Journal of Clinical Neurology最新文献

{"title":"Pembrolizumab-Associated Unilateral Optic Neuritis.","authors":"Jihae Park, Jeong-Min Hwang, Hee Kyung Yang","doi":"10.3988/jcn.2023.0201","DOIUrl":"10.3988/jcn.2023.0201","url":null,"abstract":"","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":null,"pages":null},"PeriodicalIF":3.1,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10921044/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139424868","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":"Smooth Pursuit and Reflexive Saccade in Discriminating Multiple-System Atrophy With Predominant Parkinsonism From Parkinson's Disease.","authors":"Yaqin Yu, Jinyu Wang, Lihong Si, Huanxin Sun, Xiaolei Liu, Xinyi Li, Weihong Yan","doi":"10.3988/jcn.2022.0413","DOIUrl":"10.3988/jcn.2022.0413","url":null,"abstract":"<p><strong>Background and purpose: </strong>Performing the differential diagnosis of Parkinson's disease (PD) and multiple-system atrophy of parkinsonian type (MSA-P) is challenging. The oculomotor performances of patients with PD and MSA-P were investigated to explore their potential role as a biomarker for this differentiation.</p><p><strong>Methods: </strong>Reflexive saccades and smooth pursuit were examined in 56 patients with PD and 34 with MSA-P in the off-medication state.</p><p><strong>Results: </strong>Patients with PD and MSA-P had similar oculomotor abnormalities of prolonged and hypometric reflexive saccades. The incidence rates of decreased reflexive saccadic velocity and saccadic smooth pursuit were significantly higher in MSA-P than in PD (<i>p</i><0.05 for both). Multiple logistic regression analysis indicated that slowed reflexive saccades (odds ratio [OR]=8.14, 95% confidence interval [CI]=1.45-45.5) and saccadic smooth pursuit (OR=5.27, 95% CI=1.24-22.43) were significantly related to MSA-P.</p><p><strong>Conclusions: </strong>The distinctive oculomotor abnormalities of saccadic smooth pursuit and slowed reflexive saccades in MSA-P may serve as useful biomarkers for discriminating MSA-P from PD.</p>","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":null,"pages":null},"PeriodicalIF":3.1,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10921038/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139087073","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":"Predicting the Future Fall Risk Using Challenging Tasks: Importance of Sensor-Based Quantitative Measurements of Gait in Parkinson's Disease.","authors":"Do-Young Kwon","doi":"10.3988/jcn.2024.0051","DOIUrl":"10.3988/jcn.2024.0051","url":null,"abstract":"","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":null,"pages":null},"PeriodicalIF":3.1,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10921056/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140021785","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":"Association Between Baseline Gait Parameters and Future Fall Risk in Patients With De Novo Parkinson's Disease: Forward Versus Backward Gait.","authors":"Kyum-Yil Kwon, Jihwan You, Rae On Kim, Eun Ji Lee, Jungyeun Lee, Ilsoo Kim, Jinhee Kim, Seong-Beom Koh","doi":"10.3988/jcn.2022.0299","DOIUrl":"10.3988/jcn.2022.0299","url":null,"abstract":"<p><strong>Background and purpose: </strong>Falls are not uncommon even in patients with early stages of Parkinson's disease (PD). The aims of this study were to determine the relationships between gait parameters and falls and identify crucial gait parameters for predicting future falls in patients with de novo PD.</p><p><strong>Methods: </strong>We prospectively recruited patients with de novo PD, and evaluated their baseline demographics, global cognitive function on the Montreal Cognitive Assessment test, and parkinsonian motor symptoms including their subtypes. Both forward gait (FG) and backward gait (BG) were measured using the GAITRite system. The history of falls in consecutive patients with de novo PD was examined along with 1 year of follow-up data.</p><p><strong>Results: </strong>Among the 76 patients with de novo PD finally included in the study, 16 (21.1%) were classified as fallers. Fallers had slower gait and shorter stride for FG and BG parameters than did non-fallers, while stride-time variability was greater in fallers but only for BG. Multivariable logistic regression analysis revealed that slow gait was an independent risk factor in BG.</p><p><strong>Conclusions: </strong>Among the patients with de novo PD, gait speed and stride length were more impaired for both FG and BG in fallers than in non-fallers. It was particularly notable that slow BG was significantly associated with future fall risk, indicating that BG speed is a potential biomarker for predicting future falls in patients with early-stage PD.