Annals of Neurology最新文献

{"title":"Correction to “Mutations in CLCN6 as a Novel Genetic Cause of Neuronal Ceroid Lipofuscinosis in Patients and a Murine Model”","authors":"","doi":"10.1002/ana.27076","DOIUrl":"10.1002/ana.27076","url":null,"abstract":"<p>\u0000 <span>He, H</span>, <span>Cao, X</span>, <span>He, F</span>, et al. <span>Mutations in <i>CLCN6</i> as a novel genetic cause of neuronal ceroid lipofuscinosis in patients and a murine model</span>. <i>Ann Neurol</i> <span>2024</span>; <span>96</span>: <span>608</span>–<span>624</span>. https://doi.org/10.1002/ana.27002\u0000 </p><p>In the above article, the title was incorrectly published as “Mutations in <i>CLCN6</i> as a Novel Genetic Cause of Neuronal Ceroid Lipofuscinosis in a Murine Model.”</p><p>The correct title is “Mutations in <i>CLCN6</i> as a Novel Genetic Cause of Neuronal Ceroid Lipofuscinosis in Patients and a Murine Model.” The online version of this article has been corrected accordingly.</p><p>We apologize for this error.</p>","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":"96 4","pages":"822"},"PeriodicalIF":8.1,"publicationDate":"2024-08-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/ana.27076","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142102523","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":"Single-Nucleus RNA Sequencing Unravels Early Mechanisms of Human Becker Muscular Dystrophy","authors":"Zhihao Xie PhD,&nbsp;Chang Liu MD,&nbsp;Chengyue Sun MD, PhD,&nbsp;Yilin Liu MD, PhD,&nbsp;Jieru Peng MD,&nbsp;Lingchao Meng MD,&nbsp;Jianwen Deng PhD,&nbsp;Zhaoxia Wang MD, PhD,&nbsp;Chunxia Yang PhD,&nbsp;Yun Yuan MD, PhD,&nbsp;Zhiying Xie MD, PhD","doi":"10.1002/ana.27070","DOIUrl":"10.1002/ana.27070","url":null,"abstract":"<div>\u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>The transcriptional heterogeneity at a single-nucleus level in human Becker muscular dystrophy (BMD) dystrophic muscle has not been explored. Here, we aimed to understand the transcriptional heterogeneity associated with myonuclei, as well as other mononucleated cell types that underly BMD pathogenesis by performing single-nucleus RNA sequencing.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We profiled single-nucleus transcriptional profiles of skeletal muscle samples from 7 BMD patients and 3 normal controls.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>A total of 17,216 nuclei (12,879 from BMD patients and 4,337 from controls) were classified into 13 known cell types, including 9 myogenic lineages and 4 non-myogenic lineages, and 1 unclassified nuclear type according to their cell identities. Among them, type IIx myonuclei were the first to degenerate in response to dystrophin reduction. Differential expression analysis revealed that the fibro-adipogenic progenitors (FAPs) population had the largest transcriptional changes among all cell types. Sub-clustering analysis identified a significantly compositional increase in the activated FAPs (aFAPs) subpopulation in BMD muscles. Pseudotime analysis, regulon inference, and deconvolution analysis of bulk RNA-sequencing data derived from 29 BMD patients revealed that the aFAPs subpopulation, a distinctive and previously unrecognized mononuclear subtype, was profibrogenic and expanded in BMD patients. Muscle quantitative real-time polymerase chain reaction and immunofluorescence analysis confirmed that the mRNA and protein levels of the aFAPs markers including <i>LUM</i>, <i>DCN</i>, and <i>COL1A1</i> in BMD patients were significantly higher than those in controls, respectively.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>Our results provide insights into the transcriptional diversity of human BMD muscle at a single-nucleus resolution and new potential targets for anti-fibrosis therapies in BMD. ANN NEUROL 2024;96:1070–1085</p>\u0000 </section>\u0000 </div>","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":"96 6","pages":"1070-1085"},"PeriodicalIF":8.1,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142078526","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":"Reply to “Letter to the Editor: Assessing the Efficacy and Limitations of Rescue Thrombectomy in Acute Ischemic Stroke”","authors":"Aaron Rodriguez-Calienes MD,&nbsp;Fazeel M. Siddiqui MD,&nbsp;Milagros Galecio-Castillo MD,&nbsp;Mahmoud H. Mohammaden MD,&nbsp;Jaydevsinh N. Dolia MD,&nbsp;Jonathan A. Grossberg MD,&nbsp;Aqueel