Journal of Clinical Neurology最新文献

{"title":"Rethinking Temporopolar White-Matter Hyperintensity in CADASIL: A Context-Dependent Biomarker.","authors":"Tae-Jin Song, Seong-Ho Koh","doi":"10.3988/jcn.2026.0215","DOIUrl":"10.3988/jcn.2026.0215","url":null,"abstract":"","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":"22 4","pages":"387-388"},"PeriodicalIF":3.6,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13364521/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148412135","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":"Neural Correlates of Intellectual Dysfunction in Episodic Ataxia Type 2.","authors":"Hyo-Jung Kim, Eek-Sung Lee, Seoyeon Kim, Seung-Han Lee, Jae-Myung Kim, Seunghee Na, Jeong-Yoon Choi, Jae-Hwan Choi, Ji-Soo Kim","doi":"10.3988/jcn.2025.0760","DOIUrl":"10.3988/jcn.2025.0760","url":null,"abstract":"<p><strong>Background and purpose: </strong>Episodic ataxia type 2 (EA2) is characterized by episodes of vertigo and ataxia due to mutations in <i>CACNA1A</i> that encodes the α1A subunit of the P/Q-type voltage-gated calcium channel. This study aimed to identify neural correlates of cognitive dysfunction in EA2 by investigating brain atrophy in these patients and determining the relationships between regional brain volumes and intellectual dysfunction.</p><p><strong>Methods: </strong>We recruited 12 patients with EA2 (including 6 males; age=30.5±14.3 years, mean±standard deviation) in 3 university hospitals of South Korea from 2019 to 2023. Regional brain volumes were quantified using voxel-based morphometry and the brainstem-structures feature of FreeSurfer. The results were compared with those for healthy controls. The relationships between regional gray-matter volumes (GMVs) and cognitive function were assessed using voxel-wise multiple regression analyses within the general linear model framework.</p><p><strong>Results: </strong>Brain volumetry revealed a significant decrease in cerebellar volumes, particularly in the vermis (lobules IV, V, and VIII), bilateral flocculi (lobule X), and brainstem. The Full-Scale Intelligence Quotient was positively correlated with the GMVs in the left parahippocampal gyrus, right caudate nucleus, and right cerebellar crus II; the Verbal Comprehension Index was correlated with the GMVs in the bilateral cerebellar crura I and II; and the Processing Speed Index was correlated with the GMV in the right parahippocampal gyrus.</p><p><strong>Conclusions: </strong>Volumetric analyses revealed brain atrophy in patients with EA2 that was correlated with the clinical features observed in this disorder. These findings may further expand the imaging spectrum of disorders associated with <i>CACNA1A</i> mutations, although the identified correlations need to be interpreted with caution.</p>","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":"22 4","pages":"467-476"},"PeriodicalIF":3.6,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13364530/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148412073","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":"Recurrent Embolic Stroke From Aortic Intimal Sarcoma Mimicking Atherosclerotic Disease.","authors":"Yun Jeong Hong, Do Yeon Kim, Hyeryung Kwon, Taewon Kim","doi":"10.3988/jcn.2026.0011","DOIUrl":"10.3988/jcn.2026.0011","url":null,"abstract":"","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":"22 4","pages":"483-485"},"PeriodicalIF":3.6,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13364518/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148412058","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":"Expansion of the Clinical and Genetic Spectra of hnRNPA1-Associated Distal Myopathy in Two Korean Families.","authors":"Kilim Lee, Yun Jung Choi, Young-Chul Choi, Hyung Jun Park","doi":"10.3988/jcn.2025.0716","DOIUrl":"10.3988/jcn.2025.0716","url":null,"abstract":"","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":"22 4","pages":"492-494"},"PeriodicalIF":3.6,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13364522/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148412138","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":"Altered Brain Functional Connectivity in Postherpetic Neuralgia and Its Association With Neuropathic Pain.","authors":"Daeseok Oh, Dong Ah Lee, Ho-Joon Lee, Kang Min Park","doi":"10.3988/jcn.2026.0005","DOIUrl":"10.3988/jcn.2026.0005","url":null,"abstract":"<p><strong>Background and purpose: </strong>Postherpetic neuralgia (PHN) is a chronic neuropathic pain condition that can occur after herpes zoster, but the relationship between the brain network organization and the clinical symptoms of PHN remains unclear.