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

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Spinocerebellar Ataxia 48 Patient With a Novel De Novo Variant of STUB1. 脊髓小脑性共济失调48例伴有新的新生变异STUB1。
IF 3.1
Journal of Clinical Neurology (Seoul, Korea) Pub Date : 2022-11-01 DOI: 10.3988/jcn.2022.18.6.714
Soyoun Choi, Soo Ryun Park, Ja-Hyun Jang, Jong Hyeon Ahn
{"title":"Spinocerebellar Ataxia 48 Patient With a Novel De Novo Variant of <i>STUB1</i>.","authors":"Soyoun Choi,&nbsp;Soo Ryun Park,&nbsp;Ja-Hyun Jang,&nbsp;Jong Hyeon Ahn","doi":"10.3988/jcn.2022.18.6.714","DOIUrl":"https://doi.org/10.3988/jcn.2022.18.6.714","url":null,"abstract":"","PeriodicalId":324902,"journal":{"name":"Journal of Clinical Neurology (Seoul, Korea)","volume":" ","pages":"714-716"},"PeriodicalIF":3.1,"publicationDate":"2022-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/dd/92/jcn-18-714.PMC9669560.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40699598","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
Electroclinical Characteristics and 18F-Fluorodeoxyglucose Positron-Emission Tomography Findings of Familial Scotosensitivity. 家族性斯科特敏感性的电临床特征和18f -氟脱氧葡萄糖正电子发射断层扫描结果。
IF 3.1
Journal of Clinical Neurology (Seoul, Korea) Pub Date : 2022-11-01 DOI: 10.3988/jcn.2022.18.6.717
Kyung Wook Kang, Ja-Hae Kim, Jae-Myung Kim, Myeong-Kyu Kim
{"title":"Electroclinical Characteristics and <sup>18</sup>F-Fluorodeoxyglucose Positron-Emission Tomography Findings of Familial Scotosensitivity.","authors":"Kyung Wook Kang,&nbsp;Ja-Hae Kim,&nbsp;Jae-Myung Kim,&nbsp;Myeong-Kyu Kim","doi":"10.3988/jcn.2022.18.6.717","DOIUrl":"https://doi.org/10.3988/jcn.2022.18.6.717","url":null,"abstract":"","PeriodicalId":324902,"journal":{"name":"Journal of Clinical Neurology (Seoul, Korea)","volume":" ","pages":"717-719"},"PeriodicalIF":3.1,"publicationDate":"2022-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/1e/1f/jcn-18-717.PMC9669563.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40699599","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
Vertical Saccadic Slowing in Episodic Ataxia Type 2. 2型共济失调的垂直跳跃性减慢。
IF 3.1
Journal of Clinical Neurology (Seoul, Korea) Pub Date : 2022-11-01 DOI: 10.3988/jcn.2022.18.6.726
Seoyeon Kim, Seondeuk Kim, Seonkyung Lee, Hyo-Jung Kim
{"title":"Vertical Saccadic Slowing in Episodic Ataxia Type 2.","authors":"Seoyeon Kim,&nbsp;Seondeuk Kim,&nbsp;Seonkyung Lee,&nbsp;Hyo-Jung Kim","doi":"10.3988/jcn.2022.18.6.726","DOIUrl":"https://doi.org/10.3988/jcn.2022.18.6.726","url":null,"abstract":"Dear Editor, Episodic ataxia (EA) is a group of disorders characterized by recurrent attacks of truncal ataxia and incoordination.1,2 Among the eight subtypes, EA type 2 (EA2) is the most common and is inherited in an autosomal dominant pattern due to mutations in the voltagegated Cav2.1 P/Q-type calcium channel (CACNA1A).2,3 Patients with EA2 typically present with recurrent ataxia that lasts minutes to hours with interictal ocular motor abnormalities mostly due to cerebellar dysfunction.1 Here we report vertical saccade slowing in a patient with EA2, which is a previously undescribed finding in this disorder. A 17-year-old man with a family history of EA2 had been followed up in a dizziness clinic for 7 years. His elder sister had a confirmed EA2 diagnosis based on characteristic clinical features and a heterozygous mutation (c.4953+1G&gt;A) at the intron 31 splice donor site in the CACNA1A gene (reference sequence=NG_011569).4 Findings of the initial evaluation at 10 years old were conspicuous for gaze-evoked and downbeat nystagmus during lateral gaze (side-pocket nystagmus), and rebound upbeat nystagmus when resuming the