{"title":"Janus Kinase Inhibitor: A Possible Cause of Hyperammonemic Encephalopathy?","authors":"Jong-Ho Park, Do-Hyung Kim","doi":"10.3988/jcn.2025.0039","DOIUrl":"https://doi.org/10.3988/jcn.2025.0039","url":null,"abstract":"","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":"21 3","pages":"247-249"},"PeriodicalIF":2.9,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12056130/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143982272","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}
Dong Young Jeong, Bum Joon Kim, Jae Han Bae, Chulhong Kim, Sun U Kwon
{"title":"Impact of <i>RNF213</i> Polymorphism in Isolated Intracranial Arterial Steno-Occlusive Disease.","authors":"Dong Young Jeong, Bum Joon Kim, Jae Han Bae, Chulhong Kim, Sun U Kwon","doi":"10.3988/jcn.2024.0495","DOIUrl":"https://doi.org/10.3988/jcn.2024.0495","url":null,"abstract":"<p><strong>Background and purpose: </strong>To determine whether the <i>RNF213</i> p.R4810K mutation modifies the number of moyamoya disease manifestations and recurrent strokes in isolated intracranial arterial steno-occlusive disease (ICAD).</p><p><strong>Methods: </strong>This retrospective case-control study analyzed patients who visited the Asan Medical Center with steno-occlusive lesions in the M1 segment of the middle cerebral artery and terminal internal carotid artery, and underwent <i>RNF213</i> genetic testing for screening moyamoya disease between January 2010 and November 2022. Patients with supportive findings of moyamoya disease or moderate-to-severe stenosis in the extracranial arteries were excluded. After matching antiplatelet drugs, the presentation of moyamoya disease and stroke recurrence were analyzed using chi-squared analysis and Kaplan-Meier survival curve analysis.</p><p><strong>Results: </strong>The 1,567 patients who underwent evaluations of <i>RNF213</i> polymorphisms included 753 with ICAD, among whom females predominated (<i>n</i>=452, 60.0%) and 289 (38.4%) had an <i>RNF213</i> mutation. The follow-up period was 2.47±3.51 years (mean±standard deviation; median=1.00 year, interquartile range=0-4 years). The risk of progression to moyamoya disease was higher in the <i>RNF213</i>-related-vasculopathy group than the <i>RNF213</i>-negative stenosis group (<i>n</i>=27 [9.3%] versus <i>n</i>=6 [1.3%], p<0.01), as were the risks of ischemic stroke (<i>n</i>=13 [4.5%] versus <i>n</i>=7 [1.5%], <i>p</i>=0.01) and hemorrhagic stroke (<i>n</i>=5 [1.7%] versus <i>n</i>=1 [0.2%], <i>p</i>=0.02, respectively). Furthermore, the presence of an <i>RNF213</i> mutation was significantly associated with the risk of stroke recurrence (odds ratio=2.34, 95% confidence interval=1.44-3.80, <i>p</i><0.01).</p><p><strong>Conclusions: </strong>Evaluations of <i>RNF213</i> polymorphisms may help to identify patients with isolated ICAD at a high risk of progression to moyamoya disease and stroke.</p>","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":"21 3","pages":"173-181"},"PeriodicalIF":2.9,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12056131/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143968895","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":"Challenges in Translating Recent RCTs on Endovascular Therapy for Large Core Infarctions Into Real-World Practice.","authors":"Doo Hyuk Kwon, Jun Lee","doi":"10.3988/jcn.2025.0108","DOIUrl":"https://doi.org/10.3988/jcn.2025.0108","url":null,"abstract":"","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":"21 3","pages":"230-231"},"PeriodicalIF":2.9,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12056139/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144007650","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}
Sung Hoon Kang, Jee Hyang Jeong, Jung-Min Pyun, Geon Ha Kim, Young Ho Park, YongSoo Shim, Seong-Ho Koh, Chi-Hun Kim, Young Chul Youn, Dong Won Yang, Hyuk-Je Lee, Han Lee, Dain Kim, Kyunghwa Sun, So Young Moon, Kee Hyung Park, Seong Hye Choi
