Journal of Clinical Neurology (Seoul, Korea)最新文献

筛选
英文 中文
Severity of Daytime Sleepiness and Parkinsonian-Like Symptoms in Korean Adults Aged 50-64 Years. 韩国50-64岁成人日间嗜睡和帕金森样症状的严重程度
IF 3.1
Journal of Clinical Neurology (Seoul, Korea) Pub Date : 2022-01-01 DOI: 10.3988/jcn.2022.18.1.33
Chol Shin, Regina E Y Kim, Robert J Thomas, Chang-Ho Yun, Seung Ku Lee, Robert D Abbott
{"title":"Severity of Daytime Sleepiness and Parkinsonian-Like Symptoms in Korean Adults Aged 50-64 Years.","authors":"Chol Shin,&nbsp;Regina E Y Kim,&nbsp;Robert J Thomas,&nbsp;Chang-Ho Yun,&nbsp;Seung Ku Lee,&nbsp;Robert D Abbott","doi":"10.3988/jcn.2022.18.1.33","DOIUrl":"https://doi.org/10.3988/jcn.2022.18.1.33","url":null,"abstract":"<p><strong>Background and purpose: </strong>While excessive daytime sleepiness can predate Parkinson's disease in late-life, its association with parkinsonian-like (P-L) symptoms in middle age are unknown. Since neurodegeneration can appear decades before a diagnosis of Parkinson's disease, identifying clinical features associated with this early progression is important. The purpose of this study was to determine the association of daytime sleepiness with P-L symptoms in a population-based sample of middle-aged Korean adults.</p><p><strong>Methods: </strong>During 2013 and 2014, daytime sleepiness and P-L symptoms were assessed in 2,063 males and females aged 50-64 years who were participating in the Korean Genome and Epidemiology Study. The severity of daytime sleepiness was quantified by the score on the Epworth Sleepiness Scale (ESS). Self-reported P-L symptoms included nine motor disorders commonly associated with Parkinson's disease. Participants with parkinsonism and related conditions are excluded.</p><p><strong>Results: </strong>The prevalence of excessive daytime sleepiness (ESS score >10) was 7.0%. The frequencies of P-L symptoms ranged from 0.5% (for \"trouble buttoning buttons\") to 18.4% (for \"handwriting smaller than it once was\"). After adjustment for covariates and multiple testing, the relative odds of P-L symptoms comparing the 80th and 20th percentiles of ESS scores was 1.6 (<i>p</i>=0.001) for \"voice is softer than it once was,\" 2.1 (<i>p</i><0.001) for \"balance when walking is poor,\" and 1.5 (<i>p</i>=0.002) for \"loss of facial expression.\" The prevalence of excessive daytime sleepiness increased from 6.3% to 19.8% when the number of symptoms increased from zero to three (<i>p</i>=0.004).</p><p><strong>Conclusions: </strong>In Korean adults aged 50-64 years, daytime sleepiness is significantly associated with P-L symptoms. Whether coexisting daytime sleepiness and P-L symptoms predate extrapyramidal and other impairments in later life warrants further investigation.</p>","PeriodicalId":324902,"journal":{"name":"Journal of Clinical Neurology (Seoul, Korea)","volume":" ","pages":"33-40"},"PeriodicalIF":3.1,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/76/e1/jcn-18-33.PMC8762500.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39814655","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Isolated Vestibular Syndrome With "Double-Panda" Sign in CNS Lymphoma. 中枢神经系统淋巴瘤伴“双熊猫”征的孤立前庭综合征。
IF 3.1
Journal of Clinical Neurology (Seoul, Korea) Pub Date : 2022-01-01 DOI: 10.3988/jcn.2022.18.1.111
Jeong-Yeon Kim, Jae-Hwan Choi, Eun Hye Oh, Seo-Young Choi, Hak-Jin Kim, Kwang-Dong Choi