</p>","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":null,"pages":null},"PeriodicalIF":3.1,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10921052/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139087068","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":"Chikungunya Encephalitis Presenting as Rhombencephalitis.","authors":"Doyeon Kook, Sangwon Joe, Jung Hyun Lee, Han Soo Yoo","doi":"10.3988/jcn.2023.0151","DOIUrl":"10.3988/jcn.2023.0151","url":null,"abstract":"","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":null,"pages":null},"PeriodicalIF":3.1,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10782084/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139097943","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":"Therapeutic Outcomes and Electrophysiological Biomarkers in Anti-Myelin-Associated Glycoprotein Neuropathy: A Multicenter Cohort Study in South Korea.","authors":"Young Gi Min, Hee-Jo Han, Ha Young Shin, Jong-Gyu Baek, Jun-Soon Kim, Kyung-Seok Park, Seol-Hee Baek, Ilhan Yoo, So-Young Huh, Young Nam Kwon, Seok-Jin Choi, Sung-Min Kim, Yoon-Ho Hong, Jung-Joon Sung","doi":"10.3988/jcn.2023.0127","DOIUrl":"10.3988/jcn.2023.0127","url":null,"abstract":"<p><strong>Background and purpose: </strong>Unlike other immune-mediated neuropathies, anti-myelin-associated glycoprotein (MAG) neuropathy is often refractory to immunotherapy. It is necessary to compare the relative efficacies of various immunotherapies and develop objective biomarkers in order to optimize its clinical management.</p><p><strong>Methods: </strong>This study recruited 91 patients with high anti-MAG antibody titers from 7 tertiary hospitals in South Korea. We analyzed the baseline characteristics, therapeutic outcomes, and nerve conduction study (NCS) findings of 68 patients and excluded 23 false positive cases.</p><p><strong>Results: </strong>The rate of positive responses to treatment was highest using zanubrutinib (50%) and rituximab (36.4%), followed by corticosteroids (16.7%), immunosuppressants (9.5%), intravenous immunoglobulin (5%), and plasma exchange (0%). Disability and weakness were significantly associated with multiple NCS parameters at the time of diagnosis, especially distal compound muscle action potential (CMAP) amplitudes. Moreover, the longitudinal trajectory of the average CMAP amplitudes paralleled the clinical courses, with a 16.2 percentile decrease as an optimal cutoff for predicting a clinical exacerbation (area under the receiver operating characteristic curve=0.792).</p><p><strong>Conclusions: </strong>Our study supports the use of NCS as an objective marker for estimating disease burden and tracking clinical changes in patients with anti-MAG neuropathy. We have described the beneficial effects of rituximab and a new drug, zanubrutinib, compared with conventional immunotherapies.</p>","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":null,"pages":null},"PeriodicalIF":3.1,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10782088/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139097954","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":"Clinicopathological Reclassification of Idiopathic Inflammatory Myopathy to Match the Serological Results of Myositis-Specific Antibodies.","authors":"Young-Eun Park, Dae-Seong Kim, Minsung Kang, Jin-Hong Shin","doi":"10.3988/jcn.2022.0432","DOIUrl":"10.3988/jcn.2022.0432","url":null,"abstract":"<p><strong>Background and purpose: </strong>Advances in serological tests are transforming the classification of idiopathic inflammatory myopathy (IIM). The new criteria suggested by the 119th European Neuromuscular Center international workshop divide IIM cases into four main diseases according to clinical and pathological findings, adding immune-mediated necrotizing myositis and nonspecific myositis to the classic categories of polymyositis and dermatomyositis.</p><p><strong>Methods: </strong>Seventy one cases of IIM with sufficient available clinical and pathological data were reviewed to be reclassified according to the new criteria.</p><p><strong>Results: </strong>Most of the cases previously classified as polymyositis (77.8%, 35/45) were reclassified as immune-mediated necrotizing myopathy. The results of myositis-specific antibodies matched well with the new clinicopathological classification.</p><p><strong>Conclusions: </strong>This new clinicopathological classification for IIM in combination with serological test results could be applied to our previous case series. Adoption of the new criteria will lead to a better understanding of the disease and hence new therapeutic insights.</p>","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":null,"pages":null},"PeriodicalIF":3.1,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10782087/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139097944","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":"Episodic Neurological Dysfunction in X-Linked Charcot-Marie-Tooth Disease: Expansion of the Phenotypic and Genetic Spectrum.","authors":"Feixia Zhan, Wotu Tian, Yuwen Cao, Jingying Wu, Ruilong Ni, Taotao Liu, Yun Yuan, Xinghua Luan, Li Cao","doi":"10.3988/jcn.2023.0104","DOIUrl":"10.3988/jcn.2023.0104","url":null,"abstract":"<p><strong>Background and purpose: </strong>X-linked Charcot-Marie-Tooth disease type 1 (CMTX1) is characterized by peripheral neuropathy with or without episodic neurological dysfunction. We performed clinical, neuropathological, and genetic investigations of a series of patients with mutations of the gap-junction beta-1 gene (<i>GJB1</i>) to extend the phenotypic and genetic description of CMTX1.