Pabaney MD,&nbsp;Ameer E. Hassan MD,&nbsp;Wondwossen G. Tekle MD,&nbsp;Hamzah Saei MD,&nbsp;Samantha Miller MD,&nbsp;Shahram Majidi MD,&nbsp;Johanna T. Fifi MD,&nbsp;Gabrielle Valestin MD,&nbsp;James E. Siegler MD,&nbsp;Mary Penckofer MD,&nbsp;Linda Zhang MD,&nbsp;Sunil A. Sheth MD,&nbsp;Sergio Salazar-Marioni MD,&nbsp;Ananya Iyyangar MD,&nbsp;Thanh N. Nguyen MD,&nbsp;Mohamad Abdalkader MD,&nbsp;Italo Linfante MD,&nbsp;Guilherme Dabus MD,&nbsp;Brijesh P. Mehta MD,&nbsp;Joy Sessa MD,&nbsp;Mouhammad A. Jumma MD,&nbsp;Rebecca M. Sugg MD,&nbsp;Guillermo Linares MD,&nbsp;Raul G. Nogueira MD,&nbsp;David S. Liebeskind MD,&nbsp;Diogo C. Haussen MD,&nbsp;Santiago Ortega-Gutierrez MD, MSc","doi":"10.1002/ana.27064","DOIUrl":"10.1002/ana.27064","url":null,"abstract":"","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":"96 4","pages":"820-821"},"PeriodicalIF":8.1,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142078597","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":"Mapping Functional Connectivity Signatures of Pharmacoresistant Focal Cortical Dysplasia-Related Epilepsy.","authors":"Hua Xie, Venkata Sita Priyanka Illapani, L Gilbert Vezina, Taha Gholipour, Chima Oluigbo, William D Gaillard, Nathan T Cohen","doi":"10.1002/ana.27069","DOIUrl":"https://doi.org/10.1002/ana.27069","url":null,"abstract":"<p><strong>Objective: </strong>To determine common network alterations in focal cortical dysplasia pharmacoresistant epilepsy (FCD-PRE) using functional connectivity analysis of resting-state functional magnetic resonance imaging (rsfMRI).</p><p><strong>Methods: </strong>This is a retrospective imaging cohort from Children's National Hospital (Washington, DC, USA) from January, 2011 to January, 2022. Patients with 3-T MRI-confirmed FCD-PRE underwent rsfMRI as part of routine clinical care. Patients were included if they were age 5-22 years at the time of the scan, and had a minimum of 18 months of follow-up. Healthy, typically-developing controls were included from Children's National Hospital (n = 16) and matched from Human Connectome Project-Development public dataset (n = 100).</p><p><strong>Results: </strong>A total of 42 FCD-PRE patients (20 M:22 F, aged 14.2 ± 4.1 years) and 116 healthy controls (56 M:60 F, aged 13.7 ± 3.3 years) with rsfMRI were included. Seed-based functional connectivity maps were generated for each FCD, and each seed was used to generate a patient-specific z-scored connectivity map on 116 controls. FCD-PRE patients had mutual altered connectivity in regions of dorsal attention, default mode, and control networks. Functional connectivity was diminished within the FCD dominant functional network, as well as in homotopic regions. Cluster specific connectivity patterns varied by pathological subtype. Higher FCD connectivity to the limbic network was associated with increased odds of Engel I outcome.</p><p><strong>Interpretation: </strong>This study demonstrates diminished functional connectivity patterns in FCD-PRE, which may represent a neuromarker for the disease, independent of FCD location, involving the dorsal attention, default mode, and control functional networks. Higher connectivity to the limbic network is associated with a seizure-free outcome. Future multicenter, prospective studies are needed to allow for much earlier detection of signatures of treatment-resistant epilepsy. ANN NEUROL 2024.</p>","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":" ","pages":""},"PeriodicalIF":8.1,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142078596","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":"Miyazaki Syndrome as a Complication of Shunt Drainage","authors":"Rick H. G. J. van Lanen MD, MSc,&nbsp;Jasper van Aalst MD, PhD,&nbsp;Mariël P. Ter Laak-Poort MD, PhD","doi":"10.1002/ana.27067","DOIUrl":"10.1002/ana.27067","url":null,"abstract":"&lt;p&gt;A 41-year-old female was referred to the neurosurgery outpatient clinic with progressive bipyramidal syndrome. Medical history included premature birth and prior intraventricular hemorrhage with hydrocephalus. Ventricular-peritoneal (VP) shunting was performed in the management of hydrocephalus. However, complications related to over-drainage, along with hyperostosis of the skull and craniosynostosis, caused scaphocephaly.