{"title":"Isolated Vestibular Syndrome With \"Double-Panda\" Sign in CNS Lymphoma.","authors":"Jeong-Yeon Kim,&nbsp;Jae-Hwan Choi,&nbsp;Eun Hye Oh,&nbsp;Seo-Young Choi,&nbsp;Hak-Jin Kim,&nbsp;Kwang-Dong Choi","doi":"10.3988/jcn.2022.18.1.111","DOIUrl":"https://doi.org/10.3988/jcn.2022.18.1.111","url":null,"abstract":"Dear Editor, Central nervous system (CNS) lymphoma is a rare type of non-Hodgkin lymphoma in which malignant cells from lymphoid tissue form in the brain and spinal cord (primary) or spread from other parts of the body to the brain and spinal cord (secondary).1 CNS lymphoma can manifest with various neurological symptoms depending on its location.2 A characteristic imaging finding of primary CNS lymphoma (PCNSL) is a computed tomography (CT) hyperdense enhanced supratentorial mass that is hypointense in T1-weighted magnetic resonance imaging (MRI) and isoto hypointense in T2-weighted MRI, with vivid homogeneous enhancement and restricted diffusion.1-3 We describe distinctive neuro-otological findings and the typical “double-panda” sign on brain MRI in a patient with CNS lymphoma. A 65-year-old male presented with acute dizziness with a 3-day history. Oculography showed horizontal gaze-evoked nystagmus (GEN) during bilateral gaze, and impaired horizontal smooth pursuit (Supplementary Fig. 1A in the online-only Data Supplement). Saccades were normal. Video head impulse tests (HITs) revealed decreased vestibulo-ocular reflex (VOR) gains of both horizontal and posterior semicircular canals (Supplementary Fig. 1B in the online-only Data Supplement). Ocular vestibular evoked myogenic potentials (VEMPs) were decreased during right-ear stimulation, while cervical VEMPs were symmetric (Supplementary Fig. 1C and D in the online-only Data Supplement). The levels of serum ceruloplasmin and 24-hour urine copper were normal, and Kayser-Fleischer rings were not observed. Brain MRI showed the characteristic “double-panda” sign with additional increased T2-weighted signal intensities along the bilateral ventricular walls, thalamus, hypothalamus, mammillary body, optic tract, dorsal pons, and upper medulla, and contrast enhancement in the ependyma (Fig. 1A-C). There was slight improvement of symptoms after treatment with steroid. One month later the patient was readmitted due to a sudden decrease in mentality. Brain CT showed obstructive hydrocephalus (Fig. 1F), and an external ventricular drain was placed. The endoscopic biopsy confirmed diffuse large-B-cell lymphoma (Supplementary Fig. 2 in the online-only Data Supplement). To the best of our knowledge, the present patient is unique since he showed the typical “double-panda” sign on brain MRI due to CNS lymphoma, which is traditionally considered to be characteristic of Wilson’s disease.4 The midbrain face of the “giant-panda” sign comprises normal-intensity red nuclei (eyes) and lateral portions of substantia nigra pars reticulata (ears), with high signal intensity in the tegmentum and hypointensity in the superior colliculus (mouth) (Fig. 1D). The pons face of the “miniature-panda” sign consists of relative hypointensity of the central tegmental tracts (eyes) contrasting with the hyperintensity of the aqueduct opening into the fourth ventricle (nose and mouth) (Fig. 1E).4 Combining these features produces the ","PeriodicalId":324902,"journal":{"name":"Journal of Clinical Neurology (Seoul, Korea)","volume":" ","pages":"111-113"},"PeriodicalIF":3.1,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/f4/69/jcn-18-111.PMC8762499.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39904203","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Case Series of Osmotic Demyelination Syndrome Treated With Plasmapheresis: Experience From Two Tertiary Hospitals. 血浆置换治疗渗透性脱髓鞘综合征病例系列:两所三级医院的经验。
IF 3.1
Journal of Clinical Neurology (Seoul, Korea) Pub Date : 2022-01-01 DOI: 10.3988/jcn.2022.18.1.117
Kuan Yee Lim, Yuen Kang Chia, Ching Soong Khoo, Hui Jan Tan