straight-ahead gaze.4 There was a curved trajectory in the upward saccades, but the velocity was within the normal range.4 Smooth pursuit and visual enhancement of the vestibulo-ocular reflex were impaired in both the horizontal and vertical directions.4 The other findings of the patient and his family members have been described previously.4 A follow-up evaluation 7 years later additionally indicated slowing of the vertical saccades in both directions (Fig. 1; Supplementary Video 1 in the online-only Data Supplement). Horizontal saccades were normal (Fig. 1). Other findings of the neurological examination remained unchanged. Genetic analysis confirmed the same mutation as the one found in his sister. Our patient with EA2 due to a mutation in the CACNA1A gene presented with vertical saccade slowing during the follow-up at 7 years after the initial presentation. Premotor commands for saccades are generated in the brainstem via interactions between the burst and omnipause neurons.5 Disruption of these brainstem neural networks results in saccade slowing. The excitatory burst neurons for horizontal saccades lie in the paramedian pontine reticular formation, while those for vertical saccades reside in the rostral interstitial nucleus of the medial longitudinal fasciculus.5 In EA2, interictal ocular motor abnormalities have mostly been ascribed to cerebellar dysfunction and include downbeat nystagmus, gaze-evoked nystagmus, positional nystagmus, impaired smooth pursuit, and hypermetric saccades.2,6 However, the selective vertical saccade slowing observed in our patient with EA2 indicated an additional involvement of the upper midbrain in this disorder.7 It is interesting that previous reports also described slow horizontal saccades8 and internuclear ophthalmoparesis9 as rare findings in EA2, which also indicates a brainstem involveme","PeriodicalId":324902,"journal":{"name":"Journal of Clinical Neurology (Seoul, Korea)","volume":" ","pages":"726-728"},"PeriodicalIF":3.1,"publicationDate":"2022-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/69/17/jcn-18-726.PMC9669551.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40699602","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
Amino Acid Levels as Potential Biomarkers of Multiple Sclerosis in Elderly Patients: Preliminary Report. 氨基酸水平作为老年多发性硬化症的潜在生物标志物:初步报告。
IF 3.1
Journal of Clinical Neurology (Seoul, Korea) Pub Date : 2022-09-01 DOI: 10.3988/jcn.2022.18.5.529
Łukasz Rzepiński, Piotr Kośliński, Marcin Gackowski, Marcin Koba, Zdzisław Maciejek
{"title":"Amino Acid Levels as Potential Biomarkers of Multiple Sclerosis in Elderly Patients: Preliminary Report.","authors":"Łukasz Rzepiński,&nbsp;Piotr Kośliński,&nbsp;Marcin Gackowski,&nbsp;Marcin Koba,&nbsp;Zdzisław Maciejek","doi":"10.3988/jcn.2022.18.5.529","DOIUrl":"https://doi.org/10.3988/jcn.2022.18.5.529","url":null,"abstract":"<p><strong>Background and purpose: </strong>Aging in multiple sclerosis is associated with both disease- and age-dependent neurodegeneration. Serum metabolomic profiling of amino acids seems to be a promising method for searching for biomarkers of neurodegenerative disorders. The aim of this study was to determine the profile of nonessential amino acids in the serum of elderly patients with secondary progressive multiple sclerosis (SPMS).</p><p><strong>Methods: </strong>We used high-performance liquid chromatography to evaluate the serum concentrations of nonessential amino acids in subjects aged >65 years: six patients with SPMS and 20 control subjects (CS).