025-01-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143423540","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Genetic Ablation of Sarm1 Mitigates Disease Acceleration after Traumatic Brain Injury in the SOD1G93A Transgenic Mouse Model of Amyotrophic Lateral Sclerosis","authors":"Elif O. Dogan MD,&nbsp;Sean R. Simonini,&nbsp;James Bouley BA,&nbsp;Alexandra Weiss BS,&nbsp;Robert H. Brown Jr DPhil, MD,&nbsp;Nils Henninger MD, PhD, Dr Med","doi":"10.1002/ana.27174","DOIUrl":"10.1002/ana.27174","url":null,"abstract":"<div>\u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>Approximately 20% of familial cases of amyotrophic lateral sclerosis (ALS) are caused by mutations in the gene encoding superoxide dismutase 1 (SOD1). Epidemiological data have identified traumatic brain injury (TBI) as an exogenous risk factor for ALS; however, the mechanisms by which TBI may worsen SOD1 ALS remain largely undefined.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We sought to determine whether repetitive TBI (rTBI) accelerates disease onset and progression in the transgenic SOD1^G93A mouse ALS model, and whether loss of the primary regulator of axonal degeneration sterile alpha and TIR motif containing 1 (<i>Sarm1</i>) mitigates the histological and behavioral pathophysiology. We subjected wild-type (n = 23), <i>Sarm1</i> knockout (KO; n = 17), SOD1^G93A (n = 19), and SOD1^G93AxSarm1^KO (n = 26) mice of both sexes to rTBI or sham surgery at age 64 days (62–68 days). Body weight and ALS-deficit score were serially assessed up to 17 weeks after surgery and histopathology assessed in layer V of the primary motor cortex at the study end point.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>In sham injured SOD1^G93A mice, genetic ablation of <i>Sarm1</i> did not attenuate axonal loss, improve neurological deficits, or survival. The rTBI accelerated onset of G93A-SOD1 ALS, as indicated by accentuated body weight loss, earlier onset of hindlimb tremor, and shortened survival. The rTBI also triggered TDP-43 mislocalization, enhanced axonal and neuronal loss, microgliosis, and astrocytosis. Loss of <i>Sarm1</i> significantly diminished the impact of rTBI on disease progression and rescued rTBI-associated neuropathology.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>SARM1-mediated axonal death pathway promotes pathogenesis after TBI in SOD1^G93A mice suggesting that anti-SARM1 therapeutics are a viable approach to preserve neurological function in injury-accelerated G93A-SOD1 ALS. ANN NEUROL 2025;97:963–975</p>\u0000 </section>\u0000 </div>","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":"97 5","pages":"963-975"},"PeriodicalIF":8.1,"publicationDate":"2025-01-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142941771","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}