</p><p><strong>Methods: </strong>Resting-state functional magnetic resonance imaging data were acquired from 42 patients with PHN and 41 sex-matched healthy controls. Graph-theoretical analyses were used to assess global network measures including the characteristic path length, mean clustering coefficient, eccentricity, global efficiency, and local efficiency across 132 brain regions, while regional properties were evaluated using the betweenness centrality. Group comparisons were performed between healthy controls and PHN patients stratified by Douleur Neuropathique 4 (DN4) scores. Correlations with clinical variables were examined, with the results corrected for multiple comparisons using the family-wise error rate.</p><p><strong>Results: </strong>At the global network level, the mean clustering coefficient, eccentricity, and local efficiency were lower in patients with PHN than in healthy controls after correction for multiple comparisons (all adjusted <i>p</i><0.05). Regionally, the betweenness centrality in the anterior division of the cingulate gyrus (ACC) was significantly lower in patients with PHN than in healthy controls, with this difference remaining significant after Bonferroni correction (adjusted <i>p</i>=0.013). In the PHN patients, local efficiency was higher in the high-DN4-score group than in the low-DN4-score group, and DN4 scores were positively correlated with local efficiency.</p><p><strong>Conclusions: </strong>PHN was associated with reductions in local clustering and efficiency as well as hubness of the ACC, indicating alterations to the brain network organization. Conversely, in the PHN patients, higher DN4 scores were associated with higher local efficiency. These findings suggest that symptom-informed connectomic profiling can capture clinically relevant heterogeneity that provides insights into network reorganization linked to the neuropathic symptom burden.</p>","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":"22 4","pages":"456-466"},"PeriodicalIF":3.6,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13364526/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148412008","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":"Statin Therapy and Myasthenic Crisis: Is It a Transient Signal or a Clinically Meaningful Risk?","authors":"Jin-Sung Park, Ji-Ye Jeon","doi":"10.3988/jcn.2026.0276","DOIUrl":"10.3988/jcn.2026.0276","url":null,"abstract":"","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":"22 4","pages":"389-391"},"PeriodicalIF":3.6,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13364519/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148412079","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":"Efficacy and Safety of Nipocalimab in Adults With Generalised Myasthenia Gravis: An Asian Subgroup Analysis of the Vivacity-MG3 Study.","authors":"Yuriko Nagane, Huan Yang, Jin-Sung Park, Jiann-Horng Yeh, Ibrahim Turkoz, Yogeshwar Makanji","doi":"10.3988/jcn.2025.0290","DOIUrl":"10.3988/jcn.2025.0290","url":null,"abstract":"<p><strong>Background and purpose: </strong>The phase 3, randomised, double-blind, placebo-controlled Vivacity-MG3 trial demonstrated that nipocalimab, a neonatal Fc receptor blocker, provided sustained disease control over 6 months when added to standard of care therapies in patients with antibody-positive generalised myasthenia gravis (gMG). Here we present the Vivacity-MG3 Asian subgroup analysis.</p><p><strong>Methods: </strong>Recruited patients were randomised (1:1) to receive either nipocalimab (30 mg/kg loading dose, then 15 mg/kg every 2 weeks for maintenance) or placebo infusions every 2 weeks, both alongside standard of care therapy, for 24 weeks. The primary endpoint was the difference between nipocalimab and placebo groups in least-squares mean change from baseline in Myasthenia Gravis-Activities of Daily Living (MG-ADL) total score over weeks 22-24 in seropositive patients. Other endpoints included changes in Quantitative Myasthenia Gravis (QMG) score and MG-ADL response rates. Adverse events were evaluated in all treated patients.