&lt;/p&gt;&lt;p&gt;Over the course of several months, she experienced progressive difficulties walking and developed right-sided weakness. Neurological examination revealed right-sided hemiparesis, hyperreflexia, and bilateral Babinski's sign. Imaging of the brain and cervical spine (Fig 1) showed scaphocephaly with slit-like ventricles and myelopathy extending from the C1 to C3 levels, along with the presence of spinal epidural venous engorgement. In the absence of a diagnosis, she was placed under long-term follow-up care by her neurologist. Follow-up magnetic resonance imaging (MRI) of the cervical spine revealed progressive myelopathy, before the diagnosis of Miyazaki syndrome was made. She underwent VP-shunt revision, with implantation of a Miethke proGAV 2.0 0-20/20 (programmable valve pressure setting 8). Outpatient clinic follow-up at 6 months showed stabilization of the bipyramidal symptoms. Follow-up MRI showed improvement of the epidural venous engorgement.&lt;/p&gt;&lt;p&gt;Miyazaki syndrome arises as a complication of cerebrospinal fluid (CSF) hypotension caused by excessive drainage through VP-shunting. Although CSF hypotension is often associated with well-known symptoms, like orthostatic headache and cranial nerve palsies, the development of epidural venous engorgement leading to spinal cord compression is a less common but critical manifestation.&lt;/p&gt;&lt;p&gt;The syndrome is characterized by cervical myelopathy or radiculopathy due to cervical epidural venous congestion, results from complex pathophysiological mechanisms.&lt;span&gt;&lt;sup&gt;1, 2&lt;/sup&gt;&lt;/span&gt; These include changes in CSF pressure, consistent with the Monro-Kellie doctrine, and dysfunction in the Starling resistor, leading to the enlargement and dilation of the spinal epidural venous plexus.&lt;span&gt;&lt;sup&gt;1&lt;/sup&gt;&lt;/span&gt; Venous congestion can lead to spinal cord or nerve roots compression or circulation hampering,&lt;span&gt;&lt;sup&gt;1&lt;/sup&gt;&lt;/span&gt; causing neurological symptoms, whereas presenting a unique diagnostic challenge. One of the striking features of Miyazaki syndrome is that it can manifest without the typical symptom of orthostatic headache, which is commonly associated with CSF hypotension. Instead, patients with Miyazaki syndrome may develop myelopathy symptoms slowly over time, making it challenging to diagnose, potentially leading to misdiagnosis.&lt;span&gt;&lt;sup&gt;3&lt;/sup&gt;&lt;/span&gt; This underlines the importance of considering Miyazaki syndrome in the differential diagnosis of patients with VP-shunts who present with myelopathy but do not experience headaches. Timely recognition is crucial and","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":"96 6","pages":"1230-1231"},"PeriodicalIF":8.1,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/ana.27067","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142071516","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":"Population-Based Evidence for the Use of Serum Neurofilaments as Individual Diagnostic and Prognostic Biomarkers in Amyotrophic Lateral Sclerosis","authors":"Simon Witzel MD,&nbsp;André Huss PhD,&nbsp;Gabriele Nagel MD,&nbsp;Angela Rosenbohm MD,&nbsp;Dietrich Rothenbacher MD,&nbsp;Raphael S. Peter PhD,&nbsp;Hansjörg Bäzner MD,&nbsp;Axel Börtlein MD,&nbsp;Silke Dempewolf MD,&nbsp;Martin Schabet MD,&nbsp;Martin Hecht MD,&nbsp;Andreas Kohler MD,&nbsp;Christian Opherk MD,&nbsp;Andrea Naegele MD,&nbsp;Norbert Sommer MD,&nbsp;Alfred Lindner MD,&nbsp;Christoforos Alexudis,&nbsp;Franziska Bachhuber PhD,&nbsp;Steffen Halbgebauer PhD,&nbsp;David Brenner MD,&nbsp;Wolfgang Ruf MD,&nbsp;Ulrike Weiland MD,&nbsp;Benjamin Mayer PhD,&nbsp;Joachim Schuster PhD,&nbsp;Johannes Dorst MD,&nbsp;Hayrettin Tumani MD,&nbsp;Albert C. Ludolph MD,&nbsp;and the ALS Registry Swabia Study Group","doi":"10.1002/ana.27054","DOIUrl":"10.1002/ana.27054","url":null,"abstract":"<div>\u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>Neurofilament light chains (NfL) and phosphorylated neurofilament heavy chains (pNfH), established as diagnostic and prognostic biomarkers in hospital-based amyotrophic lateral sclerosis (ALS) cohorts, are now surrogate markers in clinical trials. This study extends their evaluation to a population level, with the aim of advancing their full establishment and assessing the transferability of biomarker findings from controlled cohorts to real-world ALS populations.