Neurology and Clinical Neuroscience最新文献

Systemic association of myelin oligodendrocyte glycoprotein antibody disease: A systematic review of literature 髓鞘少突胶质细胞糖蛋白抗体病的系统关联性：文献系统回顾

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Neurology and Clinical Neuroscience Pub Date : 2024-01-09 DOI: 10.1111/ncn3.12790

Camelia Porey, B. Jaiswal

引用次数: 0

Reduction rate of anti‐acetylcholine receptor antibody titer levels is an early prognostic indicator for myasthenia gravis 抗乙酰胆碱受体抗体滴度水平的降低率是肌无力的早期预后指标

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Neurology and Clinical Neuroscience Pub Date : 2024-01-08 DOI: 10.1111/ncn3.12793

Yasuhiro Hamada, Kazushi Deguchi, Keita Takaba, Rie Kawakita, Tadayuki Takata, A. Morishita, H. Kobara, Tsutomu Masaki

{"title":"Reduction rate of anti‐acetylcholine receptor antibody titer levels is an early prognostic indicator for myasthenia gravis","authors":"Yasuhiro Hamada, Kazushi Deguchi, Keita Takaba, Rie Kawakita, Tadayuki Takata, A. Morishita, H. Kobara, Tsutomu Masaki","doi":"10.1111/ncn3.12793","DOIUrl":"https://doi.org/10.1111/ncn3.12793","url":null,"abstract":"A realistic treatment goal for myasthenia gravis (MG) is achieving minimal manifestations or better status with prednisolone at ≤5 mg/day (MM‐or‐better‐5 mg), considering a patient's health‐related quality of life. Prognosis prediction during the early phases of immunotherapies might be critical for determining subsequent treatment strategies; however, the appropriate biomarkers remain unknown.This study aimed to clarify whether the reduction rate of anti‐acetylcholine receptor antibody (RR‐AChR Ab) titer levels is a useful biomarker for predicting MM‐or‐better‐5 mg achievement.We retrospectively investigated patients with MG and AChR Abs who received immunotherapy for the first time. The RR‐AChR Ab titer levels were calculated in the early (within 30 days), middle (31–60 days), and late (61–100 days) periods after starting immunotherapies. A receiver operating characteristic (ROC) curve was generated to determine an appropriate cutoff value for RR‐AChR Abs to achieve an MM‐or‐better‐5 mg.Of 53 patients, 24 (45%) achieved MM‐or‐better‐5 mg after 1 year. For the early period, the RR‐AChR Ab cutoff value to predict MM‐or‐better‐5 mg was 1.68%/day with an area under the curve (AUC) of 0.75 (sensitivity, 85%; specificity, 70%). However, the middle and late posttreatment AUC values did not predict MM‐or‐better‐5 mg achievement.The RR‐AChR Ab might be an appropriate prognostic biomarker during the early period of MM‐or‐better‐5 mg achievement. In the era of early fast‐acting treatment strategies, the RR‐AChR Ab trend after starting immunotherapies may guide the subsequent treatment choices.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":"42 18","pages":""},"PeriodicalIF":0.4,"publicationDate":"2024-01-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139448081","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: 0

Focal 18F‐fluorodeoxyglucose uptake in spinal dural arteriovenous fistula: A report of two cases 脊髓硬膜动静脉瘘的局灶性 18F - 氟脱氧葡萄糖摄取：两例病例报告

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Neurology and Clinical Neuroscience Pub Date : 2024-01-04 DOI: 10.1111/ncn3.12794

Hitoshi Hayashida, K. Masaki, H. Ogata, Takahiro Yamaguchi, Koji Tanaka, Koichi Arimura, Yasuhiro Maruoka, K. Kikuchi, Osamu Togao, Ryo Yamasaki, N. Isobe

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Efficacy and safety of modified Atkins diet therapy for drug‐resistant epilepsy in children and adults: A systematic review and meta‐analysis 改良阿特金斯饮食疗法治疗儿童和成人耐药性癫痫的有效性和安全性：系统回顾和荟萃分析

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Neurology and Clinical Neuroscience Pub Date : 2023-12-27 DOI: 10.1111/ncn3.12791

