Neurology and Clinical Neuroscience最新文献

{"title":"Non‐ergot dopamine agonist therapy for Parkinson's disease in Japan: A claims database analysis","authors":"K. Wada-isoe, Yoshio Tsuboi, Hiroyuki Kondo, Yoshitsugu Kojima, T. Takeshima, K. Iwasaki","doi":"10.1111/ncn3.12689","DOIUrl":"https://doi.org/10.1111/ncn3.12689","url":null,"abstract":"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.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":"11 1","pages":"63 - 71"},"PeriodicalIF":0.4,"publicationDate":"2023-01-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45670485","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}

{"title":"A genotype–phenotype description in two Indian patients: Broadening the spectrum in VRK1‐related complex motor disorders","authors":"Valaparambil Karthika Ajit, Sruthi Sivaraman Nair, Sethu Madhavan Anjali, Pravi Vidyadharan, Madhusoodanan Urulangodi, R. Menon, Soumya Sundaram","doi":"10.1111/ncn3.12694","DOIUrl":"https://doi.org/10.1111/ncn3.12694","url":null,"abstract":"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.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":"11 1","pages":"93 - 96"},"PeriodicalIF":0.4,"publicationDate":"2023-01-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46742962","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}

{"title":"Issue Information","authors":"","doi":"10.1111/ncn3.12616","DOIUrl":"https://doi.org/10.1111/ncn3.12616","url":null,"abstract":"","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":" ","pages":""},"PeriodicalIF":0.4,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46818695","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}

{"title":"Steroid administration for post-COVID-19 Parkinsonism: A case report.","authors":"Witoon Mitarnun, Metha Apiwattanakul, Thanatchanan Thodthasri, Praewa Tantisungvarakoon, Wilasinee Pangwong","doi":"10.1111/ncn3.12679","DOIUrl":"10.1111/ncn3.12679","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":"11 1","pages":"49-51"},"PeriodicalIF":0.4,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9874587/pdf/NCN3-11-49.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10584263","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}

{"title":"Neurological manifestations in SARS-CoV-2 infection: A single-center cross-sectional study in Malaysia.","authors":"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","doi":"10.1111/ncn3.12677","DOIUrl":"10.1111/ncn3.12677","url":null,"abstract":"<p><strong>Background: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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 (<i>p</i> = 0.033), symptoms of stroke [limb weakness (<i>p</i> < 0.001), slurred speech (<i>p</i> < 0.001)]; and encephalitis or encephalopathy [confusion (<i>p</i> < 0.001), forgetfulness (<i>p</i> = 0.006) and seizure (<i>p</i> = 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, <i>p</i> = 0.047). Anosmia and dysgeusia were less associated with neurological complications (adjusted OR = 0.22; 95% CI: 0.05, 0.96, <i>p</i> = 0.044). The odds of neurological complications were increased by 18% in patients with leukocytosis (adjusted OR = 1.18, 95% CI: 1.003, <i>p</i> = 0.0460).</p><p><strong>Conclusions: </strong>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.</p>","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":"11 1","pages":"17-26"},"PeriodicalIF":0.4,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9874463/pdf/NCN3-11-17.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10584265","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}

{"title":"Age‐related changes in autonomic response: A pupillary light reflex and heart rate variability study","authors":"Roberta Bril Paroz, S. Barak Lanciano, Alex Omelchenko, U. Nussinovitch","doi":"10.1111/ncn3.12693","DOIUrl":"https://doi.org/10.1111/ncn3.12693","url":null,"abstract":"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.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":"11 1","pages":"72 - 78"},"PeriodicalIF":0.4,"publicationDate":"2022-12-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46800372","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}

{"title":"Clinical development of novel therapies for Duchenne muscular dystrophy—Current and future","authors":"Tsukasa Tominari, Y. Aoki","doi":"10.1111/ncn3.12691","DOIUrl":"https://doi.org/10.1111/ncn3.12691","url":null,"abstract":"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.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":"11 1","pages":"111 - 118"},"PeriodicalIF":0.4,"publicationDate":"2022-12-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46296122","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}

{"title":"Two recovered cases of large pontine hematoma","authors":"Ajaya Kumar Ayyappan Unnithan","doi":"10.1111/ncn3.12692","DOIUrl":"https://doi.org/10.1111/ncn3.12692","url":null,"abstract":"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.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":"11 1","pages":"90 - 92"},"PeriodicalIF":0.4,"publicationDate":"2022-12-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47062578","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}

{"title":"Complete occlusion of vertebral artery and medial medullary infarction","authors":"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","doi":"10.1111/ncn3.12690","DOIUrl":"https://doi.org/10.1111/ncn3.12690","url":null,"abstract":"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","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":"11 1","pages":"107 - 108"},"PeriodicalIF":0.4,"publicationDate":"2022-12-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42524886","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}

{"title":"A fact‐finding survey of medical care provided to neuromuscular disease patients at the National Center of Neurology and Psychiatry in Japan","authors":"A. Hanai, K. Yorimoto, Ryo Ohkubo, T. Tsukamoto, K. Mizuno, Yuji Takahashi","doi":"10.1111/ncn3.12688","DOIUrl":"https://doi.org/10.1111/ncn3.12688","url":null,"abstract":"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.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":"11 1","pages":"32 - 40"},"PeriodicalIF":0.4,"publicationDate":"2022-12-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45364907","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}