{"title":"Case Series of Osmotic Demyelination Syndrome Treated With Plasmapheresis: Experience From Two Tertiary Hospitals.","authors":"Kuan Yee Lim,&nbsp;Yuen Kang Chia,&nbsp;Ching Soong Khoo,&nbsp;Hui Jan Tan","doi":"10.3988/jcn.2022.18.1.117","DOIUrl":"https://doi.org/10.3988/jcn.2022.18.1.117","url":null,"abstract":"","PeriodicalId":324902,"journal":{"name":"Journal of Clinical Neurology (Seoul, Korea)","volume":" ","pages":"117-119"},"PeriodicalIF":3.1,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/9b/7a/jcn-18-117.PMC8762491.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39904205","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 2
Huntington's Disease Presenting as Adult-Onset Parkinsonism. 亨廷顿氏病表现为成人发病的帕金森病。
IF 3.1
Journal of Clinical Neurology (Seoul, Korea) Pub Date : 2022-01-01 DOI: 10.3988/jcn.2022.18.1.87
In Hee Kwak, Na Hee Kim, Hyeo-Il Ma, Young Eun Kim
{"title":"Huntington's Disease Presenting as Adult-Onset Parkinsonism.","authors":"In Hee Kwak,&nbsp;Na Hee Kim,&nbsp;Hyeo-Il Ma,&nbsp;Young Eun Kim","doi":"10.3988/jcn.2022.18.1.87","DOIUrl":"https://doi.org/10.3988/jcn.2022.18.1.87","url":null,"abstract":"Dear Editor, Huntington’s disease (HD) is archetypally characterized as progressive cognitive decline, psychiatric disturbance, and involuntary movements.1-7 Chorea is the most common movement feature, appearing in over 90% of HD patients.3 These symptoms usually start between 30 and 40 years of age, with this variation in the onset age being influenced by the cytosineadenine-guanine (CAG) repeat expansion length.2-4 The Westphal variant of HD that presents as a distinct clinical entity characterized by parkinsonism is prevalent in juvenile-onset HD and has longer CAG expansions than the typical choreic form of adult-onset HD.1-3,7 Therefore, a sole manifestation with parkinsonism in adult-onset HD is rare.1,2,6,8 Previous studies have found that the Westphal variant accounts for 85% of cases of juvenile-onset HD, whereas only 6%–9% of patients with adult-onset HD have initial manifestations of parkinsonism.3,5,6 Here we report the clinical assessment and course of an adult patient with HD who presented as young-onset parkinsonism with a diffuse presynaptic dopaminergic deficit and showed early responsiveness to levodopa. A 34-year-old male developed progressive bradykinesia and gait disturbance over 4 months. He had no underlying medical problems or medication use, but there was a 10-year history of vivid dream and dream enactment behaviors. He was an only child and had no definite family history on his mother’s side. The findings of a neurological examination of his mother were normal. His father died in his 40s after suffering from progressive motor deterioration with psychiatric problems following a traffic collision. No reliable family history was obtained from his father or other relatives because he had not had contact with them since he was a child. A neurological examination of the patient revealed generalized symmetric parkinsonism including masked face, bradykinesia, rigidity, and postural instability (Supplementary Video 1 in the online-only Data Supplement). His speech was mildly dysarthric, and deep tendon reflexes were increased. He had a score of 36 on the Unified Parkinson’s Disease Rating Scale (UPDRS) motor subscale, and was at stage 3 on the Hoehn and Yahr scale. Administering levodopa at 200 mg daily provided symptomatic improvement, and his UPDRS motor score reduced to 26. Possible causes of young-onset parkinsonism were evaluated based on these findings. Brain magnetic resonance imaging (MRI) using various imaging sequences did not reveal any signal abnormality other than brain atrophy (Fig. 1A-H). 18F-FP-CIT positron-emission tomography (PET) for presynaptic dopamine transporter imaging revealed symmetric decreased uptake in the bilateral striatum including caudate and putamen overall compared with a healthy control (Fig. 1I-L). Considering the unclear family history, gene tests were performed for spinocerebellar ataxia 2 and PARK2, and the findings for both were normal. However, unlike for cognition, the scores on the Min","PeriodicalId":324902,"journal":{"name":"Journal of Clinical Neurology (Seoul, Korea)","volume":" ","pages":"87-89"},"PeriodicalIF":3.1,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/dd/7e/jcn-18-87.PMC8762510.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39904196","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Impact of General Factors on Glioma Immunotherapy. 一般因素对神经胶质瘤免疫治疗的影响。