{"title":"Eligibility for Lecanemab Treatment in the Republic of Korea: Real-World Data From Memory Clinics.","authors":"Sung Hoon Kang, Jee Hyang Jeong, Jung-Min Pyun, Geon Ha Kim, Young Ho Park, YongSoo Shim, Seong-Ho Koh, Chi-Hun Kim, Young Chul Youn, Dong Won Yang, Hyuk-Je Lee, Han Lee, Dain Kim, Kyunghwa Sun, So Young Moon, Kee Hyung Park, Seong Hye Choi","doi":"10.3988/jcn.2024.0550","DOIUrl":"https://doi.org/10.3988/jcn.2024.0550","url":null,"abstract":"<p><strong>Background and purpose: </strong>We aimed to determine the proportion of Korean patients with early Alzheimer's disease (AD) who are eligible to receive lecanemab based on the United States Appropriate Use Recommendations (US AUR), and also identify the barriers to this treatment.</p><p><strong>Methods: </strong>We retrospectively enrolled 6,132 patients with amnestic mild cognitive impairment or mild amnestic dementia at 13 hospitals from June 2023 to May 2024. Among them, 2,058 patients underwent amyloid positron emission tomography (PET) and 1,199 (58.3%) of these patients were amyloid-positive on PET. We excluded 732 patients who did not undergo brain magnetic resonance imaging between June 2023 and May 2024. Finally, 467 patients were included in the present study.</p><p><strong>Results: </strong>When applying the criteria of the US AUR, approximately 50% of patients with early AD were eligible to receive lecanemab treatment. Among the 467 included patients, 36.8% did not meet the inclusion criterion of a Mini-Mental State Examination (MMSE) score of ≥22.</p><p><strong>Conclusions: </strong>Eligibility for lecanemab treatment was not restricted to Korean patients with early AD except for those with an MMSE score of ≥22. The MMSE criteria should therefore be reconsidered in areas with a higher proportion of older people, who tend to have lower levels of education.</p>","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":"21 3","pages":"182-189"},"PeriodicalIF":2.9,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12056135/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143985381","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":"Usefulness of Nystagmus Patterns in Distinguishing Peripheral From Central Acute Vestibular Syndromes at the Bedside: A Critical Review.","authors":"Sun-Uk Lee, Alexander A Tarnutzer","doi":"10.3988/jcn.2025.0105","DOIUrl":"https://doi.org/10.3988/jcn.2025.0105","url":null,"abstract":"<p><p>Vertigo and dizziness are amongst the most frequent presenting symptoms in the emergency room, accounting for up to 4% of all emergency consultations. The broadness of their differential diagnosis and the often transient nature of these symptoms pose a significant challenge to the treating physician. Combining various subtle oculomotor signs at the bedside has been very successful in distinguishing peripheral from central causes in acutely dizzy patients meeting diagnostic criteria for the acute vestibular syndrome (i.e., acute and prolonged vertigo or dizziness accompanied by nausea or vomiting, gait imbalance, motion intolerance, and [not mandatory] nystagmus). While the diagnostic accuracy of the HINTS (Head-Impulse-Nystagmus-Test-of-Skew) algorithm has been studied extensively, less is known about the value of various nystagmus patterns seen at the bedside in patients with an acute vestibular syndrome. Here we review both spontaneous and triggered presenting nystagmus patterns and discuss their impacts and limitations, including primary-gaze horizontal, vertical, and torsional nystagmus, nystagmus during eccentric gaze, and nystagmus triggered by stimuli such as head-shaking, hyperventilation, positional testing, vibration, and the Valsalva maneuver. We conclude that the usefulness of nystagmus patterns in discriminating peripheral and central causes strongly depends on the pattern seen and the type of testing performed, being highly predictive of a central cause for torsional and vertical spontaneous nystagmus, downbeat, or apogeotropic horizontal and treatment-refractory positional nystagmus. The predictive value for central causes was moderate only for vertical nystagmus after horizontal head-shaking (\"perverted\" head-shaking nystagmus) since it can also occur in peripheral cases, while the predictive value was low for vibration-induced nystagmus and Valsalva-induced nystagmus.</p>","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":"21 3","pages":"161-172"},"PeriodicalIF":2.9,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12056143/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143970846","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":"Alterations of the Temporal Lobe Network in De Novo Psychosis After Epilepsy Surgery.","authors":"Dong Ah Lee, Byung In Lee, Kang Min Park","doi":"10.3988/jcn.2024.0569","DOIUrl":"https://doi.org/10.3988/jcn.2024.0569","url":null,"abstract":"","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":"21 3","pages":"232-234"},"PeriodicalIF":2.9,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12056134/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144064128","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}