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[Paraneoplastic Disorders of Peripheral Nervous System]. [周围神经系统副肿瘤性疾病]。
Brain and Nerve Pub Date : 2024-05-01 DOI: 10.11477/mf.1416202643
Akio Kimura, Yoya Ono, Takayoshi Shimohata
{"title":"[Paraneoplastic Disorders of Peripheral Nervous System].","authors":"Akio Kimura, Yoya Ono, Takayoshi Shimohata","doi":"10.11477/mf.1416202643","DOIUrl":"10.11477/mf.1416202643","url":null,"abstract":"<p><p>Paraneoplastic disorders of the peripheral nervous system are immune-mediated neurological syndromes associated with tumors. Several clinical phenotypes have been associated with these disorders. Sensory neuronopathy is the most well-known clinical phenotype, and is caused by neuronal cell injury to the dorsal root ganglia. Symptoms of the peripheral nervous system usually lead to the discovery of tumors. Antineuronal antibodies are occasionally identified in the serum and/or cerebrospinal fluid of these patients. The prevalence of small-cell lung cancer is notable in these patients. Early tumor resection, coupled with the initiation of immunotherapy, may prove effective in improving and stabilizing clinical symptoms.</p>","PeriodicalId":52507,"journal":{"name":"Brain and Nerve","volume":"76 5","pages":"555-561"},"PeriodicalIF":0.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140917443","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
[Autoimmune Autonomic Ganglionopathy and Acute Autonomic Sensory Neuropathy]. [自身免疫性自主神经节病和急性自主神经感觉神经病]。
Brain and Nerve Pub Date : 2024-05-01 DOI: 10.11477/mf.1416202644
Shunya Nakane
{"title":"[Autoimmune Autonomic Ganglionopathy and Acute Autonomic Sensory Neuropathy].","authors":"Shunya Nakane","doi":"10.11477/mf.1416202644","DOIUrl":"10.11477/mf.1416202644","url":null,"abstract":"<p><p>Autoimmune autonomic ganglionopathy (AAG) and acute autonomic sensory neuropathy (AASN) are immune-mediated neuropathies that affect the autonomic and/or dorsal root ganglia. Autoantibodies against the nicotinic ganglionic acetylcholine receptor (gAChR) detected in the sera of patients with AAG play a key role in the pathogenesis of this condition. Notably, gAChR antibodies are not detected in the sera of patients with AASN. Currently, AAG and AASN are not considered to be on the same spectrum with regard to disease concept based on clinical symptoms and laboratory findings. However, extra-autonomic brain symptoms (including psychiatric symptoms and personality changes) and endocrine disorders occur in both diseases, which suggests shared pathophysiology between the two conditions.</p>","PeriodicalId":52507,"journal":{"name":"Brain and Nerve","volume":"76 5","pages":"562-568"},"PeriodicalIF":0.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140917467","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
[Treatment Strategies for Anti-Synthetase Syndrome]. [抗合成酶综合征的治疗策略]。
Brain and Nerve Pub Date : 2024-05-01 DOI: 10.11477/mf.1416202656
Ran Nakashima
{"title":"[Treatment Strategies for Anti-Synthetase Syndrome].","authors":"Ran Nakashima","doi":"10.11477/mf.1416202656","DOIUrl":"10.11477/mf.1416202656","url":null,"abstract":"<p><p>Anti-aminoacyl tRNA synthetase (ARS) antibodies are the most frequent in idiopathic inflammatory myopathy, notably associated with anti-synthetase syndrome (ASyS), which is characterized by six clinical features: arthritis, myositis, interstitial lung disease (ILD), fever, Raynaud's phenomenon, and mechanical hands. Although patients with ASyS often respond well to initial glucocorticoid (GC) therapy, they tend to have a chronic, recurrent disease course. In anti-ARS-positive patients, the treatment goal involves suppressing disease recurrence and progression while achieving a minimal GC dose. In this regard, the administration and continuation of immunosuppressants, such as calcineurin inhibitors, have been suggested. B-cell depletion therapies are expected to be valuable in patients with refractory ASyS. Moreover, additional antifibrotic agents may be beneficial for patients with progressive fibrosing ILD.</p>","PeriodicalId":52507,"journal":{"name":"Brain and Nerve","volume":"76 5","pages":"655-659"},"PeriodicalIF":0.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140917475","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
[Utilization of Peripheral Neuropathology Tests]. [外周神经病理测试的利用]。
Brain and Nerve Pub Date : 2024-05-01 DOI: 10.11477/mf.1416202632
Ryota Sato
