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Effective efgartigimod treatment for severe thymoma‐associated myasthenia gravis experiencing myasthenic crisis: A case report 依加替莫德治疗重症胸腺瘤相关性肌无力危象疗效显著：病例报告
IF 0.4
Neurology and Clinical Neuroscience Pub Date : 2024-03-26 DOI: 10.1111/ncn3.12813
Yukari Morita, Yasushi Osaki, Tomohiro Shogase, Daiji Yoshimoto, Tomomi Terada, Sho Ohtsuru, Kako Yamasaki, Yu Hashimoto, Takuya Matsushita
{"title":"Effective efgartigimod treatment for severe thymoma‐associated myasthenia gravis experiencing myasthenic crisis: A case report","authors":"Yukari Morita, Yasushi Osaki, Tomohiro Shogase, Daiji Yoshimoto, Tomomi Terada, Sho Ohtsuru, Kako Yamasaki, Yu Hashimoto, Takuya Matsushita","doi":"10.1111/ncn3.12813","DOIUrl":"https://doi.org/10.1111/ncn3.12813","url":null,"abstract":"We report the long‐term treatment of a 38‐year‐old man with generalized thymoma‐associated myasthenia gravis who was treated with efgartigimod. He received chemotherapy and oral prednisolone before the thymectomy. However, after initial improvement in symptoms, he deteriorated and needed continuous noninvasive positive‐pressure ventilation, with the highest Myasthenia Gravis‐Activities of Daily Living score of 14. Immunosuppressive therapy and plasma exchange showed limited benefit, and the anti‐acetylcholine receptor antibody titer peaked to 130 nmol/L. Seven cycles of efgartigimod treatment were administered, which improved the symptoms, achieving a Myasthenia Gravis‐Actvities of Daily Living score of 0. The anti‐acetylcholine receptor antibody titer fluctuated until the end of the fifth cycle, thereafter, plateauing at 22 nmol/L. These findings allowed the prednisolone dose to be tapered and the interval between the treatment cycles to be increased.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":null,"pages":null},"PeriodicalIF":0.4,"publicationDate":"2024-03-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140379305","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
Impact of demographic factors, comorbidities, and co‐medication on progression and survival in a large Chinese ALS cohort 中国大量 ALS 患者的人口统计学因素、合并症和联合用药对病情发展和生存期的影响
IF 0.4
Neurology and Clinical Neuroscience Pub Date : 2024-03-25 DOI: 10.1111/ncn3.12802
Nan Hu, Lei Zhang, D. Shen, Xun-zhe Yang, Mingsheng Liu, Liying Cui
{"title":"Impact of demographic factors, comorbidities, and co‐medication on progression and survival in a large Chinese ALS cohort","authors":"Nan Hu, Lei Zhang, D. Shen, Xun-zhe Yang, Mingsheng Liu, Liying Cui","doi":"10.1111/ncn3.12802","DOIUrl":"https://doi.org/10.1111/ncn3.12802","url":null,"abstract":"To clarify the impact of specific risk/protective factors on the disease progression and survival in a large population of Chinese sporadic ALS (sALS) patients.We investigated a cohort of 937 sALS patients prospectively. Uni‐ and multivariate regression analysis were performed to analyze the influence of demographic factors, comorbidities and medication on the progression and survival of ALS.Our results showed younger age of onset (p < 0.05), long diagnostic delay (p < 0.001) and intake of riluzole (p < 0.001) were significantly related to low progression rate and long survival time. History of smoking (p < 0.05) and bulbar onset (p < 0.001) were risk factors for rapid progression and poor prognosis. Baseline ALSFRS‐R score (p < 0.001) and total MRC score (p < 0.05) were positively related to mean survival time of included patients. Neither comorbidities nor intake of antihypertensive drugs, antidiabetics, and statins as dependent variables showed considerable influence on ALS progression or survival.Our study revealed early onset and long diagnostic delay were indicators of slow progression and long survival. Smoking and bulbar onset were risk factors for shorter survival times and abstaining from smoking was beneficial to patients with ALS in improving median survival time. Long‐term intake of riluzole should be recommended for affordable patients.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":null,"pages":null},"PeriodicalIF":0.4,"publicationDate":"2024-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140382447","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