IF 3.1
Journal of Clinical Neurology (Seoul, Korea) Pub Date : 2022-01-01 DOI: 10.3988/jcn.2022.18.1.3
Qilin Huang, Dongmei Wang, Guojie Yao, Hongxiang Wang
{"title":"Impact of General Factors on Glioma Immunotherapy.","authors":"Qilin Huang,&nbsp;Dongmei Wang,&nbsp;Guojie Yao,&nbsp;Hongxiang Wang","doi":"10.3988/jcn.2022.18.1.3","DOIUrl":"https://doi.org/10.3988/jcn.2022.18.1.3","url":null,"abstract":"<p><p>Glioma remains the most common malignant tumor in the brain and is also the most difficult to treat. Immunotherapy achieving long-lasting tumor remission in multiple cancer types has received considerable attention due to its potential to improve the treatment outcomes of patients with glioma. However, clinical trials have not yet demonstrated major improvements in prognoses, which might be attributable to the extrinsic components and intrinsic mechanisms involved in the tumor microenvironment and immune system. It is particularly noteworthy that there is emerging evidence that current routine treatment modalities and the physical and psychological characteristics of patients have different impacts on the efficacy of glioma immunotherapy. This article addresses how these factors interact with the host immune system and tumor microenvironment, and highlights their potential roles in glioma immunotherapy, with the ultimate goal of developing better immunotherapy-based personalized medicine strategies.</p>","PeriodicalId":324902,"journal":{"name":"Journal of Clinical Neurology (Seoul, Korea)","volume":" ","pages":"3-13"},"PeriodicalIF":3.1,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/1f/b0/jcn-18-3.PMC8762502.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39814304","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 2
Novel Variants in the FIG4 Gene Associated With Chinese Sporadic Amyotrophic Lateral Sclerosis With Slow Progression. 与进展缓慢的散发性肌萎缩性侧索硬化症相关的FIG4基因新变异
IF 3.1
Journal of Clinical Neurology (Seoul, Korea) Pub Date : 2022-01-01 DOI: 10.3988/jcn.2022.18.1.41
Chang-Yun Liu, Ji-Lan Lin, Shu-Yan Feng, Chun-Hui Che, Hua-Pin Huang, Zhang-Yu Zou
{"title":"Novel Variants in the <i>FIG4</i> Gene Associated With Chinese Sporadic Amyotrophic Lateral Sclerosis With Slow Progression.","authors":"Chang-Yun Liu,&nbsp;Ji-Lan Lin,&nbsp;Shu-Yan Feng,&nbsp;Chun-Hui Che,&nbsp;Hua-Pin Huang,&nbsp;Zhang-Yu Zou","doi":"10.3988/jcn.2022.18.1.41","DOIUrl":"https://doi.org/10.3988/jcn.2022.18.1.41","url":null,"abstract":"<p><strong>Background and purpose: </strong>Mutations in the <i>FIG4</i> gene have been linked to amyotrophic lateral sclerosis (ALS) type 11 in Caucasian populations. The purpose of this study was to identify <i>FIG4</i> variants in a cohort of 15 familial ALS (FALS) indexes and 275 sporadic ALS (SALS) patients of Han Chinese origin.</p><p><strong>Methods: </strong>All 23 exons of <i>FIG4</i> were sequenced using targeted next-generation sequencing. An extensive literature review was performed to detect genotype-phenotype associations of <i>FIG4</i> mutations.