</p><p><strong>Methods: </strong>Detailed clinical evaluations, sural nerve biopsy, and genetic analysis were applied to patients with CMTX1.</p><p><strong>Results: </strong>We collected 27 patients with CMTX1 with <i>GJB1</i> mutations from 14 unrelated families. The age at onset (AAO) was 20.9±12.2 years (mean±standard deviation; range, 2-45 years). Walking difficulties, weakness in the legs, and pes cavus were common initial symptoms. Compared with female patients, males tended to have a younger AAO (males vs. females=15.4±9.6 vs. 32.0±8.8 years, <i>p</i>=0.002), a longer disease course (16.8±16.1 vs. 5.5±3.8 years, <i>p</i>=0.034), and more-severe electrophysiological results. Besides peripheral neuropathy, six of the patients had special episodic central nervous system (CNS) evidence from symptoms, signs, and/or reversible white-matter lesions. Neuropathology revealed the loss of large myelinated fibers, increased number of regenerated axon clusters with abnormally thin myelin sheaths, and excessively folded myelin. Genetic analysis identified 14 <i>GJB1</i> variants, 6 of which were novel.</p><p><strong>Conclusions: </strong>These findings expand the phenotypic and genetic spectrum of CMTX1. Although CMTX1 was found to have high phenotypic and CNS involvement variabilities, detailed neurological examinations and nerve conduction studies will provide critical clues for accurate diagnoses. Further exploration of the underlying mechanisms of connexin 32 involvement in neuropathy or CNS dysfunction is warranted to develop promising therapies.</p>","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":null,"pages":null},"PeriodicalIF":3.1,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10782082/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139097947","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":"Outcome and Sequelae of Autoimmune Encephalitis.","authors":"Kathryn A Kvam, Jean-Paul Stahl, Felicia C Chow, Ariane Soldatos, Pierre Tattevin, James Sejvar, Alexandra Mailles","doi":"10.3988/jcn.2023.0242","DOIUrl":"10.3988/jcn.2023.0242","url":null,"abstract":"<p><p>Autoimmune etiologies are a common cause for encephalitis. The clinical syndromes consistent with autoimmune encephalitis are both distinct and increasingly recognized, but less is known about persisting sequelae or outcomes. We searched PubMed for reports on outcomes after autoimmune encephalitis. Studies assessing validated, quantitative outcomes were included. We performed a narrative review of the published literature of outcomes after autoimmune encephalitis. We found 146 studies that produced outcomes data. The mortality rates were 6%-19% and the relapse risks were 10%-62%. Most patients achieved a good outcome based on a score on the modified Rankin Scale (mRS) of ≤2. Forty-nine studies evaluated outcomes beyond mRS; these studies investigated cognitive outcome, psychiatric sequelae, neurological deficits, global function, and quality-of-life/patient-reported outcomes using various tools at varying time points after the index hospital discharge. These more-detailed assessments revealed that most patients had persistent impairments, with frequent deficits in cognitive function, especially memory and attention. Depression and anxiety were also common. Many of these sequelae continued to improve over months or even years after the acute illness. While we found that lasting impairments were common among survivors of autoimmune encephalitis, additional research is needed to better understand the nature and impact of these sequelae. Standardized evaluation protocols are needed to improve the ability to compare outcomes across studies, guide rehabilitation strategies, and inform outcomes of interest in treatment trials as the field advances.</p>","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":null,"pages":null},"PeriodicalIF":3.1,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10782092/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139097950","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":"Autism-Like Presentation of Possible Autoimmune Encephalitis With Complete Recovery After Immunotherapy.","authors":"Yongmoo Kim, Yoonhyuk Jang, Seolah Lee, Kon Chu","doi":"10.3988/jcn.2023.0245","DOIUrl":"10.3988/jcn.2023.0245","url":null,"abstract":"","PeriodicalId":15432,"journal":{"name":"Journal of Clinical Neurology","volume":null,"pages":null},"PeriodicalIF":3.1,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10782083/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139097942","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}