Atypical lesion of the body of fornix in autoimmune glial fibrillary acidic protein astrocytopathy 自身免疫性胶质纤维酸性蛋白星形细胞病的穹窿体非典型病变
IF 0.4
Neurology and Clinical Neuroscience Pub Date : 2024-03-22 DOI: 10.1111/ncn3.12808
Kenta Tominaga, Yu Matsuda, Kazuki Fujita, Michiko Tsutsumiuchi, Akio Kimura, T. Shimohata, Yoshio Sakiyama
{"title":"Atypical lesion of the body of fornix in autoimmune glial fibrillary acidic protein astrocytopathy","authors":"Kenta Tominaga, Yu Matsuda, Kazuki Fujita, Michiko Tsutsumiuchi, Akio Kimura, T. Shimohata, Yoshio Sakiyama","doi":"10.1111/ncn3.12808","DOIUrl":"https://doi.org/10.1111/ncn3.12808","url":null,"abstract":"","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":null,"pages":null},"PeriodicalIF":0.4,"publicationDate":"2024-03-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140212159","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
Gluten sensitivity based on HLA genotyping in patients with epilepsy in a tertiary hospital in Malaysia 马来西亚一家三甲医院癫痫患者基于 HLA 基因分型的麸质敏感性
IF 0.4
Neurology and Clinical Neuroscience Pub Date : 2024-03-20 DOI: 10.1111/ncn3.12806
Shalini Bhaskar, Nur Malina Mat Reffien, L. Teh, Azman Ali Raymond, Mohd Zaki Salleh
{"title":"Gluten sensitivity based on HLA genotyping in patients with epilepsy in a tertiary hospital in Malaysia","authors":"Shalini Bhaskar, Nur Malina Mat Reffien, L. Teh, Azman Ali Raymond, Mohd Zaki Salleh","doi":"10.1111/ncn3.12806","DOIUrl":"https://doi.org/10.1111/ncn3.12806","url":null,"abstract":"Gluten sensitivity (GS) is present in approximately 1% of the Malaysian population (Yap et al., PLoS One, 10, 2015, e0121908). GS is associated with several neurological conditions including epilepsy. Individuals with GS often exhibit a genetic predisposition associated with human leukocyte antigens (HLA) (Cecilio & Bonatto, Arq Bras Cir Dig, 28, 2015, 183). The purpose of this study was to investigate the prevalence of GS based on HLA genotyping in patients with epilepsy (PWE).In total, 50 PWE and 50 controls were recruited. DNA was extracted from the venous blood samples and genotyped for HLA‐DQ2.2, DQ2.5, DQ7 and DQ 8. In this study, GS was diagnosed if any subject showed positive for one or more of the HLA‐DQ alleles. The type of epilepsy and number of antiseizure medication (ASM) used were recorded.Only 2 alleles (HLA‐DQ 2.2 & HLA‐DQ8) were detected among 46 out of 100 subjects. 18 PWE and 19 controls were positive for HLA‐DQ8 (p = 0.836). 9 of the PWE, but no controls were positive for HLA‐DQ 2.2 (p = 0.003). 8 of these 9 PWE who were positive for HLA‐DQ 2.2 were also positive for HLA‐DQ8 (double positive), and these patients required multiple ASM for seizure control (p = 0.006).HLA‐DQ2.2 was seen highly prevalent in PWE. The double positives required more than one ASM for seizure control postulating malabsorption of ASM in these individuals. These findings suggest that HLA‐DQ genotyping may be a valuable additional test in PWE especially in those needing more than one ASM. Prescribing gluten‐free diet (GFD) to PWE with GS may potentially be an additional measure to achieve seizure control.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":null,"pages":null},"PeriodicalIF":0.4,"publicationDate":"2024-03-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140226194","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
Goldberg–Shprintzen syndrome—A rare case from India 戈德伯格-施普雷岑综合征--来自印度的罕见病例
IF 0.4
Neurology and Clinical Neuroscience Pub Date : 2024-03-12 DOI: 10.1111/ncn3.12807
Murugasamy Pradeepkumar, Mohandass Kaviya, M. Gomathi
{"title":"Goldberg–Shprintzen syndrome—A rare case from India","authors":"Murugasamy Pradeepkumar, Mohandass Kaviya, M. Gomathi","doi":"10.1111/ncn3.12807","DOIUrl":"https://doi.org/10.1111/ncn3.12807","url":null,"abstract":"Goldberg–Shprintzen Syndrome (GOSHS, OMIM# 182212) is a rare autosomal dominant disorder with the characteristic features of craniosynostosis, developmental delays, intellectual disability, hypotonia, joint laxity, respiratory dysfunction, cardiovascular abnormalities, and abdominal hernias. Here we presented a rare case of 5‐years old male child with facial dysmorphism, global developmental delay, microcephaly, hypotonia, respiratory infection, breathing abnormalities, hematochezia, and meconium ileus. Whole exome sequencing revealed a novel homozygous deletion mutation in kinesin family binding protein (KIFBP) gene, NM_015634.4:c.1151del (p.Leu384Ter), which confirmed the molecular diagnosis of GOSHS.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":null,"pages":null},"PeriodicalIF":0.4,"publicationDate":"2024-03-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140250943","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