</p><p><strong>Results: </strong>No <i>FIG4</i> variants were identified in the FALS patients. One novel heterozygous missense variant (c.352G>T [p.D118Y]) and one novel heterozygous nonsense variant (c.2158G>T [p.E720X]) in <i>FIG4</i> were identified in two SALS patients. The p.E720X variant is interpreted as likely pathogenic while the p.D118Y variant is a variant of uncertain significance. The patient carrying the p.E720X mutation developed lower-limb-onset slowly progressive ALS, and survived for 11.5 years. The patient harboring the <i>FIG4</i> p.D118Y variant also presented with progressive ALS, with the score on the ALS Functional Rating Scale-Revised (ALSFRS-R) decreasing by 0.4 per month. The rate of decrease in the ALSFRS-R scores from symptom onset to diagnosis seemed to be lower in the patients carrying <i>FIG4</i> variants than the no-<i>FIG4</i>-mutation ALS patients in this study.</p><p><strong>Conclusions: </strong>Our findings suggest that ALS patients carrying <i>FIG4</i> mutations are not common in the Chinese population and are more likely to exhibit slow progression.</p>","PeriodicalId":324902,"journal":{"name":"Journal of Clinical Neurology (Seoul, Korea)","volume":" ","pages":"41-47"},"PeriodicalIF":3.1,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/54/95/jcn-18-41.PMC8762508.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39814656","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 5
Association of Zolpidem With Increased Mortality in Patients With Brain Cancer: A Retrospective Cohort Study Based on the National Health Insurance Service Database. 唑吡坦与脑癌患者死亡率增加的关系:基于国家健康保险服务数据库的回顾性队列研究
IF 3.1
Journal of Clinical Neurology (Seoul, Korea) Pub Date : 2022-01-01 DOI: 10.3988/jcn.2022.18.1.65
Sungeun Hwang, Hyoshin Son, Manho Kim, Sang Kun Lee, Ki-Young Jung
{"title":"Association of Zolpidem With Increased Mortality in Patients With Brain Cancer: A Retrospective Cohort Study Based on the National Health Insurance Service Database.","authors":"Sungeun Hwang,&nbsp;Hyoshin Son,&nbsp;Manho Kim,&nbsp;Sang Kun Lee,&nbsp;Ki-Young Jung","doi":"10.3988/jcn.2022.18.1.65","DOIUrl":"https://doi.org/10.3988/jcn.2022.18.1.65","url":null,"abstract":"<p><strong>Background and purpose: </strong>Zolpidem is one of the most common hypnotics prescribed to treat insomnia worldwide. However, there are numerous reports of a positive association between zolpidem and mortality, including an association with increased cancer-specific mortality found in a Taiwanese cohort study. This study aimed to determine the association between zolpidem use and brain-cancer-specific mortality in patients with brain cancer.</p><p><strong>Methods: </strong>This population-based, retrospective cohort study analyzed data in the National Health Insurance Service database. All incident cases of brain cancer at an age of ≥18 years at the time of brain cancer diagnosis over a 15-year period (2003-2017) were included. A multivariate Cox regression analysis after adjustment for covariables was performed to evaluate the associations of zolpidem exposure with brain-cancer-specific and all-cause mortality.</p><p><strong>Results: </strong>This study identified 38,037 incident cases of brain cancer, among whom 11,823 (31.1%) patients were exposed to zolpidem. In the multivariate Cox regression model, the brain-cancer-specific mortality rate was significantly higher in patients who were prescribed zolpidem than in those with no zolpidem prescription (adjusted hazard ratio [HR]=1.14, 95% confidence interval [CI]=1.08-1.21, <i>p</i><0.001). Zolpidem exposure was significantly associated with increased brain-cancer-specific mortality after adjustment in younger adults (age 18-64 years; adjusted HR=1.37, 95% CI=1.27-1.49) but not in older adults (age ≥65 years; adjusted HR=0.94, 95% CI=0.86-1.02).</p><p><strong>Conclusions: </strong>Zolpidem exposure was significantly associated with increased brain-cancer-specific mortality in patients with brain cancer aged 18-64 years. Further prospective studies are warranted to understand the mechanism underlying the effect of zolpidem on mortality in patients with brain cancer.</p>","PeriodicalId":324902,"journal":{"name":"Journal of Clinical Neurology (Seoul, Korea)","volume":" ","pages":"65-70"},"PeriodicalIF":3.1,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/48/07/jcn-18-65.PMC8762495.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39814658","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Efficacy of the Ketogenic Diet for Pediatric Epilepsy According to the Presence of Detectable Somatic mTOR Pathway Mutations in the Brain. 根据大脑中可检测的体细胞mTOR通路突变的存在,生酮饮食对儿童癫痫的疗效。
