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Non‐ergot dopamine agonist therapy for Parkinson's disease in Japan: A claims database analysis 日本帕金森病的非麦角多巴胺激动剂治疗:一项索赔数据库分析
IF 0.4 Q4 CLINICAL NEUROLOGY Pub Date : 2023-01-10 DOI: 10.1111/ncn3.12689
K. Wada-isoe, Yoshio Tsuboi, Hiroyuki Kondo, Yoshitsugu Kojima, T. Takeshima, K. Iwasaki
Levodopa monotherapy and combination therapy with non‐ergot dopamine agonists (DAs; pramipexole, ropinirole, and rotigotine) are commonly used in patients with Parkinson's disease (PD). Changes in prescription preferences for each DA were reported previously; however, information regarding the selection of individual DAs is insufficient.
左旋多巴单药及与非麦角多巴胺激动剂(DAs)联合治疗;普拉克索、罗匹尼罗和罗替戈汀)常用于帕金森病(PD)患者。以前曾报道过每种DA的处方偏好变化;然而,关于个别da的选择的信息是不足的。
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引用次数: 0
A genotype–phenotype description in two Indian patients: Broadening the spectrum in VRK1‐related complex motor disorders 两名印度患者的基因型-表型描述:拓宽了VRK1相关复杂运动障碍的频谱
IF 0.4 Q4 CLINICAL NEUROLOGY Pub Date : 2023-01-10 DOI: 10.1111/ncn3.12694
Valaparambil Karthika Ajit, Sruthi Sivaraman Nair, Sethu Madhavan Anjali, Pravi Vidyadharan, Madhusoodanan Urulangodi, R. Menon, Soumya Sundaram
VRK1 (vaccinia‐related kinase 1) was initially reported to be associated with spinal muscular atrophy–pontocerebellar hypoplasia (SMA‐PCH). Congenital or infantile‐onset progressive sensory‐motor neuropathy with microcephaly, adult‐onset distal SMA, and adult‐onset motor neuron disease are the other phenotypes described recently with VRK1. Since VRK1‐related complex motor disorders other than PCH is rare, we aim to depict the diverse clinical phenotypes and the genotypes of two patients with VRK1 variants from India. Proband‐1 is a 7‐year‐old girl who presented with distal muscle weakness and wasting of upper and lower limbs with brisk deep tendon reflexes (DTR), mild intellectual disability, and behavioral problems. She had a homozygous c.1159 + 1G > A pathogenic variant in VRK1, inherited from parents. Proband‐2 is a 25‐year‐old adopted male with sensory‐motor neuropathy and brisk DTR. He had a homozygous c.7C > T (p.R3C) missense variant of uncertain significance in VRK1 predicted to be damaging by multiple computational tools.
VRK1(牛痘相关激酶1)最初被报道与脊髓性肌萎缩-桥小脑发育不全(SMA - PCH)相关。先天性或婴儿期发病的进行性感觉运动神经病变伴小头畸形,成年期发病的远端SMA和成年期发病的运动神经元疾病是最近用VRK1描述的其他表型。由于除了PCH之外,VRK1相关的复杂运动障碍很少见,我们的目标是描述来自印度的两名VRK1变异患者的不同临床表型和基因型。Proband - 1是一名7岁女孩,表现为远端肌肉无力,上肢和下肢萎缩,伴有深肌腱反射(DTR),轻度智力残疾和行为问题。她有一个纯合子c.1159 + 1G > VRK1致病变异,遗传自父母。Proband - 2是一名25岁被收养的男性,患有感觉-运动神经病变和快速DTR。他在VRK1中有一个不确定意义的纯合子c.7C . > T (p.R3C)错义变异,多种计算工具预测该变异具有破坏性。
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引用次数: 0
Issue Information 问题信息
IF 0.4 Q4 CLINICAL NEUROLOGY Pub Date : 2023-01-01 DOI: 10.1111/ncn3.12616
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引用次数: 0
Steroid administration for post-COVID-19 Parkinsonism: A case report. 类固醇治疗 COVID-19 后帕金森症:病例报告。
IF 0.4 Q4 CLINICAL NEUROLOGY Pub Date : 2023-01-01 Epub Date: 2022-10-31 DOI: 10.1111/ncn3.12679
Witoon Mitarnun, Metha Apiwattanakul, Thanatchanan Thodthasri, Praewa Tantisungvarakoon, Wilasinee Pangwong

A 45-year-old man developed parkinsonism 3 weeks after being diagnosed with mild COVID-19. Levodopa and benserazide failed to improve his symptoms, necessitating ropinirole, and steroid treatment, which included a 5-day course of methylprednisolone followed by a 3-month oral prednisolone taper. One month after initiating steroid treatment, his symptoms improved significantly.