The time course of complement blockage by ravulizumab in neuromyelitis optica spectrum disorder 雷珠单抗在神经脊髓炎视网膜谱系障碍中阻断补体的时间过程
IF 0.4
Neurology and Clinical Neuroscience Pub Date : 2024-03-05 DOI: 10.1111/ncn3.12798
R. Amano
{"title":"The time course of complement blockage by ravulizumab in neuromyelitis optica spectrum disorder","authors":"R. Amano","doi":"10.1111/ncn3.12798","DOIUrl":"https://doi.org/10.1111/ncn3.12798","url":null,"abstract":"The classical 50% hemolytic complement test is commonly used to monitor complement function. However, its use in monitoring the effect of ravulizumab on complement assays is unknown. A 47‐year‐old man with anti‐aquaporin 4 antibody‐positive neuromyelitis optica spectrum disorder who was treated with oral prednisolone and azathioprine experienced several episodes of optic neuritis or myelitis. After his latest relapse, ravulizumab was administered to prevent relapse. Serum classical 50% hemolytic complement activity decreased below detectable levels (<10 U/mL) within 1 h of ravulizumab injection. Eighteen weeks later, the patient showed no adverse events or relapse, suggesting that serum classical 50% hemolytic complement activity can monitor ravulizumab effect. In Japan, where 3.5% of the population shows a reduced response to anti‐C5 therapy, serum classical 50% hemolytic complement activity proves useful in confirming ravulizumab efficacy.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":null,"pages":null},"PeriodicalIF":0.4,"publicationDate":"2024-03-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140263571","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 case of autoimmune glial fibrillary acidic protein astrocytopathy with excessive startle response and cortical hyperexcitability 一例伴有过度惊吓反应和皮质过度兴奋的自身免疫性胶质纤维酸性蛋白星形胶质细胞病变病例
IF 0.4
Neurology and Clinical Neuroscience Pub Date : 2024-03-01 DOI: 10.1111/ncn3.12803
Junjiro Tanimura, Kikuko Kaneko, Takao Hashimoto
{"title":"A case of autoimmune glial fibrillary acidic protein astrocytopathy with excessive startle response and cortical hyperexcitability","authors":"Junjiro Tanimura, Kikuko Kaneko, Takao Hashimoto","doi":"10.1111/ncn3.12803","DOIUrl":"https://doi.org/10.1111/ncn3.12803","url":null,"abstract":"We herein report a 60‐year‐old man with autoimmune glial fibrillary acidic protein astrocytopathy (GFAP‐A) with excessive startle response (ESR), or hyperekplexia. Short‐latency somatosensory evoked potentials (SSEPs) revealed high‐amplitude potentials of the early cortical component. Anti‐GFAPα antibodies in the spinal fluid were positive. The hyperekplexia, other clinical symptoms, and SSEP abnormalities were resolved by corticosteroid therapy. The present case demonstrates that ESR can be associated with GFAP‐A, and its pathophysiology may be cortical hyperexcitability.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":null,"pages":null},"PeriodicalIF":0.4,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140089373","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
Patchy FDG uptake on FDG PET/CT in a patient with vasculitic neuropathy in restricted lower‐limb vasculitis 一名局限性下肢血管炎血管神经病变患者的 FDG PET/CT 上出现斑片状 FDG 摄取
IF 0.4
Neurology and Clinical Neuroscience Pub Date : 2024-03-01 DOI: 10.1111/ncn3.12804
Hiroshi Sakiyama, M. Yamadera, Hideki Yorifuji, Misa Nakano, Yoshinori Katada