</p><p><strong>Results: </strong>This analysis included 56 patients from East Asia (nipocalimab <i>n</i>=28; placebo <i>n</i>=28). The least-squares mean difference in change in MG-ADL score from baseline to weeks 22-24 was -1.35 (95% confidence interval [CI] -2.93 to 0.24) in favour of nipocalimab in the Asian population, consistent with the overall population (-1.45 [95% CI -2.38 to -0.52]). Numerical improvements in QMG score and MG-ADL response rates were observed with nipocalimab in the Asian population, generally aligning with the overall study findings. Treatment-emergent adverse event incidence was comparable between treatment arms.</p><p><strong>Conclusions: </strong>Nipocalimab demonstrated a favourable efficacy and safety in the East Asian subgroup, with outcomes generally consistent with the overall Vivacity-MG3 study, supporting its role as a treatment option for gMG in this population.</p>","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":"22 4","pages":"446-455"},"PeriodicalIF":3.6,"publicationDate":"2026-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13364529/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148412051","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":"Clinical Significance of Plasma Sphingosine-1-Phosphate Decline in Progression of Spinal and Bulbar Muscular Atrophy.","authors":"Young-Heun Jung, Hung Youl Seok, Minsung Kang, Young Woo Do, Junyoung O Park, Ju-Hyun Kim, Jin-Sung Park","doi":"10.3988/jcn.2025.0556","DOIUrl":"10.3988/jcn.2025.0556","url":null,"abstract":"<p><strong>Background and purpose: </strong>Spinal and bulbar muscular atrophy (SBMA) is a rare X-linked neuromuscular disorder characterized by slowly progressive motor decline. However, there is no specific fluid marker that reflects the severity or progression of the disease. Therefore, identifying measurable markers that reflect disease progression remains a major unmet need in SBMA management.</p><p><strong>Methods: </strong>Plasma samples from 21 Korean patients with SBMA were collected at baseline and after 3 years. Untargeted and targeted metabolomics profiling was performed, and plasma sphingosine-1-phosphate (S1P) was the final candidate biomarker. S1P was then quantified using liquid chromatography-mass spectrometry. Associations between longitudinal S1P changes and clinical parameters, including the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R), serum creatine kinase (CK), 6-minute walk test (6MWT), and forced vital capacity (FVC), were examined.</p><p><strong>Results: </strong>Plasma S1P levels significantly declined in the fast progression group over 3 years, while remaining relatively stable in the slow progression group. Longitudinal changes in plasma S1P showed weak-to-moderate positive trends with clinical measurements, including ALSFRS-R, FVC, and 6MWT, and a negative trend with serum CK at follow-up.</p><p><strong>Conclusions: </strong>Plasma S1P represents a promising quantitative biomarker for monitoring SBMA progression. Validation in larger cohorts and mechanistic studies of S1P regulation are warranted to establish its role in clinical management.</p>","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":"22 3","pages":"327-337"},"PeriodicalIF":3.6,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13161591/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147868498","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":"Positional Elliptical Pendular Nystagmus in Inferior Olivary Pseudohypertrophy.","authors":"Chenghao Li, Hyunji Kim, Jeong-Yoon Choi, Ji-Soo Kim","doi":"10.3988/jcn.2025.0731","DOIUrl":"10.3988/jcn.2025.0731","url":null,"abstract":"","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":"22 3","pages":"381-383"},"PeriodicalIF":3.6,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13161543/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147868537","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":"Bilateral \"Three-Quarters\" Gaze Palsy Syndrome Caused by Unilateral Thalamomesencephalic Infarction.","authors":"Takuro Shiga, Hajime Ikenouchi, Kaoru Endo","doi":"10.3988/jcn.2025.0641","DOIUrl":"10.3988/jcn.2025.0641","url":null,"abstract":"","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":"22 3","pages":"372-374"},"PeriodicalIF":3.6,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13161538/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147868538","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}