IF 3.1
Journal of Clinical Neurology (Seoul, Korea) Pub Date : 2022-01-01 DOI: 10.3988/jcn.2022.18.1.71
Ara Ko, Nam Suk Sim, Han Som Choi, Donghwa Yang, Se Hee Kim, Joon Soo Lee, Dong Seok Kim, Jeong Ho Lee, Heung Dong Kim, Hoon-Chul Kang
{"title":"Efficacy of the Ketogenic Diet for Pediatric Epilepsy According to the Presence of Detectable Somatic mTOR Pathway Mutations in the Brain.","authors":"Ara Ko,&nbsp;Nam Suk Sim,&nbsp;Han Som Choi,&nbsp;Donghwa Yang,&nbsp;Se Hee Kim,&nbsp;Joon Soo Lee,&nbsp;Dong Seok Kim,&nbsp;Jeong Ho Lee,&nbsp;Heung Dong Kim,&nbsp;Hoon-Chul Kang","doi":"10.3988/jcn.2022.18.1.71","DOIUrl":"https://doi.org/10.3988/jcn.2022.18.1.71","url":null,"abstract":"<p><strong>Background and purpose: </strong>A multifactorial antiepileptic mechanism underlies the ketogenic diet (KD), and one of the proposed mechanisms of action is that the KD inhibits the mammalian target of rapamycin (mTOR) pathway. To test this clinically, this study aimed to determine the efficacy of the KD in patients with pathologically confirmed focal cortical dysplasia (FCD) due to genetically identifiable mTOR pathway dysregulation.</p><p><strong>Methods: </strong>A cohort of patients with pathologically confirmed FCD after epilepsy surgery and who were screened for the presence of germline and somatic mutations related to the mTOR pathway in peripheral blood and resected brain tissue was constructed prospectively. A retrospective review of the efficacy of the prior KD in these patients was performed.</p><p><strong>Results: </strong>Twenty-five patients with pathologically confirmed FCD and who were screened for the presence of detectable somatic mTOR pathway mutations had received a sufficient KD. Twelve of these patients (48.0%) had germline or somatic detectable mTOR pathway mutations. A response was defined as a ≥50% reduction in seizure frequency. The efficacy of the KD after 3 months of dietary therapy was superior in patients with detectable mTOR pathway mutations than in patients without detectable mTOR pathway mutations, although the difference was not statistically significant (responder rates of 58.3% vs. 38.5%, <i>p</i>=0.434).</p><p><strong>Conclusions: </strong>A greater proportion of patients with mTOR pathway responded to the KD, but there was no statistically significant difference in efficacy of the KD between patients with and without detectable mTOR pathway mutations. Further study is warranted due to the smallness of the sample and the limited number of mTOR pathway genes tested in this study.</p>","PeriodicalId":324902,"journal":{"name":"Journal of Clinical Neurology (Seoul, Korea)","volume":" ","pages":"71-78"},"PeriodicalIF":3.1,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/c2/1a/jcn-18-71.PMC8762511.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39904194","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 4
Varicella Zoster Virus Reactivation in Central and Peripheral Nervous Systems Following COVID-19 Vaccination in an Immunocompetent Patient. 免疫正常患者接种COVID-19疫苗后中枢和周围神经系统水痘带状疱疹病毒再激活
IF 3.1
Journal of Clinical Neurology (Seoul, Korea) Pub Date : 2022-01-01 DOI: 10.3988/jcn.2022.18.1.99
Seungyon Koh, Hong Nam Kim, Yoon Seob Kim, Tae-Joon Kim