{"title":"Patchy FDG uptake on FDG PET/CT in a patient with vasculitic neuropathy in restricted lower‐limb vasculitis","authors":"Hiroshi Sakiyama, M. Yamadera, Hideki Yorifuji, Misa Nakano, Yoshinori Katada","doi":"10.1111/ncn3.12804","DOIUrl":"https://doi.org/10.1111/ncn3.12804","url":null,"abstract":"A 54‐year‐old man with diabetes mellitus presented with fever, pain, muscle weakness, and sensory disturbances in the lower limbs. His serum C‐reactive protein level was markedly increased; However, no autoantibodies, other than anti‐glutamic acid decarboxylase antibody, were detected. Nerve conduction studies revealed axonal polyneuropathy. 18F‐fluorodeoxyglucose positron emission tomography/computed tomography revealed patchy uptake patterns specific to restricted lower‐limb vasculitis. The muscle and nerve biopsy specimens suggested vasculitic neuropathy. Treatment with oral prednisolone and cyclophosphamide pulse therapy markedly improved his lower‐limb weakness and paresthesia. Clinicians should consider the involvement of vasculitic neuropathy, as well as myopathy, in patients with restricted lower‐limb vasculitis.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":null,"pages":null},"PeriodicalIF":0.4,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140088090","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
Surgical resection of a symptomatic intra‐axial lesion in a patient with Hennekam's syndrome: Case report with review of the literature 一名亨内卡姆综合征患者轴内无症状病变的手术切除：病例报告与文献综述
IF 0.4
Neurology and Clinical Neuroscience Pub Date : 2024-03-01 DOI: 10.1111/ncn3.12799
John E. Dugan, E. Lesha, Camille Milton, Clifford Yudkoff, Taylor Orr, Alan D. Boom, L. M. Michael
{"title":"Surgical resection of a symptomatic intra‐axial lesion in a patient with Hennekam's syndrome: Case report with review of the literature","authors":"John E. Dugan, E. Lesha, Camille Milton, Clifford Yudkoff, Taylor Orr, Alan D. Boom, L. M. Michael","doi":"10.1111/ncn3.12799","DOIUrl":"https://doi.org/10.1111/ncn3.12799","url":null,"abstract":"Hennekam syndrome (HS) is a rare genetic disorder involving malformations of the lymphatic system. Structural abnormalities and malformations within the central nervous system (CNS) have also been reported along with intellectual disability and developmental delay. We report a patient with HS who presented with seizures and was found to have an intra‐axial lesion, which was resected via craniotomy. While this case represents, to our knowledge, the first instance in the literature that a HS patient has been treated for a CNS abnormality via neurosurgical intervention, we provide a literature review of CNS lesions in patients with HS.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":null,"pages":null},"PeriodicalIF":0.4,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140088046","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
Deteriorating neurological symptoms of Degos disease despite the treatment with eculizumab and beraprost: A case report with detailed radiological findings 尽管使用了依库珠单抗和贝前列素，德戈斯病的神经症状仍在恶化：附详细放射学检查结果的病例报告
IF 0.4
Neurology and Clinical Neuroscience Pub Date : 2024-02-25 DOI: 10.1111/ncn3.12801
Yamato Nakamura, Masanori Sawamura, Tatsuhiko Miyazaki, Aya Muramatsu, Makoto Suzuki, Hiroaki Yagi, Akinobu Hori, Yusuke Katsuyama, Hidefumi Yoshida, Kang Kim, Hiroshi Yamada, Ryosuke Takahashi, K. Harada
{"title":"Deteriorating neurological symptoms of Degos disease despite the treatment with eculizumab and beraprost: A case report with detailed radiological findings","authors":"Yamato Nakamura, Masanori Sawamura, Tatsuhiko Miyazaki, Aya Muramatsu, Makoto Suzuki, Hiroaki Yagi, Akinobu Hori, Yusuke Katsuyama, Hidefumi Yoshida, Kang Kim, Hiroshi Yamada, Ryosuke Takahashi, K. Harada","doi":"10.1111/ncn3.12801","DOIUrl":"https://doi.org/10.1111/ncn3.12801","url":null,"abstract":"Degos disease is a rare vasculopathy with a poor prognosis. Here, we report a case of a 17‐year‐old Japanese male with Degos disease who exhibited progressive deterioration of neurological symptoms despite treatment with eculizumab and beraprost. We also report detailed radiological findings including brain magnetic resonance imaging and perfusion computed tomography, and hereby suggest the importance of the perfusion study in detecting early changes associated with Degos disease.","PeriodicalId":19154,"journal":{"name":"Neurology and Clinical Neuroscience","volume":null,"pages":null},"PeriodicalIF":0.4,"publicationDate":"2024-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140432228","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
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