Journal of Clinical Neurology最新文献

{"title":"Early Suspected Progressive Supranuclear Palsy-Cerebellar Type From MRI and Dopaminergic Imaging in a Patient Initially Presenting With Ataxia.","authors":"Jihwan You, Geunhyung Gye, Jaehyun Yoon, Kyum-Yil Kwon","doi":"10.3988/jcn.2025.0584","DOIUrl":"10.3988/jcn.2025.0584","url":null,"abstract":"","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":"22 3","pages":"375-377"},"PeriodicalIF":3.6,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13161570/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147868485","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Modified Ultrafast Dynamic CT Myelography: Method, Outcomes, and Comparison With Noninvasive Imaging.","authors":"Hoe Jong Jeong, So Youn Choi, Hye Jin Yoo, Ja-Young Choi, Mi Ji Lee","doi":"10.3988/jcn.2025.0636","DOIUrl":"10.3988/jcn.2025.0636","url":null,"abstract":"<p><strong>Background and purpose: </strong>Spontaneous intracranial hypotension (SIH) is primarily caused by cerebrospinal fluid (CSF) leakage; however, precise localization of the leakage remains challenging. This study evaluated the outcomes of ultrafast CT myelography in Korea.</p><p><strong>Methods: </strong>Data were prospectively collected from patients with SIH who underwent ultrafast CT myelography at Seoul National University Hospital between August 2023 and December 2024. Success rates and leakage sites were documented. We also examined whether the most prominent lateral extradural fluid collection observed on heavily T2-weighted magnetic resonance (MR) myelography could predict the actual leak site identified by ultrafast CT myelography.</p><p><strong>Results: </strong>Fifty-one patients (22 males and 29 females) underwent ultrafast CT myelography, with a mean age of 42.9±10.3 years (range, 25-70 years). Technical success rate was 90.2% (46/51). Leakage sites were identified in 44 patients (95.7%) of the 46 with successful examinations. Among these 44 patients, 4 (9.1%) demonstrated spinal cord herniation at the dural defect site. The leak site was cervical in 2 patients (5.0%) and thoracic in 38 patients (95.0%): 28 at the upper, 8 at the mid, and 2 at the lower thoracic levels. Among the 40 patients with visible leakage, only 5 (12.5%) had exact concordance between the leakage site and the most prominent lateral extradural fluid collection seen on pre-procedural MR myelography.</p><p><strong>Conclusions: </strong>The upper thoracic level was the most common site of CSF leakage, which differed from assumptions based on noninvasive spine MR myelography or non-contrast spine CT.</p>","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":"22 3","pages":"355-363"},"PeriodicalIF":3.6,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13161547/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147868521","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Diagnostic Value of Flow-Void Sign for Localizing Ventral Dural Defects in Spine MRI.","authors":"Soo-Im Jang, Soyoun Choi, Seung Ae Kim, Hoe Jong Jeong, Ja-Young Choi, Mi Ji Lee","doi":"10.3988/jcn.2025.0618","DOIUrl":"10.3988/jcn.2025.0618","url":null,"abstract":"<p><strong>Background and purpose: </strong>Ultrafast dynamic CT myelography and digital subtraction myelography are used to localize dural defects in spontaneous intracranial hypotension; however, their invasiveness and technical demands limit widespread availability. The \"flow-void sign\" on T2-weighted spine MRI has been proposed as a potential non-invasive marker of the ventral dural defect, but its clinical value remains unclear. This study aimed to evaluate the utility of the flow-void sign for predicting dural defect sites in ventral cerebrospinal fluid leaks.</p><p><strong>Methods: </strong>This retrospective cohort study, using data from the headache registry at Seoul National University Hospital (March 2022-April 2025), included patients with ventral dural defects confirmed by ultrafast dynamic CT myelography or surgery. Three neurologists assessed the flow-void sign on T2-weighted spine magnetic resonance sequences and evaluated the spinal CT images for causative structural lesions. The concordance between the flow-void sign and ventral dural defect was analyzed using kappa statistics. Logistic regression analysis was performed to identify factors associated with this concordance.</p><p><strong>Results: </strong>Among 64 patients (57.8% female; median age, 40.0 years), the flow-void sign was observed in 43 (67.2%) patients. In the flow-void-positive group, 32 (74.4%) patients demonstrated concordance with the ventral dural defect level (κ=0.660, 95% confidence interval [CI], 0.476-0.825). The presence of a causative spinal lesion was significantly associated with the concordance (odds ratio, 6.48; 95% CI, 1.41-29.71; <i>p</i>=0.016).</p><p><strong>Conclusions: </strong>The flow-void sign may provide an indirect clue for localizing a ventral dural defect and supplement the definitive imaging modalities.