</p><p><strong>Results: </strong>The serine and alanine levels were significantly higher in SPMS patients than in CS, whereas the concentrations of aspartic acid, arginine, and cysteine were significantly lower in SPMS patients. These observations indicate that amino acids may be involved in SPMS neurodegeneration mechanisms. There were no significant differences in the serum concentrations of the other four amino acids investigated (glutamic acid, glycine, proline, and tyrosine) between patients with SPMS and CS.</p><p><strong>Conclusions: </strong>The preliminary results obtained in the study suggest that the metabolism of some amino acids is altered in patient with SPMS. We also conclude that amino acid profiling might be helpful in searching for putative biomarkers of central nervous system diseases. However, considering the multifactorial, heterogeneous, and complex nature of SPMS, further validation research involving larger study samples is required before applying these biomarkers in diagnostic practice.</p>","PeriodicalId":324902,"journal":{"name":"Journal of Clinical Neurology (Seoul, Korea)","volume":" ","pages":"529-534"},"PeriodicalIF":3.1,"publicationDate":"2022-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/18/6d/jcn-18-529.PMC9444553.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40350818","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: 3
Clinical Usefulness of Simultaneous Electroencephalography and Functional Magnetic Resonance Imaging in Children With Focal Epilepsy. 小儿局灶性癫痫同时脑电图和功能磁共振成像的临床价值。
IF 3.1
Journal of Clinical Neurology (Seoul, Korea) Pub Date : 2022-09-01 DOI: 10.3988/jcn.2022.18.5.535
Yun Jeong Lee, Hyunwoo Bae, Jun Chul Byun, Soonhak Kwon, Sung Suk Oh, Saeyoon Kim
{"title":"Clinical Usefulness of Simultaneous Electroencephalography and Functional Magnetic Resonance Imaging in Children With Focal Epilepsy.","authors":"Yun Jeong Lee,&nbsp;Hyunwoo Bae,&nbsp;Jun Chul Byun,&nbsp;Soonhak Kwon,&nbsp;Sung Suk Oh,&nbsp;Saeyoon Kim","doi":"10.3988/jcn.2022.18.5.535","DOIUrl":"https://doi.org/10.3988/jcn.2022.18.5.535","url":null,"abstract":"<p><strong>Background and purpose: </strong>The current study analyzed the interictal epileptiform discharge (IED)-related hemodynamic response and aimed to determine the clinical usefulness of simultaneous electroencephalography and functional magnetic resonance imaging (EEG-fMRI) in defining the epileptogenic zone (EZ) in children with focal epilepsy.</p><p><strong>Methods: </strong>Patients with focal epilepsy showing IEDs on conventional EEG were evaluated using EEG-fMRI. Statistical analyses were performed using the times of spike as events modeled with multiple hemodynamic response functions. The area showing the most significant <i>t</i>-value for blood-oxygen-level-dependent (BOLD) changes was compared with the presumed EZ. Moreover, BOLD responses between -9 and +9 s around the spike times were analyzed to track the hemodynamic response patterns over time.</p><p><strong>Results: </strong>Half (<i>n</i>=13) of 26 EEG-fMRI investigations of 19 patients were successful. Two patients showed 2 different types of spikes, resulting in 15 analyses. The maximum BOLD response was concordant with the EZ in 11 (73.3%) of the 15 analyses. In 10 (66.7%) analyses, the BOLD response localized the EZs more specifically. Focal BOLD responses in the EZs occurred before IEDs in 11 analyses and were often widespread after IEDs. Hemodynamic response patterns were consistent in the same epilepsy syndrome or when repeating the investigation in the same patients.</p><p><strong>Conclusions: </strong>EEG-fMRI can provide additional information for localizing the EZ in children with focal epilepsy, and also reveal the pathogenesis of pediatric epilepsy by evaluating the patterns in the hemodynamic response across time windows of IEDs.</p>","PeriodicalId":324902,"journal":{"name":"Journal of Clinical Neurology (Seoul, Korea)","volume":" ","pages":"535-546"},"PeriodicalIF":3.1,"publicationDate":"2022-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/6b/5a/jcn-18-535.PMC9444567.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40350819","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