Nobel Budiputra, C. L. Budiputri, Mary Christina Elsa

{"title":"Efficacy and safety of modified Atkins diet therapy for drug‐resistant epilepsy in children and adults: A systematic review and meta‐analysis","authors":"Nobel Budiputra, C. L. Budiputri, Mary Christina Elsa","doi":"10.1111/ncn3.12791","DOIUrl":"https://doi.org/10.1111/ncn3.12791","url":null,"abstract":"Diet‐based treatments have been proposed for drug‐resistant epilepsy (DRE), and the Modified Atkins Diet (MAD) is an alternative. This study aimed to assess the efficacy and safety of MAD as an adjunctive therapy for reducing seizures in patients with drug‐resistant epilepsy.A literature search was done on six databases. Randomized controlled trial (RCT) studies were included, comparing DRE patients of all ages on standard antiseizure medications (ASD) who received MAD compared to no‐dietary or other dietary interventions. The outcomes are the seizure frequency reduction and the adverse events. Cochrane risk‐of‐bias tool and GRADE were used to assess study quality and the overall certainty of evidence. The effect size of the efficacy of MAD was computed as risk ratio (95% CI). Subgroup analysis based on each type of comparator was done.Ten RCT studies (905 participants) were included, comparing MAD to no dietary intervention, ketogenic diet (KD), and low glycemic index treatment (LGIT). Overall, MAD is constantly and significantly more efficacious than no dietary intervention in reducing seizures ≥50% (RR 7.90, 95% CI 4.59–13.62), ≥90% (RR 4.61, 95% CI 2.05–10.36), and inducing complete seizure‐free (RR 7.79, 95% 2.61–23.22) with high certainty. Other dietary therapies and MAD did not differ significantly from one another in terms of efficacy. The risk of adverse events in MAD is insignificantly higher than in others (RR 1.19, 95% 0.92–1.55).The Modified Atkins Diet has better seizure reduction than no diet intervention but is comparable to other dietary interventions with a negligible increased risk of adverse events.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":"59 10","pages":""},"PeriodicalIF":0.4,"publicationDate":"2023-12-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139154295","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: 0

A rare presentation of unilateral posterior reversible encephalopathy syndrome following a trivial head trauma 头部轻微外伤后出现单侧后可逆性脑病综合征的罕见病例

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Neurology and Clinical Neuroscience Pub Date : 2023-12-27 DOI: 10.1111/ncn3.12792

Bonny Sen, Jasodhara Chaudhuri, Tapasaya Nanda, Sourav Panda, Manamita Mandal, Sourav Nanda

引用次数: 0

Long‐term safety of oral edaravone in Japanese patients with amyotrophic lateral sclerosis: Sub‐analysis of a global, open‐label, phase 3 study 日本肌萎缩性脊髓侧索硬化症患者口服依达拉奉的长期安全性：一项全球性开放标签 3 期研究的子分析

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Neurology and Clinical Neuroscience Pub Date : 2023-12-11 DOI: 10.1111/ncn3.12789

Xuezhu Sun, M. Hirai, Tomoaki Tezuka, Shinya Hirota, Satoshi Yuki

{"title":"Long‐term safety of oral edaravone in Japanese patients with amyotrophic lateral sclerosis: Sub‐analysis of a global, open‐label, phase 3 study","authors":"Xuezhu Sun, M. Hirai, Tomoaki Tezuka, Shinya Hirota, Satoshi Yuki","doi":"10.1111/ncn3.12789","DOIUrl":"https://doi.org/10.1111/ncn3.12789","url":null,"abstract":"Patients with amyotrophic lateral sclerosis (ALS) experience a slower rate of physical function decline when treated with intravenous edaravone. The oral suspension formulation of edaravone (105 mg) has a similar pharmacokinetic profile to intravenous edaravone (60 mg/60 min). The long‐term safety of oral edaravone in Japanese patients with ALS has not been reported. Therefore, a sub‐analysis of Japanese patients in a global phase 3 study (NCT04165824) was conducted to evaluate the safety and tolerability of oral edaravone in Japanese patients with ALS.This study was a global, open‐label, phase 3 study to evaluate the long‐term safety and tolerability of oral edaravone in patients with ALS. Patients with ALS received oral edaravone (105 mg/day) for 48 weeks. Adverse events in Japanese patients were assessed at week 48.Among the 185 patients enrolled globally, 65 patients were enrolled in Japan (mean age, 59.3 years; mean disease duration, 1.5 years). Most patients experienced treatment‐emergent adverse events (TEAEs) (84.6%), and the most common TEAEs by week 48 were constipation (15.4%), insomnia (12.3%), and back pain (10.8%). Two serious TEAEs were reported: atrial fibrillation and pleural effusion (both n = 1). Three adverse drug reactions (ADRs) were reported: diarrhea, abnormal hepatic function, and fatigue (all n = 1). There were no serious ADRs or TEAEs/ADRs that led to study drug discontinuation.Oral edaravone had a similar safety profile to intravenous edaravone in Japanese patients, and good tolerability over 48 weeks. No new safety concerns were observed in this population.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":"11 1","pages":""},"PeriodicalIF":0.4,"publicationDate":"2023-12-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138980772","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: 0