</p>","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":"22 3","pages":"364-371"},"PeriodicalIF":3.6,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13161546/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147868495","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"MOG-IgG Positivity Does Not Equal MOGAD: Diagnostic Pitfall and Misapplication of the International MOGAD Panel Proposed Criteria in Real-World Practice.","authors":"You-Ri Kang, Ki Hoon Kim, Jae-Won Hyun, Ho Jin Kim, Su-Hyun Kim","doi":"10.3988/jcn.2025.0529","DOIUrl":"10.3988/jcn.2025.0529","url":null,"abstract":"<p><strong>Background and purpose: </strong>Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) has been increasingly recognized, yet concerns remain regarding the overuse and misinterpretation of MOG-IgG testing. The recently published MOGAD diagnostic criteria emphasize selective testing and rigorous interpretation in the context of compatible clinical syndromes and supportive features. We investigated, in real-world practice, the extent to which these criteria are appropriately applied and the frequency and characteristics of their misapplication at a referral center.</p><p><strong>Methods: </strong>We retrospectively reviewed patients referred to the National Cancer Center with externally reported MOG-IgG positivity between January 2021 and December 2024. External assay results were based on laboratory-specific cutoffs, which had not been verified against clinically validated thresholds. Final diagnoses were determined by expert consensus after comprehensive evaluation, applying the 2023 international MOGAD diagnostic criteria.</p><p><strong>Results: </strong>Fifty-seven patients with external MOG-IgG positivity were referred, of whom 48 (84.2%) had been labeled as MOGAD. Upon re-evaluation, only 39 patients (68.4%) met the diagnostic criteria. Of the 18 non-confirmed patients, 5 (27.8%) manifested nonspecific symptoms incompatible with core demyelinating events, 9 patients (50.0%) received an alternative diagnosis, and 4 (22.2%) presented core events but lacked supportive features.</p><p><strong>Conclusions: </strong>Over one-third of patients with externally reported positive results did not meet diagnostic criteria, illustrating the risk of non-targeted MOG-IgG testing and overinterpretation of antibody results. Selective test utilization and strict adherence to diagnostic criteria in the context of appropriate clinical syndromes and supportive findings are essential to prevent overdiagnosis and inappropriate treatment.</p>","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":"22 3","pages":"338-342"},"PeriodicalIF":3.6,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13161573/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147868529","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Impact of Schizophrenia on Inpatient Outcomes for Acute Ischemic Stroke: Evidence From the 2005-2020 United States Nationwide Inpatient Sample.","authors":"Yuan-Chang Hsu, Chih-Tsai Chen, Kuo-Ping Li, Pesus Chou","doi":"10.3988/jcn.2025.0232","DOIUrl":"10.3988/jcn.2025.0232","url":null,"abstract":"<p><strong>Background and purpose: </strong>This study investigated the impact of schizophrenia on the outcomes of hospitalizations for acute ischemic stroke (AIS). Previous studies have suggested that outcomes are worse for patients with both AIS and schizophrenia, but a definitive conclusion is lacking.</p><p><strong>Methods: </strong>A retrospective analysis of the 2005-2020 United States Nationwide Inpatient Sample was conducted. Patients aged ≥20 years who were hospitalized for AIS were divided into two groups: with and without schizophrenia. The groups were matched using propensity-score matching at a 1:4 ratio. The assessed outcomes included in-hospital mortality, unfavorable discharge, length of hospital stay (LOS), and major complications. Logistic and linear regression analyses were used to assess outcome relationships.</p><p><strong>Results: </strong>Matching resulted in 43,545 patients being analyzed: 8,709 with schizophrenia and 34,836 without schizophrenia. Schizophrenia was associated with higher risks of mortality (odds ratio [OR]=1.16), unfavorable discharge (OR=2.00), prolonged LOS (OR=1.61), and major complications (OR=1.39) including acute myocardial infarction, venous thromboembolism, pneumonia, sepsis, and acute kidney injury. Schizophrenia was also associated with higher total hospital costs (beta=6.73). The associations were consistent across most subgroups.</p><p><strong>Conclusions: </strong>Schizophrenia significantly worsens the outcomes in AIS patients, contributing to higher mortality, prolonged LOS, more complications, and increased hospital costs. These findings highlight the need to apply tailored management strategies to AIS patients with schizophrenia.