Occurrence of Intracranial Hemorrhage and Associated Risk Factors in Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy: A Systematic Review and Meta-Analysis. 脑常染色体显性动脉病变伴皮质下梗死和白质脑病颅内出血的发生及相关危险因素:一项系统综述和荟萃分析
IF 3.1
Journal of Clinical Neurology (Seoul, Korea) Pub Date : 2022-09-01 DOI: 10.3988/jcn.2022.18.5.499
Qi-Lun Lai, Yin-Xi Zhang, Jun-Jun Wang, Ye-Jia Mo, Li-Ying Zhuang, Lin Cheng, Shi-Ting Weng, Song Qiao, Lu Liu
{"title":"Occurrence of Intracranial Hemorrhage and Associated Risk Factors in Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy: A Systematic Review and Meta-Analysis.","authors":"Qi-Lun Lai,&nbsp;Yin-Xi Zhang,&nbsp;Jun-Jun Wang,&nbsp;Ye-Jia Mo,&nbsp;Li-Ying Zhuang,&nbsp;Lin Cheng,&nbsp;Shi-Ting Weng,&nbsp;Song Qiao,&nbsp;Lu Liu","doi":"10.3988/jcn.2022.18.5.499","DOIUrl":"https://doi.org/10.3988/jcn.2022.18.5.499","url":null,"abstract":"<p><strong>Background and purpose: </strong>Intracranial hemorrhage (ICH) is thought to be a rare but probably underestimated presentation of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). We conducted a systematic review and meta-analysis with the aim of comprehensively revealing the occurrence of ICH in patients with CADASIL.</p><p><strong>Methods: </strong>English-language studies published up to September 30, 2021 were searched for in the MEDLINE (PubMed), Web of Science, and Cochrane Library databases. The design, patient characteristics, occurrence rate of ICH, and associated risk factors were retrieved for each identified relevant study.</p><p><strong>Results: </strong>We enrolled 13 studies in the final meta-analysis, which included 1,310 patients with CADASIL. The probability of ICH occurrence in patients with CADASIL was 10.1% (95% confidence interval [CI]=5.6%-18.0%, I²=85.1%). When stratified by geographic region, the occurrence rate of ICH was much higher in Asians (17.7%; 95% CI=11.0%-28.5%, I²=76.3%) than in Europeans (2.0%; 95% CI=0.4%-10.8%, I²=82.8%). A higher burden of cerebral microbleeds (CMBs) and a history of hypertension were the most commonly recorded risk factors for ICH, which were available for three and two of the included studies, respectively.</p><p><strong>Conclusions: </strong>Our study suggests that ICH is an important clinical manifestation of CADASIL, especially in Asians. A higher burden of CMBs and the existence of hypertension were found to be associated with a higher probability of ICH occurrence in patients with CADASIL.</p>","PeriodicalId":324902,"journal":{"name":"Journal of Clinical Neurology (Seoul, Korea)","volume":" ","pages":"499-506"},"PeriodicalIF":3.1,"publicationDate":"2022-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/9b/cd/jcn-18-499.PMC9444563.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40351387","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: 3
Multidimensional Early Prediction Score for Drug-Resistant Epilepsy. 耐药癫痫多维早期预测评分。
IF 3.1
Journal of Clinical Neurology (Seoul, Korea) Pub Date : 2022-09-01 DOI: 10.3988/jcn.2022.18.5.553
Kyung Wook Kang, Yong Won Cho, Sang Kun Lee, Ki-Young Jung, Ji Hyun Kim, Dong Wook Kim, Sang-Ahm Lee, Seung Bong Hong, In-Seop Na, So-Hyun Lee, Won-Ki Baek, Seok-Yong Choi, Myeong-Kyu Kim