{"title":"Varicella Zoster Virus Reactivation in Central and Peripheral Nervous Systems Following COVID-19 Vaccination in an Immunocompetent Patient.","authors":"Seungyon Koh,&nbsp;Hong Nam Kim,&nbsp;Yoon Seob Kim,&nbsp;Tae-Joon Kim","doi":"10.3988/jcn.2022.18.1.99","DOIUrl":"https://doi.org/10.3988/jcn.2022.18.1.99","url":null,"abstract":"Dear Editor, The increasing vaccination rate for coronavirus disease 2019 (COVID-19) worldwide is leading to concerns about adverse events. Reactivation of varicella zoster virus (VZV) as herpes zoster (HZ) after COVID-19 vaccination has been reported in a series of adult patients,1,2 especially in older subjects. VZV reactivation occurs as a wide clinical spectrum, from HZ to a central nervous system manifestation such as meningitis or encephalitis. Here we report a case of an immunocompetent young adult with both VZV meningitis and HZ following COVID-19 vaccination. A healthy 24-year-old male with normal cognition visited our emergency room (ER) presenting with a headache. His medical history was remarkable, with varicella as a child and aseptic meningitis at 12 years of age. He was vaccinated with the first dose of the PfizerBNT162b2 vaccine against SARS-CoV-2 into his left deltoid at 3 days prior to the visit. From the following day, myalgia and fatigue gradually developed. On the 4th day after vaccination, he decided to visit the ER because a headache started and small vesicles had formed on his left upper arm, just below the vaccine injection site. On presentation, the patient was febrile (37.7°C) with otherwise normal vital signs. He had no neurological deficit, but neck stiffness and positive jolt accentuation were observed. An erythematous patch with grouped vesicles suggestive of HZ was present on his left upper arm within the C5 dermatome (Fig. 1). Laboratory tests revealed a white blood cell (WBC) count of 5,700/μL (21% lymphocytes) in the complete blood count. The findings of other tests including a coagulation panel, electrolyte, chemistry panel, serology, urinalysis, and chest X-ray were within the normal ranges. Anti-VZV IgM/IgG, anti-cytomegalovirus IgM, and anti-HSV IgM antibodies were negative in the blood test. A cerebrospinal fluid (CSF) study was performed, which revealed an opening pressure of 180 mm H2O, WBC count of 11/μL, 98% lymphocytes, 50.6 mg/dL protein, and 53 mg/dL glucose, while the blood glucose level was 90 mg/dL. CSF polymerase chain reaction for VZV yielded a positive result, and a diagnosis of VZV meningitis was made. He refused admission, and so was treated with oral famciclovir. At the follow-up visit after a week, he reported a mild headache that lasted about 1 week, and then his general condition and fever had improved as the systemic VZV reactivation and meningitis resolved. This case illustrates the central and peripheral nervous system manifestations of VZV reactivation in an immunocompetent young adult following COVID-19 vaccination. To our knowledge, this is the first report of concomitant VZV meningitis and HZ following COVID-19 vaccination. Cases of HZ reactivation as adverse events following immunization were also observed during the prepandemic era for both inactivated virus vaccines such as influenza vaccines and attenuated virus vaccines such as Japanese encephalitis vacSeungyon Koh Hong Nam Ki","PeriodicalId":324902,"journal":{"name":"Journal of Clinical Neurology (Seoul, Korea)","volume":" ","pages":"99-101"},"PeriodicalIF":3.1,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/40/5e/jcn-18-99.PMC8762505.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39904200","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 4
Association between the Levofloxacin Plasma Concentration and Neurological Adverse Events in an Elderly Patient 老年患者左氧氟沙星血浆浓度与神经系统不良事件的关系