一名 45 岁男子在被诊断为轻度 COVID-19 后 3 周出现帕金森氏症。左旋多巴和苄丝肼未能改善他的症状,因此需要使用罗匹尼罗和类固醇治疗,其中包括一个为期 5 天的甲基强的松龙疗程,然后是为期 3 个月的口服强的松龙减量治疗。开始类固醇治疗一个月后,他的症状明显好转。
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引用次数: 0
Neurological manifestations in SARS-CoV-2 infection: A single-center cross-sectional study in Malaysia. SARS-CoV-2 感染的神经系统表现:马来西亚单中心横断面研究。
IF 0.4 Q4 CLINICAL NEUROLOGY Pub Date : 2023-01-01 Epub Date: 2022-11-14 DOI: 10.1111/ncn3.12677
Hui Jan Tan, Cheon Han Goh, Ching Soong Khoo, Chen Fei Ng, Juen Kiem Tan, Wan Asyraf Wan Zaidi, Zhe Kang Law, Muhamad Danial Zulkifli, Shahrul Azmin Md Rani, Wan Nur Nafisah Wan Yahya, Rabani Remli, Norlinah Mohamed Ibrahim, Rozita Hod, Shahizon Azura Mohamed Mukari, Aida-Widure Mustapha Mohd Mustapha, Najma Kori, Petrick Periyasamy

Background: Neurological involvement associated with SARS-CoV-2 infection has been reported from different regions of the world. However, data from South East Asia are scarce. We described the neurological manifestations and their associated factors among the hospitalized COVID-19 patients from an academic tertiary hospital in Malaysia.

Methods: A cross-sectional observational study of hospitalized COVID-19 patients was conducted. The neurological manifestations were divided into the self-reported central nervous system (CNS) symptoms, stroke associated symptoms, symptoms of encephalitis or encephalopathy and specific neurological complications. Multiple logistic regression was performed using demographic and clinical variables to determine the factors associated with outcome.

Results: Of 156 hospitalized COVID-19 patients with mean age of 55.88 ± 6.11 (SD) years, 23.7% developed neurological complications, which included stroke, encephalitis and encephalopathy. Patients with neurological complications were more likely to have diabetes mellitus (p = 0.033), symptoms of stroke [limb weakness (p < 0.001), slurred speech (p < 0.001)]; and encephalitis or encephalopathy [confusion (p < 0.001), forgetfulness (p = 0.006) and seizure (p = 0.019)]. Unvaccinated patients had a 4.25-fold increased risk of having neurological complications (adjusted OR = 4.25; 95% CI: 1.02, 17.71, p = 0.047). Anosmia and dysgeusia were less associated with neurological complications (adjusted OR = 0.22; 95% CI: 0.05, 0.96, p = 0.044). The odds of neurological complications were increased by 18% in patients with leukocytosis (adjusted OR = 1.18, 95% CI: 1.003, p = 0.0460).

Conclusions: Stroke, encephalitis and encephalopathy were the common neurological complications from our study. Diabetes mellitus, presence of symptoms of stroke, symptoms of encephalitis or encephalopathy, leukocytosis, and being unvaccinated against COVID-19 were the associated risk factors of developing neurological complications.