{"title":"Multidimensional Early Prediction Score for Drug-Resistant Epilepsy.","authors":"Kyung Wook Kang,&nbsp;Yong Won Cho,&nbsp;Sang Kun Lee,&nbsp;Ki-Young Jung,&nbsp;Ji Hyun Kim,&nbsp;Dong Wook Kim,&nbsp;Sang-Ahm Lee,&nbsp;Seung Bong Hong,&nbsp;In-Seop Na,&nbsp;So-Hyun Lee,&nbsp;Won-Ki Baek,&nbsp;Seok-Yong Choi,&nbsp;Myeong-Kyu Kim","doi":"10.3988/jcn.2022.18.5.553","DOIUrl":"https://doi.org/10.3988/jcn.2022.18.5.553","url":null,"abstract":"<p><strong>Background and purpose: </strong>Achieving favorable postoperative outcomes in patients with drug-resistant epilepsy (DRE) requires early referrals for preoperative examinations. The purpose of this study was to investigate the possibility of a user-friendly early DRE prediction model that is easy for nonexperts to utilize.</p><p><strong>Methods: </strong>A two-step genotype analysis was performed, by applying 1) whole-exome sequencing (WES) to the initial test set (<i>n</i>=243) and 2) target sequencing to the validation set (<i>n</i>=311). Based on a multicenter case-control study design using the WES data set, 11 genetic and 2 clinical predictors were selected to develop the DRE risk prediction model. The early prediction scores for DRE (EPS-DRE) was calculated for each group of the selected genetic predictors (EPS-DRE<sub>gen</sub>), clinical predictors (EPS-DRE<sub>cln</sub>), and two types of predictor mix (EPS-DRE<sub>mix</sub>) in both the initial test set and the validation set.</p><p><strong>Results: </strong>The multidimensional EPS-DRE<sub>mix</sub> of the predictor mix group provided a better match to the outcome data than did the unidimensional EPS-DRE<sub>gen</sub> or EPS-DRE<sub>cln</sub>. Unlike previous studies, the EPS-DRE<sub>mix</sub> model was developed using only 11 genetic and 2 clinical predictors, but it exhibited good discrimination ability in distinguishing DRE from drug-responsive epilepsy. These results were verified using an unrelated validation set.</p><p><strong>Conclusions: </strong>Our results suggest that EPS-DRE<sub>mix</sub> has good performance in early DRE prediction and is a user-friendly tool that is easy to apply in real clinical trials, especially by nonexperts who do not have detailed knowledge or equipment for assessing DRE. Further studies are needed to improve the performance of the EPS-DRE<sub>mix</sub> model.</p>","PeriodicalId":324902,"journal":{"name":"Journal of Clinical Neurology (Seoul, Korea)","volume":" ","pages":"553-561"},"PeriodicalIF":3.1,"publicationDate":"2022-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/5e/27/jcn-18-553.PMC9444554.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40350820","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
Comments on "A Case of MELAS With the m.3243A>G Variant of the MT-TL1 Gene Mimicking Acute Intermittent Porphyria". “1例伴有急性间歇性卟啉症的MT-TL1基因m.3243A>G变异的MELAS”
IF 3.1
Journal of Clinical Neurology (Seoul, Korea) Pub Date : 2022-09-01 DOI: 10.3988/jcn.2022.18.5.597
Josef Finsterer
{"title":"Comments on \"A Case of MELAS With the m.3243A>G Variant of the MT-TL1 Gene Mimicking Acute Intermittent Porphyria\".","authors":"Josef Finsterer","doi":"10.3988/jcn.2022.18.5.597","DOIUrl":"https://doi.org/10.3988/jcn.2022.18.5.597","url":null,"abstract":"a 23-year-old male with mitochondrial encephalopathy, lactic acidosis, and stroke-like episode (MELAS) syndrome due to the m.3243A>G variant. The patient manifested phenotypically with epilepsy, quadriparesis, wasting, generalized hypotonia, bilateral facial palsy, dysarthria, dysphagia, anemia, lactic ac-idosis, polyneuropathy, and abdominal pain. The patient was initially suspected as having acute intermittent porphyria (AIP), but the 4.5-h sunlight test produced normal findings. The study is attractive, but it","PeriodicalId":324902,"journal":{"name":"Journal of Clinical Neurology (Seoul, Korea)","volume":" ","pages":"597-598"},"PeriodicalIF":3.1,"publicationDate":"2022-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/75/8f/jcn-18-597.PMC9444556.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40351737","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