Recent topics of spinocerebellar ataxia type 31 有关脊髓小脑共济失调 31 型的最新话题

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Neurology and Clinical Neuroscience Pub Date : 2023-12-06 DOI: 10.1111/ncn3.12788

Kinya Ishikawa

{"title":"Recent topics of spinocerebellar ataxia type 31","authors":"Kinya Ishikawa","doi":"10.1111/ncn3.12788","DOIUrl":"https://doi.org/10.1111/ncn3.12788","url":null,"abstract":"Spinocerebellar ataxia type 31 (SCA31) is an autosomal‐dominant neurodegenerative condition caused by a 2.5–3.8 kb‐long complex repeat containing (TGGAA/TTCCA)n in an intron shared by two genes called brain expressed, associated with Nedd4 (BEAN1) and thymidine kinase 2 (TK2) located in the human chromosome 16q22.1. Since BEAN1 and TK2 are transcribed in mutually opposite directions in human brains, two independently transcribed RNAs containing either (UGGAA)n or (UUCCA)n are likely to associate with the pathogenesis of SCA31. Recently, a minor TK2 mRNA isoform called TK2‐EXT was confirmed to be transcribed in human cerebellum, suggesting that (UUCCA)n is indeed expressed. The level of TK2 mRNA and TK2 protein expression levels was both preserved in SCA31 human cerebellum, suggesting that the expression of (UUCCA)n does not affect the expression of TK2, and hence, the function of TK2 seemed to be preserved. On the other hand, the other penta‐nucleotide RNA repeat (UGGAA)n, expressed through BEAN1 transcription, is likely to conform toxicity through forming abnormal RNA structures called RNA foci in the nucleus of expressing cells. In addition, three proteins TDP‐43, FUS, and hnRNPA2/B1 that commonly have a capacity to bind with (UGGAA)n reduced the number of RNA foci, and ameliorated the phenotype brought by (UGGAA)n in Drosophila. A small compound naphthyridine carbamate dimer that binds to (UGGAA)n dampened the (UGGAA)n toxicity in Drosophila, further supporting the idea that (UGGAA)n expressed by BEAN1 is pathogenic. Therefore, a plausible approach to treat SCA31 may be considered by administering agents with a capacity binding to (UGGAA)n.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":"27 3","pages":""},"PeriodicalIF":0.4,"publicationDate":"2023-12-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138595844","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: 0

Association between trigeminal neuralgia and degenerative cervical myelopathy: A cross‐sectional study using US data 三叉神经痛与退行性颈椎病之间的关系：利用美国数据进行的横断面研究

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Neurology and Clinical Neuroscience Pub Date : 2023-11-16 DOI: 10.1111/ncn3.12787