背景:世界不同地区都有感染 SARS-CoV-2 导致神经系统受累的报道。然而,来自东南亚的数据却很少。我们描述了马来西亚一家学术性三甲医院的 COVID-19 住院患者的神经系统表现及其相关因素:我们对住院的 COVID-19 患者进行了横断面观察研究。神经系统表现分为自我报告的中枢神经系统(CNS)症状、中风相关症状、脑炎或脑病症状以及特定的神经系统并发症。利用人口统计学和临床变量进行多元逻辑回归,以确定与结果相关的因素:156 名平均年龄为 55.88 ± 6.11 (SD) 岁的 COVID-19 住院患者中,23.7% 出现了神经系统并发症,包括中风、脑炎和脑病。出现神经系统并发症的患者更有可能患有糖尿病(p = 0.033)、中风症状[四肢无力(p p p = 0.006)和癫痫发作(p = 0.019)]。未接种疫苗的患者出现神经系统并发症的风险增加了 4.25 倍(调整后 OR = 4.25;95% CI:1.02,17.71,p = 0.047)。嗅觉障碍和味觉障碍与神经系统并发症的关系不大(调整后 OR = 0.22;95% CI:0.05,0.96,p = 0.044)。白细胞增多的患者出现神经系统并发症的几率增加了18%(调整后OR = 1.18,95% CI:1.003,p = 0.0460):结论:中风、脑炎和脑病是本研究中常见的神经系统并发症。糖尿病、中风症状、脑炎或脑病症状、白细胞增多以及未接种 COVID-19 疫苗是发生神经系统并发症的相关风险因素。
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引用次数: 0
Age‐related changes in autonomic response: A pupillary light reflex and heart rate variability study 自主神经反应的年龄相关变化:瞳孔光反射和心率变异性研究
IF 0.4 Q4 CLINICAL NEUROLOGY Pub Date : 2022-12-28 DOI: 10.1111/ncn3.12693
Roberta Bril Paroz, S. Barak Lanciano, Alex Omelchenko, U. Nussinovitch
Autonomic nervous system (ANS) dysfunction has been implicated with cardiovascular diseases, sudden‐cardiac‐death, and aging. The pupillary light reflex (PLR) and heart rate variability (HRV) are noninvasive tools of autonomic evaluation. However, the former remains underutilized in clinical practice.
自主神经系统(ANS)功能障碍与心血管疾病、心脏性猝死和衰老有关。瞳孔光反射(PLR)和心率变异性(HRV)是自主神经评估的无创工具。然而,前者在临床实践中仍未得到充分利用。
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引用次数: 1
Clinical development of novel therapies for Duchenne muscular dystrophy—Current and future Duchenne肌营养不良新疗法的临床进展——当前和未来
IF 0.4 Q4 CLINICAL NEUROLOGY Pub Date : 2022-12-28 DOI: 10.1111/ncn3.12691
Tsukasa Tominari, Y. Aoki
Duchenne muscular dystrophy (DMD), the most common muscle degenerative disease, is an X‐linked genetic disorder caused by the loss or reduction of dystrophin protein, resulting in progressive muscle wasting, and involving skeletal, cardiac, and respiratory muscles. There is currently no cure for DMD, and an anti‐inflammatory steroid is the conventional treatment to delay disease progression. Recently, several therapeutic approaches have been developed to improve patient quality of life and even to treat the underlying cause of the disease. These approaches include exon‐skipping, stop‐codon read‐through, vector‐mediated gene therapy, and stem cell transplantation. Exon‐skipping is one of the most promising techniques, and four exon‐skipping drugs have received approval, including NS‐065/NCNP‐01 (viltolarsen) in Japan and the USA. The read‐through drug, Ataluren, has received approval in the EU. Vector‐mediated therapy and cell transplantation are also attractive approaches, and currently, clinical trials are ongoing for some drugs. Furthermore, several studies have developed innovative approaches for DMD treatment, such as multiple exon skipping, gene editing using the CRISPR/Cas9 system, and mesenchymal stromal cell (MSC)‐ or inducible pluripotent stem cell (iPSC)‐based cell transplantation. In this review, we summarize current therapeutic approaches for DMD treatment.
杜氏肌营养不良症(DMD)是最常见的肌肉退行性疾病,是一种由肌营养不良蛋白缺失或减少引起的X连锁遗传疾病,导致进行性肌肉萎缩,涉及骨骼肌、心肌和呼吸肌。目前还没有治愈DMD的方法,抗炎类固醇是延缓疾病进展的常规治疗方法。最近,已经开发了几种治疗方法来改善患者的生活质量,甚至治疗疾病的根本原因。这些方法包括外显子跳过、停止密码子读取、载体介导的基因治疗和干细胞移植。外显子跳跃是最有前途的技术之一,目前已有四种外显子跳跃药物获得批准,包括日本和美国的NS - 065/NCNP - 01 (viltolarsen)。通读药物Ataluren已在欧盟获得批准。载体介导的治疗和细胞移植也是很有吸引力的方法,目前,一些药物的临床试验正在进行中。此外,一些研究已经开发了治疗DMD的创新方法,例如多外显子跳变、使用CRISPR/Cas9系统进行基因编辑以及基于间充质基质细胞(MSC)或诱导多能干细胞(iPSC)的细胞移植。在这篇综述中,我们总结了目前治疗DMD的方法。