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We measured serum NfL and pNfH levels in all ALS patients (n = 790) and general population controls (n = 570) with available baseline samples participating in the epidemiological ALS Registry Swabia, providing platform-specific (ELLA™) reference data and Z-scores for controls, as well as reference data, disease-specific Z-scores and longitudinal data in ALS. We evaluated the diagnostic and prognostic utility of neurofilaments and quantified the impact of ALS-related factors and non-ALS confounders.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Neurofilaments showed high diagnostic and prognostic utility at the population level, with NfL superior to pNfH. The novel concept of a population-based ALS Z-score significantly improved the prognostic utility compared to absolute raw values. Both biomarkers increased more strongly with age in controls than in ALS, and age adjustment improved diagnostic accuracy. Our data show that disease progression rates, ALS phenotype, body mass index (BMI), and renal function need to be considered when interpreting neurofilament levels; longitudinal neurofilament levels were generally stable in individual patients, especially when adjusted for age and baseline levels.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>Population-based assessment enhances the utility of particularly serum NfL as a diagnostic and prognostic biomarker in ALS and improves the translation of findings from controlled cohorts to real-world populations. ANN NEUROL 2024;96:1040–1057</p>\u0000 </section>\u0000 </div>","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":"96 6","pages":"1040-1057"},"PeriodicalIF":8.1,"publicationDate":"2024-08-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/ana.27054","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142034629","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":"Electro-Clinical Features and Functional Connectivity Analysis in SYN1-Related Epilepsy.","authors":"Vincent Moya Quiros, Ahmed Adham, Philippe Convers, Gaetan Lesca, François Mauguiere, Hugo Soulier, Alexis Arzimanoglou, Allan Bayat, Hilde Braakman, Jean-Philippe Camdessanche, Philippe Casenave, Laurence Chaton, Yves Chaix, Maxime Chochoi, Christel Depienne, Vincent Desportes, Jessie De Ridder, Vera Dinkelacker, Elena Gardella, Gerhard J Kluger, Julien Jung, Martine Lemesle Martin, Maria Margherita Mancardi, Markus Mueller, Anne-Lise Poulat, Konrad Platzer, Agathe Roubertie, Marijn F Stokman, Anneke T Vulto-van Silfhout, Gert Wiegand, Laure Mazzola","doi":"10.1002/ana.27063","DOIUrl":"https://doi.org/10.1002/ana.27063","url":null,"abstract":"<p><strong>Objective: </strong>There is currently scarce data on the electroclinical characteristics of epilepsy associated with synapsin 1 (SYN1) pathogenic variations. We examined clinical and electro-encephalographic (EEG) features in patients with epilepsy and SYN1 variants, with the aim of identifying a distinctive electroclinical pattern.</p><p><strong>Methods: </strong>In this retrospective multicenter study, we collected and reviewed demographic, genetic, and epilepsy data of 19 male patients with SYN1 variants. Specifically, we analyzed interictal EEG data for all patients, and electro-clinical data from 10 epileptic seizures in 5 patients, using prolonged video-EEG monitoring recordings. Inter-ictal EEG functional connectivity parameters and frequency spectrum of the 10 patients over 12 years of age, were computed and compared with those of 56 age- and sex-matched controls.</p><p><strong>Results: </strong>The main electroclinical features of epilepsy in patients with SYN1 were (1) EEG background and organization mainly normal; (2) interictal abnormalities are often rare or not visible on EEG; (3) more than 60% of patients had reflex seizures (cutaneous contact with water and defecation being the main triggers) isolated or associated with spontaneous seizures; (4) electro-clinical semiology of seizures was mainly temporal or temporo-insulo/perisylvian with a notable autonomic component; and (5) ictal EEG showed a characteristic rhythmic theta/delta activity predominating in temporo-perisylvian regions at the beginning of most seizures. Comparing patients with SYN1 to healthy subjects, we observed a shift to lower frequency bands in power spectrum of interictal EEG and an increased connectivity in both temporal regions.