R. Trager, Elainie C. Theodorou, Eric Chun Pu Chu

{"title":"Association between trigeminal neuralgia and degenerative cervical myelopathy: A cross‐sectional study using US data","authors":"R. Trager, Elainie C. Theodorou, Eric Chun Pu Chu","doi":"10.1111/ncn3.12787","DOIUrl":"https://doi.org/10.1111/ncn3.12787","url":null,"abstract":"Limited research has suggested that trigeminal neuralgia (TN), an often‐idiopathic pain disorder affecting the face and head, may arise from compression of the cervical spinal cord due to involvement of the spinal trigeminal tract. We hypothesized that adults with TN would have a greater likelihood of concurrent degenerative cervical myelopathy (DCM) compared to matched adults without TN.We retrieved de‐identified data from a US network (TriNetX, Inc.) including medical records of >113 million patients, with a query date of October 1, 2023, and data spanning the previous 20 years. We created two groups of adults (aged ≥18 years): Those with (1) TN and (2) No TN, excluding individuals with predisposing conditions for TN (e.g., multiple sclerosis, ophthalmic and oral/maxillofacial surgery) and propensity matched for confounders (e.g., age, sex, body mass index, diabetes mellitus, hypertensive diseases, migraine, osteoporosis). We calculated the point prevalence and odds ratio (OR) of DCM with 95% confidence intervals (CI).After matching there were 37,163 patients per group. The mean point prevalence of DCM in the TN group was 0.55% (95% CI: 0.47–0.63%) compared with 0.04% (0.03–0.06%) in the no TN group, yielding an OR of 12.94 (95% CI: 7.78–21.53; p < 0.0001).Adults with TN had more than 12 times greater odds of concurrent DCM compared to those without TN. These findings suggest that DCM may be a risk factor for TN, yet causality should be further examined using case–control or cohort designs.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":"498 1","pages":""},"PeriodicalIF":0.4,"publicationDate":"2023-11-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139266663","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: 0

Sensory neuropathy complicated with de novo diabetes mellitus during levodopa‐carbidopa intestinal gel infusion in a patient with Parkinson's disease: A case report 1例帕金森病患者左旋多巴-卡比多巴肠凝胶输注过程中感觉神经病变并发新生糖尿病

Neurology and Clinical Neuroscience Pub Date : 2023-11-14 DOI: 10.1111/ncn3.12785

Tatsuya Ueno, Masayuki Baba, Rie Haga, Akira Arai, Nobutaka Hattori, Masahiko Tomiyama

引用次数: 0

Factors involved in the one‐year changes in the tracheal diameter of patients with amyotrophic lateral sclerosis undergoing tracheostomy positive pressure ventilation 肌萎缩性侧索硬化症患者气管造口正压通气一年内气管直径变化的相关因素

Neurology and Clinical Neuroscience Pub Date : 2023-11-14 DOI: 10.1111/ncn3.12786

Nobuhiko Shibasaki, Kaoru Konishi, Tetsuo Miyagawa, Takaya Numayama

{"title":"Factors involved in the one‐year changes in the tracheal diameter of patients with amyotrophic lateral sclerosis undergoing tracheostomy positive pressure ventilation","authors":"Nobuhiko Shibasaki, Kaoru Konishi, Tetsuo Miyagawa, Takaya Numayama","doi":"10.1111/ncn3.12786","DOIUrl":"https://doi.org/10.1111/ncn3.12786","url":null,"abstract":"Abstract Background Tracheostomy positive pressure ventilation ( TPPV ) is associated with complications in patients with amyotrophic lateral sclerosis ( ALS ), particularly tracheostomy tube‐related problems. Aim To determine the frequency of patients with ALS who received TPPV that have tracheal enlargement and factors associated with changes in tracheal diameter. Methods We included 43 patients with ALS undergoing TPPV who were admitted at Sayama Neurological Hospital in October 2019. The tracheal diameter at the height of the tracheostomy tube cuff was measured radiographically at specific time points. Tidal volume, inspiratory maximum pressure ( PIP ), dynamic lung compliance (Cdyn), tracheostomy tube cuff data, and patient demographic information and data were also collected. Results The frequency of tracheomegaly was 60.5% at the initial data collection. The differences in tracheal diameter between the first measurement, after 12 months, and between 3 and 12 months were significant. ΔTracheal diameter correlated with TPPV duration, PIP , Cdyn, ΔPIP , ΔCdyn , and tracheal diameter. Multiple regression analysis, with Δtracheal diameter as the objective variable and TPPV duration, Cdyn, and tracheal diameter as explanatory variables, revealed an adjusted R ‐square value of 0.36. Conclusion Tracheomegaly and more enlarged tracheal diameters over time were more frequently observed in patients with ALS receiving TPPV . Furthermore, the trachea dilates over time and the tracheal diameter was related to baseline TPPV time, Cdyn, and tracheal diameter. Patients with shorter baseline TPPV duration, higher baseline Cdyn, and smaller baseline tracheal diameter were more likely to have larger tracheal diameters. Therefore, early prevention is important.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":"13 10","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-11-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134991256","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: 0