Journal of Clinical Neurology (Seoul, Korea) Pub Date : 2019-10-01 DOI: 10.3988/jcn.2019.15.4.572
Gaeun Kang, S. Min, Jong Keun Kim, K. Kang
{"title":"Association between the Levofloxacin Plasma Concentration and Neurological Adverse Events in an Elderly Patient","authors":"Gaeun Kang, S. Min, Jong Keun Kim, K. Kang","doi":"10.3988/jcn.2019.15.4.572","DOIUrl":"https://doi.org/10.3988/jcn.2019.15.4.572","url":null,"abstract":"Dear Editor, Levofloxacin is a fluoroquinolone antibiotic used to treat bacterial infections such as community-acquired pneumonia and complicated urinary-tract infections. The labels of drugs across the fluoroquinolone class mention side effects related to the central nervous system (CNS). The US Food and Drug Administration and the European Medicines Agency recently issued a warned regarding the CNS side effects related to fluoroquinolones.1,2 Here we report rare adverse effects of the fluoroquinolone drug levofloxacin along with the plasma drug concentration at the time of each occurrence, and assess the causality between levofloxacin administration and such events. A 78-year-old man with diabetes mellitus, hypertension, and a past history of old left basal ganglia hemorrhage and cerebellar lacunar infarct (10 years previously) visited our hospital with a seizure. He had no prior psychiatric history or seizure episodes. For 2 days prior to his visit he had received daily intravenous infusions of 500 mg of levofloxacin for pneumonia at another hospital. On the second day after his admission at that hospital he presented with a confused mental state, difficulty of sleep initiation, and visual hallucinations in which he reported seeing people who were not there. His other medications at the time did not include sedative agents such as hypnotics, but included the following oral medications daily: clopidogrel (75 mg), choline alfoscerate (400 mg), rebamipide (100 mg), nizatidine (150 mg), and erdosteine (300 mg). On the third day of hospitalization the patient experienced two generalized tonic-clonic seizures (at around 4 a.m. and 8 a.m.), and so he was transferred to our hospital. Levofloxacin had not been administered on the transfer day, on which his neurological signs normalized and he slept well. Upon admission to our hospital the patient exhibited an alert mental status without fever or focal neurological deficits except for the sequelae of the previous stroke. We ruled out other conditions such as dyselectrolytemia, hypoglycemia, and diabetic ketoacidosis via laboratory testing. Brain computed tomography and magnetic resonance imaging also produced no new abnormal findings. However, electroencephalography showed focal left-side slowing without epileptiform discharges. Our patient was diagnosed with remote poststroke seizure and was treated with oral lamotrigine for the seizures and intravenous levofloxacin for pneumonia. On the fourth day of hospitalization, 500 mg of intravenous levofloxacin was restarted in our hospital at approximately 9 a.m. (producing a levofloxacin plasma concentration of 0.18 μg/mL), and by 11 p.m. the patient gradually showed irritability and a confused mental state again, at which time the levofloxacin concentration had reached 2.36 μg/mL. On the fifth day of hospitalization, 250 mg of levofloxacin (half the previous dose) was administered. The plasma concentration of levofloxacin at 2 hours after that injection was ","PeriodicalId":324902,"journal":{"name":"Journal of Clinical Neurology (Seoul, Korea)","volume":"6 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2019-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"126150209","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 4
0
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
相关产品
×
本文献相关产品
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:604180095
Book学术官方微信