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引用次数: 1
Two recovered cases of large pontine hematoma 大脑桥血肿痊愈2例
IF 0.4 Q4 CLINICAL NEUROLOGY Pub Date : 2022-12-28 DOI: 10.1111/ncn3.12692
Ajaya Kumar Ayyappan Unnithan
The comatose patients with pontine hemorrhage of diameter greater than 2 cm have poor prognosis. A 59‐year‐old male was brought with Glasgow coma scale (GCS) score of E2M5V2. He was mechanically ventilated. Magnetic resonance imaging (MRI) showed a pontine hematoma, of size 3 cm. He improved. Glasgow outcome scale (GOS) score was five. A 57‐year‐old male was brought with GCS score E1M3V1. He was ventilated. MRI scan showed a pontine hematoma of size 2.8 cm. His GOS score was three at 6 months. This is a report of two survived cases of large pontine hematoma with poor prognostic factors. This is the first report of good recovery with large pontine hematoma to GOS 5.
桥脑出血直径大于2cm的昏迷患者预后较差。一名59岁男性的格拉斯哥昏迷量表(GCS)评分为E2M5V2。他进行了机械通气。核磁共振成像显示桥脑血肿,大小为3厘米,病情有所好转。格拉斯哥结果量表(GOS)评分为5分。一名57岁男性的GCS评分为E1M3V1。他进行了通风。MRI扫描显示桥脑血肿大小为2.8厘米。他的GOS评分为3分,为6分 月。这是两例存活的桥脑大血肿的报告,预后因素较差。这是第一例GOS 5桥脑大血肿恢复良好的报告。
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引用次数: 0
Complete occlusion of vertebral artery and medial medullary infarction 椎动脉完全闭塞和内髓梗死
IF 0.4 Q4 CLINICAL NEUROLOGY Pub Date : 2022-12-22 DOI: 10.1111/ncn3.12690
Yuka Nakaya, Koji Hayashi, Asuka Suzuki, M. Ueda, Rei Asano, Yudai Tanaka, Mamiko Sato, H. Hayashi, Toyoaki Miura, K. Hayashi, K. Fujita, Kaori Kawabata, Yasutaka Kobayashi
A 70yearold man, who had previous history with hypertension, intracranial dural arteriovenous fistula, and cerebral infarct, developed left hemidysesthesia and left conjugate deviation, and recovered completely within quarterhour. On three days after initial onset, he developed recurrence of these symptoms, neck pain, and vomiting. Neurological findings showed left conjugate deviation, nystagmus, severe hoarseness and dysphagia, mild left hemiplegia, left hemidysesthesia, left bathyhypoesthesia and left hyperalgesia sparing face, and hyperreflexia in bilateral lower limbs. Tongue deviation was not observed. Brain MRA showed right vertebral artery (VA) occlusion and diffusionweighted brain MRI showed hyperintensity in right medial medulla oblongata (Figure 1A,B). Medial medullary infarction
一名70岁的男性,既往有高血压、颅内硬脑膜动静脉瘘和脑梗死病史,出现左半感觉障碍和左共轭偏移,并在四分之一小时内完全康复。初次发病后三天,他出现了这些症状的复发,颈部疼痛和呕吐。神经病学表现为左共轭偏斜、眼球震颤、严重声音嘶哑和吞咽困难、轻度左偏瘫、左半感觉障碍、左深感觉减退和左痛觉过敏保留面部、双侧下肢反射亢进。未观察到舌头偏斜。脑MRA显示右侧椎动脉(VA)闭塞,脑MRI扩散加权显示右侧延髓内侧高信号(图1A,B)。髓内侧梗死
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引用次数: 0
A fact‐finding survey of medical care provided to neuromuscular disease patients at the National Center of Neurology and Psychiatry in Japan 日本国家神经病学和精神病学中心为神经肌肉疾病患者提供医疗服务的实况调查
IF 0.4 Q4 CLINICAL NEUROLOGY Pub Date : 2022-12-09 DOI: 10.1111/ncn3.12688
A. Hanai, K. Yorimoto, Ryo Ohkubo, T. Tsukamoto, K. Mizuno, Yuji Takahashi
The aim of the present study was to conduct a fact‐finding survey of medical care practices that are provided to neuromuscular diseases (NMDs) patients and to identify the placement rate and timing of implementation for each of the diseases.
本研究的目的是对提供给神经肌肉疾病(NMD)患者的医疗护理实践进行实况调查,并确定每种疾病的安置率和实施时间。
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引用次数: 0
期刊
Neurology and Clinical Neuroscience
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