</p>","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":"22 3","pages":"319-326"},"PeriodicalIF":3.6,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13161592/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147868449","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Astrocyte Functional Heterogeneity in Multiple Sclerosis.","authors":"Sehoon Hong, Kristen Gah-Hyeon Kim, Francisco J Quintana, Hong-Gyun Lee","doi":"10.3988/jcn.2026.0100","DOIUrl":"10.3988/jcn.2026.0100","url":null,"abstract":"<p><p>Multiple sclerosis (MS) is a chronic neuroinflammatory disease characterized by demyelination and axonal degeneration. Astrocytes have emerged as pivotal regulators of MS pathology, playing both pathogenic and regulatory roles. This review explores the mechanisms underlying astrocyte heterogeneity in MS, with a focus on how astrocyte functional states are influenced by extrinsic environmental factors such as inflammatory signals and dynamic interactions with immune cells and other glial populations. We discuss how the astrocyte heterogeneity established during development and further modulated by local environmental factors influences lesion formation, blood-brain barrier integrity, and tissue repair. We further outline protective and pathogenic astrocyte states together with the molecular mechanisms that govern neuroinflammatory responses. Finally, we highlight emerging therapeutic strategies for modulating astrocyte pathogenic responses. Collectively this work highlights astrocyte functional heterogeneity as a central feature of MS pathology and an emerging therapeutic opportunity for progressive MS.</p>","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":"22 3","pages":"271-282"},"PeriodicalIF":3.6,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13161585/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147868500","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Misleading Anti-Yo Positivity on Immunoblot Alone: Importance of Rat-Brain Immunohistochemistry.","authors":"Jee Won Hong, Chae Min Kang, Su Yee Mon, Soon-Tae Lee","doi":"10.3988/jcn.2025.0695","DOIUrl":"10.3988/jcn.2025.0695","url":null,"abstract":"","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":"22 3","pages":"384-386"},"PeriodicalIF":3.6,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13161540/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147868477","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Sphingosine-1-Phosphate as an Immune-Metabolic Biomarker in Neurodegenerative Diseases.","authors":"Seung Hyun Kim, Hee Kyung Jin, Jae-Sung Bae","doi":"10.3988/jcn.2026.0158","DOIUrl":"10.3988/jcn.2026.0158","url":null,"abstract":"","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":"22 3","pages":"264-267"},"PeriodicalIF":3.6,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13161544/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147868632","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Deep Learning Approach for Classifying Rapid Eye Movement Sleep Behavior Disorder Using EEGNet.","authors":"Yun Ho Choi, Sunil Kim, Jaeseung Jeong, Seonghoon Kim, Dong-Woo Ryu, Woojun Kim, Yu Jin Jung","doi":"10.3988/jcn.2025.0552","DOIUrl":"10.3988/jcn.2025.0552","url":null,"abstract":"<p><strong>Background and purpose: </strong>Rapid eye movement (REM) sleep behavior disorder (RBD) is a prodromal marker for α-synucleinopathies, and its early and accurate diagnosis provides invaluable insights into the development of neurodegenerative diseases. This study aimed to propose a deep-learning-based automated framework for RBD classification and to evaluate its performance using real-world, multi-cohort polysomnography (PSG) data.</p><p><strong>Methods: </strong>REM electromyogram (EMG) data of 227 PSG recordings collected across five tertiary hospitals were analyzed. The dataset was divided into RBD (<i>n</i>=156) and non-RBD (<i>n</i>=71) and further stratified into Parkinson's disease (PD) with RBD (<i>n</i>=60), PD without RBD (<i>n</i>=24), isolated RBD (<i>n</i>=96), and healthy controls (<i>n</i>=47). An automated framework for RBD classification, based on manual and automated sleep staging, was implemented using EEGNet.</p><p><strong>Results: </strong>The EEGNet-based classifier achieved a receiver operating characteristics-area under the curve of 0.812 (95% confidence interval [CI], 0.744-0.871) using manual staging, which was significantly higher than the 0.777 (95% CI, 0.704-0.843) achieved with the automated U-Sleep approach (<i>p</i>=0.039). However, relying solely on REM EMG data proved insufficient to accurately distinguish among the four clinical subgroups.</p><p><strong>Conclusions: </strong>Our EEGNet-based RBD classifier, focused solely on EMG data, demonstrated reasonable performance in detecting RBD. This proposed model has the potential to reduce clinicians' workload and support diagnostic decision-making and enabling more accurate RBD classification. This multi-center study highlights the feasibility of an automated and generalizable RBD detection framework, paving the way for broader clinical implementation.</p>","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":"22 3","pages":"303-312"},"PeriodicalIF":3.6,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13161577/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147868464","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Acute Small Fiber Neuropathy With Severe Autonomic Dysfunction at the Interface of Guillain-Barré Syndrome.","authors":"Dong Won Kwack, Dong Wook Kim, Jeeyoung Oh","doi":"10.3988/jcn.2025.0575","DOIUrl":"10.3988/jcn.2025.0575","url":null,"abstract":"","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":"22 3","pages":"378-380"},"PeriodicalIF":3.6,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13161537/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147868479","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}