{"title":"[Utilization of Peripheral Neuropathology Tests].","authors":"Ryota Sato","doi":"10.11477/mf.1416202632","DOIUrl":"10.11477/mf.1416202632","url":null,"abstract":"<p><p>Neuropathological findings rarely lead to a definitive diagnosis of autoimmune and inflammatory peripheral nerve diseases, and indications for invasive nerve biopsy with subsequent disability should be carefully determined. In addition to disease-specific pathological findings, identifying findings that facilitate differential diagnosis in clinical practice is necessary. This article reviews the neuropathological findings that are valuable in the differential diagnosis of autoimmune and inflammatory peripheral nerve diseases.</p>","PeriodicalId":52507,"journal":{"name":"Brain and Nerve","volume":"76 5","pages":"473-479"},"PeriodicalIF":0.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140917478","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
[Guillain-Barré syndrome]. [格林-巴利综合征]。
Brain and Nerve Pub Date : 2024-05-01 DOI: 10.11477/mf.1416202635
Kenichi Kaida
{"title":"[Guillain-Barré syndrome].","authors":"Kenichi Kaida","doi":"10.11477/mf.1416202635","DOIUrl":"10.11477/mf.1416202635","url":null,"abstract":"<p><p>Guillain-Barré syndrome (GBS), an acute immune-mediated neuropathy, occurs following immunological stimulation, such as infection, with complement-mediated neuropathy implicated in the pathophysiology of this condition. Glycolipid antibodies produced by molecular mimicry are detected in approximately 60% of cases. Recent studies have suggested the role of cell-mediated immunity in the pathogenesis of GBS. Intravenous immunoglobulin and plasma exchange are established immunotherapies. In this article, based on the latest knowledge, we describe the pathophysiology, diagnosis, treatment, prognosis, and prognostic prediction of GBS. Furthermore, we discuss some GBS guidelines published by the European Academy of Neurology/Peripheral Nerve Society.</p>","PeriodicalId":52507,"journal":{"name":"Brain and Nerve","volume":"76 5","pages":"499-507"},"PeriodicalIF":0.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140917434","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
[How to Perform Autoantibody Testing for Autoimmune and Inflammatory Diseases of Peripheral Nerves and Muscles]. [如何对周围神经和肌肉的自身免疫性疾病和炎症性疾病进行自身抗体检测]。
Brain and Nerve Pub Date : 2024-05-01 DOI: 10.11477/mf.1416202629
Michiaki Koga
{"title":"[How to Perform Autoantibody Testing for Autoimmune and Inflammatory Diseases of Peripheral Nerves and Muscles].","authors":"Michiaki Koga","doi":"10.11477/mf.1416202629","DOIUrl":"10.11477/mf.1416202629","url":null,"abstract":"<p><p>Measurement of autoantibodies is essential for the management of several peripheral nerve and muscle diseases. The clinical significance of autoantibody testing differs for each antibody. In addition, clinicians must understand several issues including the accuracy of the test, isotype and subclass distribution, and its relationship to disease activity. Moreover, many autoantibody tests are not covered by health insurance. With limited medical resources, clinicians are required to be up-to-date with the latest information to utilize test results in daily practice without misunderstanding.</p>","PeriodicalId":52507,"journal":{"name":"Brain and Nerve","volume":"76 5","pages":"443-448"},"PeriodicalIF":0.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140917435","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
[Pathogenic Mechanisms of Diabetic Neuropathy]. [糖尿病神经病变的致病机制]。
Brain and Nerve Pub Date : 2024-05-01 DOI: 10.11477/mf.1416202658
Kazunori Sango, Hideji Yako, Shizuka Takaku, Naoko Niimi
{"title":"[Pathogenic Mechanisms of Diabetic Neuropathy].","authors":"Kazunori Sango, Hideji Yako, Shizuka Takaku, Naoko Niimi","doi":"10.11477/mf.1416202658","DOIUrl":"10.11477/mf.1416202658","url":null,"abstract":"<p><p>Diabetes stands as the predominant cause of peripheral neuropathy, and diabetic neuropathy (DN) is an early-onset and most frequent complication of diabetes. Distal symmetric polyneuropathy is the major form of DN; however, various patterns of nerve injury can manifest. Growing evidence suggests that hyperglycemia-related metabolic disorders in neurons, Schwann cells, and vascular endothelial cells play a major role in the development and progression of DN; however, its pathogenesis and development of disease-modifying therapies warrant further investigation. Herein, recent studies regarding the possible pathogenic factors of DN (polyol and other collateral glycolysis pathways, glycation, oxidative stress, Rho/Rho kinase signaling pathways, etc.) and therapeutic strategies targeting these factors are introduced.</p>","PeriodicalId":52507,"journal":{"name":"Brain and Nerve","volume":"76 5","pages":"671-680"},"PeriodicalIF":0.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140917444","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
[Muscle-Specific Receptor Tyrosine Kinase Antibody Positive Myasthenia Gravis]. [肌肉特异性受体酪氨酸激酶抗体阳性肌萎缩症]。
Brain and Nerve Pub Date : 2024-05-01 DOI: 10.11477/mf.1416202652
Masakatsu Motomura, Hiroko Kitanosono, Shunsuke Yoshimura, Hirokazu Shiraishi