</p><p><strong>Interpretation: </strong>A distinct epilepsy syndrome emerges in patients with SYN1, with a rather characteristic clinical and EEG pattern suggesting predominant temporo-insular involvement. ANN NEUROL 2024.</p>","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":" ","pages":""},"PeriodicalIF":8.1,"publicationDate":"2024-08-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142034628","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":"Syrinx beyond Cord: Syringocephalia Extending to Corona Radiata","authors":"Aditi Saini MD,&nbsp;Sarbesh Tiwari DM,&nbsp;Jaskaran Singh Gosal Mch,&nbsp;Mayank Garg Mch","doi":"10.1002/ana.27056","DOIUrl":"10.1002/ana.27056","url":null,"abstract":"","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":"96 6","pages":"1225-1226"},"PeriodicalIF":8.1,"publicationDate":"2024-08-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142015712","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":"Higher Validity, Lower Radiation: A New Ictal Single-Photon Emission Computed Tomography Framework","authors":"Felix Bitzer BSc,&nbsp;Lennart Walger MSc,&nbsp;Tobias Bauer MD, BSc,&nbsp;Freya Schulte,&nbsp;Florian C. Gaertner MD,&nbsp;Matthias Schmitz,&nbsp;Martin Schidlowski PhD,&nbsp;Randi von Wrede MD,&nbsp;Attila Rácz MD,&nbsp;Tobias Baumgartner MD,&nbsp;Vadym Gnatkovsky MD,&nbsp;Daniel Paech MD,&nbsp;Valeri Borger MD,&nbsp;Hartmut Vatter MD,&nbsp;Bernd Weber MD,&nbsp;Dominik L. Michels PhD,&nbsp;Tony Stöcker PhD,&nbsp;Markus Essler MD,&nbsp;Josemir W. Sander MD, PhD,&nbsp;Alexander Radbruch MD, JD,&nbsp;Rainer Surges MD, MHBA,&nbsp;Theodor Rüber MD","doi":"10.1002/ana.27061","DOIUrl":"10.1002/ana.27061","url":null,"abstract":"<div>\u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>To assess whether arterial spin labeling perfusion images of healthy controls can enhance ictal single-photon emission computed tomography analysis and whether the acquisition of the interictal image can be omitted.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We developed 2 pipelines: The first uses ictal and interictal images and compares these to single-photon emission computed tomography and arterial spin labeling of healthy controls. The second pipeline uses only the ictal image and the analogous healthy controls. Both pipelines were compared to the gold standard analysis and evaluated on data of individuals with epilepsy who underwent ictal single-photon emission computed tomography imaging during presurgical evaluation between 2010 and 2022. Fifty healthy controls prospectively underwent arterial spin labeling imaging. The correspondence between the detected hyperperfusion and the postoperative resection cavity or the presumably affected lobe was assessed using Dice score and mean Euclidean distance. Additionally, the outcomes of the pipelines were automatically assigned to 1 of 5 concordance categories.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Inclusion criteria were met by 43 individuals who underwent epilepsy surgery and by 73 non-surgical individuals with epilepsy. Compared to the gold standard analysis, both pipelines resulted in significantly higher Dice scores and lower mean distances (<i>p</i> &lt; 0.05). The combination of both provided localizing results in 85/116 cases, compared to 54/116 generated by the current gold standard analysis and the ictal image alone produced localizing results in 60/116 (52%) cases.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>We propose a new ictal single-photon emission computed tomography protocol; it finds relevantly more ictal hyperperfusion, and halves the radiation dose in about half of the individuals. ANN NEUROL 2024;96:1160–1173</p>\u0000 </section>\u0000 </div>","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":"96 6","pages":"1160-1173"},"PeriodicalIF":8.1,"publicationDate":"2024-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/ana.27061","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142015711","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":"Assessing the Efficacy and Limitations of Rescue Thrombectomy in Acute Ischemic Stroke","authors":"Qian Xu MMed,&nbsp;Shicai Huang CS","doi":"10.1002/ana.27065","DOIUrl":"10.1002/ana.27065","url":null,"abstract":"","PeriodicalId":127,"journal":{"name":"Annals of Neurology","volume":"96 4","pages":"819"},"PeriodicalIF":8.1,"publicationDate":"2024-08-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142003168","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}