Cardiac and Pulmonary Management Status of Duchenne Muscular Dystrophy in South Korea Based on Data From the National Health Insurance Database. 基于国民健康保险数据库数据的韩国杜氏肌萎缩症患者心肺管理状况
IF 3.1
Journal of Clinical Neurology (Seoul, Korea) Pub Date : 2022-09-01 DOI: 10.3988/jcn.2022.18.5.522
Jin A Yoon, Ho Eun Park, Jinmi Kim, Jungmin Son, Yong Beom Shin
{"title":"Cardiac and Pulmonary Management Status of Duchenne Muscular Dystrophy in South Korea Based on Data From the National Health Insurance Database.","authors":"Jin A Yoon,&nbsp;Ho Eun Park,&nbsp;Jinmi Kim,&nbsp;Jungmin Son,&nbsp;Yong Beom Shin","doi":"10.3988/jcn.2022.18.5.522","DOIUrl":"https://doi.org/10.3988/jcn.2022.18.5.522","url":null,"abstract":"<p><strong>Background and purpose: </strong>The purpose of this study was to determine the cardiac and pulmonary management status of patients with Duchenne muscular dystrophy (DMD) in South Korea based on the Korean National Health Insurance database.</p><p><strong>Methods: </strong>This study used data of patients with code G71.0 in the National Health Information database, and also those with the special case of code V012. Cardiac function was assessed based on whether echocardiography and 24-h electrocardiography were performed, as well as the frequency of these investigations. Furthermore, information on the use of angiotensin-converting enzyme inhibitors (ACEi), angiotensin II receptor blockers (ARB), and beta blockers (BB) in the drug benefit list was checked. Medical charge records were also checked regarding the prescription of respiratory functional assessments and treatments.</p><p><strong>Results: </strong>The diagnostic criteria were met by 479 patients, with 41% of these patients receiving a cardiac evaluation, 29.8% being prescribed ACEi at 14.4±3.7 years of age, 59.5% undergoing pulmonary function tests, and 42.1% received pulmonary rehabilitation. The age at receiving ventilator support was 19.4±2.7 years. The frequency of cardiac and respiratory function tests increased with age, but the interval between tests was longer than the recent DMD care recommendations. The trend of taking ACEi, ARB, and BB for cardiac management in South Korea did not change during the study analysis period.</p><p><strong>Conclusions: </strong>The findings of this study will contribute to recognizing the current status and the importance of applying an anticipatory approach to cardiopulmonary function in DMD patients.</p>","PeriodicalId":324902,"journal":{"name":"Journal of Clinical Neurology (Seoul, Korea)","volume":" ","pages":"522-528"},"PeriodicalIF":3.1,"publicationDate":"2022-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/8e/2c/jcn-18-522.PMC9444560.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40350817","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
Variations of Resting-State EEG-Based Functional Networks in Brain Maturation From Early Childhood to Adolescence. 静息状态下基于脑电图的功能网络在儿童早期到青春期大脑成熟过程中的变化。
IF 3.1
Journal of Clinical Neurology (Seoul, Korea) Pub Date : 2022-09-01 DOI: 10.3988/jcn.2022.18.5.581
Yoon Gi Chung, Yonghoon Jeon, Ryeo Gyeong Kim, Anna Cho, Hunmin Kim, Hee Hwang, Jieun Choi, Ki Joong Kim
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引用次数: 3
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