{"title":"[Muscle-Specific Receptor Tyrosine Kinase Antibody Positive Myasthenia Gravis].","authors":"Masakatsu Motomura, Hiroko Kitanosono, Shunsuke Yoshimura, Hirokazu Shiraishi","doi":"10.11477/mf.1416202652","DOIUrl":"10.11477/mf.1416202652","url":null,"abstract":"<p><p>Reportedly, patients with muscle-specific kinase (MuSK) antibody-positive myasthenia gravis (MG) account for approximately 3.0% of all patients with MG in Japan. Compared with patients who have acetylcholine receptor antibody-positive MG, those with MuSK antibody-positive MG show young-onset disease with female predominance, a low rate of ocular involvement (5.9%), and greater severity of dysphagia. The aforementioned types of MG are indistinguishable based on clinical symptoms and electrophysiological tests, and measurement of MuSK antibodies is essential for diagnosis. Thymectomy and complement inhibitors are not indicated for treatment, and acetylcholinesterase inhibitors, steroids, immunosuppressants, plasma exchange, intravenous immunoglobulin therapy, and neonatal Fc receptor inhibitors are used.</p>","PeriodicalId":52507,"journal":{"name":"Brain and Nerve","volume":"76 5","pages":"623-629"},"PeriodicalIF":0.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140917440","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
[Peripheral Nerve and Muscle Disorders Associated with Sjögren's Syndrome]. [与斯约格伦综合征相关的周围神经和肌肉疾病]。
Brain and Nerve Pub Date : 2024-05-01 DOI: 10.11477/mf.1416202648
Hiroya Kuwahara, Takanori Yokota
{"title":"[Peripheral Nerve and Muscle Disorders Associated with Sjögren's Syndrome].","authors":"Hiroya Kuwahara, Takanori Yokota","doi":"10.11477/mf.1416202648","DOIUrl":"10.11477/mf.1416202648","url":null,"abstract":"<p><p>Sjögren's syndrome is often accompanied by various neurological complications, among which peripheral neuropathy is the most common. A variety of clinical phenotypes of peripheral neuropathy, including axonal polyneuropathy and sensory ataxic neuropathy are reported in the literature. We present an overview of the pathophysiology and differential diagnosis of each phenotype. Immunotherapy using corticosteroids and high-dose intravenous immunoglobulin therapy tends to elicit varied therapeutic responses depending on the peripheral neuropathy phenotype. We also discuss myositis, a possible complication of Sjögren's syndrome.</p>","PeriodicalId":52507,"journal":{"name":"Brain and Nerve","volume":"76 5","pages":"588-597"},"PeriodicalIF":0.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140917468","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
[Neuropathology of Disorders Leading to Dementia]. [导致痴呆症的神经病理学]。
Brain and Nerve Pub Date : 2024-04-01 DOI: 10.11477/mf.1416202610
Kenji Sakai, Masahito Yamada
{"title":"[Neuropathology of Disorders Leading to Dementia].","authors":"Kenji Sakai, Masahito Yamada","doi":"10.11477/mf.1416202610","DOIUrl":"https://doi.org/10.11477/mf.1416202610","url":null,"abstract":"<p><p>Dementia is characterized by acquired cognitive dysfunction caused by various neurological disorders. Many neurological conditions can cause dementia, including neurodegenerative diseases, vascular disorders, infections, inflammation, demyelination, intoxication, metabolic disorders, tumors, and head trauma. Despite recent developments in biomarkers and imaging techniques, neuropathological examination is necessary for the final diagnosis. Moreover, approximately 11% of the patients with dementia have dual or triple pathological conditions. The coexistence of neurological diseases makes it difficult for neurologists to diagnose patients accurately. Degenerative diseases are characterized by neuronal loss with gliosis in distinct parts of the brain, the presence of neuronal or glial inclusions, and abnormal protein accumulation. Senile plaques and neurofibrillary tangles are neuropathological hallmarks of Alzheimer's disease. These findings are characterized by the presence of amyloid β protein (Aβ) and phosphorylated tau protein, respectively. Although vascular dementia is common, it may be difficult to identify the relationship between vascular lesions and cognitive impairment. The incidence of sporadic Aβ-type cerebral amyloid angiopathy (CAA) tends to increase with age and causes dementia due to vascular dysfunction and leukoencephalopathy. Furthermore, patients with CAA can develop inflammation. Clinical neurologists should possess a neuropathological perspective for the appropriate diagnosis and management of patients with dementia.</p>","PeriodicalId":52507,"journal":{"name":"Brain and Nerve","volume":"76 4","pages":"333-342"},"PeriodicalIF":0.0,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140868233","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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