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[CIDP Variants]. [CIDP 变异]。
Brain and Nerve Pub Date : 2024-05-01 DOI: 10.11477/mf.1416202638
Norito Kokubun
{"title":"[CIDP Variants].","authors":"Norito Kokubun","doi":"10.11477/mf.1416202638","DOIUrl":"10.11477/mf.1416202638","url":null,"abstract":"<p><p>Chronic inflammatory demyelinating polyneuropathy (CIDP) is a heterogeneous syndrome that has several variants. Although they share macrophage-associated demyelination, clinical, neurophysiological, and pathological investigations have demonstrated that each subtype has a different pathophysiology. Multifocal CIDP exhibits a chronic course with asymmetrical symptoms. Its neurophysiological significance involves multifocal demyelination at intermediate nerve sites. Distal CIDP has a prolonged chronic course, presenting sensory and motor symptoms in a length-dependent manner. Furthermore, it frequently coexists with IgG M proteinemia or other hematologic disorders. Motor CIDP displays symmetric muscle weakness similar to typical CIDP but lacks sensory involvement. Often, motor CIDP is associated with malignancy or inflammatory diseases. Although acute deterioration after corticosteroid therapy in patients with motor CIDP is well-known, the available evidence to support this is limited.</p>","PeriodicalId":52507,"journal":{"name":"Brain and Nerve","volume":"76 5","pages":"520-525"},"PeriodicalIF":0.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140917429","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
[Dermatomyositis]. [皮肌炎］
Brain and Nerve Pub Date : 2024-05-01 DOI: 10.11477/mf.1416202654
Kazuma Sugie
{"title":"[Dermatomyositis].","authors":"Kazuma Sugie","doi":"10.11477/mf.1416202654","DOIUrl":"10.11477/mf.1416202654","url":null,"abstract":"<p><p>Dermatomyositis (DM) is distinguished from other idiopathic inflammatory myopathies by the characteristic skin rashes, muscle pathology, and muscle symptoms. Five myositis-specific autoantibodies have been identified in DM, and the correlation between each antibody and the clinical picture is clear. Pathological analysis has also identified DM as a type I interferonopathy of the skeletal muscle. Consideration of treatment strategies requires careful evaluation of muscle strength, systemic inflammatory findings, muscle pathology, muscle imaging, and complications such as malignancy and interstitial lung disease. Corticosteroids are administered as first-line treatment, and immunosuppressive agents and intravenous immunoglobulins are employed as important second-line treatments. Some patients exhibit resistance to these therapies. Currently, treatment strategies for refractory cases are not well established, necessitating further development of treatment methods.</p>","PeriodicalId":52507,"journal":{"name":"Brain and Nerve","volume":"76 5","pages":"635-645"},"PeriodicalIF":0.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140917430","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
[Immune-Mediated Necrotizing Myopathy]. [免疫介导的坏死性肌病]。
Brain and Nerve Pub Date : 2024-05-01 DOI: 10.11477/mf.1416202655
Akinori Uruha
{"title":"[Immune-Mediated Necrotizing Myopathy].","authors":"Akinori Uruha","doi":"10.11477/mf.1416202655","DOIUrl":"10.11477/mf.1416202655","url":null,"abstract":"<p><p>Immune-mediated necrotizing myopathy (IMNM) is a form of autoimmune myositis characterized by the presence of necrotic and regenerating process as a major finding in the muscle. Anti-SRP and anti-HMGCR have been identified as IMNM-specific autoantibodies. Patients with this disease often present with severe muscle weakness and markedly elevated serum creatine kinase (CK) levels. Differentiation from muscular dystrophy is challenging in certain cases. When patients meet the condition \"subacute onset\", \"hyperCKemia over 1000 IU/L\", and \"clinical diagnosis of muscular dystrophy lacking molecular diagnosis\", the possibility of IMNM should be considered. Autoantibody measurement, including of anti-SRP and HMGCR antibodies, is recommended. Treatment with corticosteroid in combination with immunosuppressants, intravenous immunoglobulin, and rituximab can be performed.</p>","PeriodicalId":52507,"journal":{"name":"Brain and Nerve","volume":"76 5","pages":"646-654"},"PeriodicalIF":0.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140917436","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
[Lambert-Eaton Myasthenic Syndrome]. [兰伯特-伊顿肌萎缩综合症]。
Brain and Nerve Pub Date : 2024-05-01 DOI: 10.11477/mf.1416202653
Hidenori Matsuo
{"title":"[Lambert-Eaton Myasthenic Syndrome].","authors":"Hidenori Matsuo","doi":"10.11477/mf.1416202653","DOIUrl":"10.11477/mf.1416202653","url":null,"abstract":"<p><p>Lambert-Eaton myasthenic syndrome (LEMS), an autoimmune disorder that affects the neuromuscular junction, is characterized by proximal muscle weakness, reduction of tendon reflexes, and autonomic dysfunction. LEMS shows a prevalence of approximately 0.25-0.27 per 100,000 population. The characteristic muscle weakness observed in patients with LEMS is attributed to the role of pathogenic autoantibodies directed against voltage-gated calcium channels (VGCC) present on the presynaptic nerve terminal. Notably, 50-60% of patients with LEMS have an associated tumor, small-cell lung carcinoma (SCLC), which also expresses functional voltage-gated calcium channels (VGCC). The Japanese LEMS diagnostic criteria 2022 recommend documentation of typical electrophysiological abnormalities combined with myasthenic symptoms for accurate diagnosis. P/Q-type VGCC antibody positivity strongly supports the diagnosis. Treatment options are categorized as oncological treatment, immunotherapy, and symptomatic treatments. Effective treatment of the tumor can improve LEMS in patients with SCLC. Most patients benefit from 3,4-diaminopyridine administration for symptomatic treatment. A treatment algorithm is established by the clinical practice guidelines 2022.</p>","PeriodicalId":52507,"journal":{"name":"Brain and Nerve","volume":"76 5","pages":"630-634"},"PeriodicalIF":0.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140917438","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 Neuropathy and Muscle Disorders as Immune-Related Adverse Events]. [作为免疫相关不良事件的周围神经病变和肌肉失调】。］
Brain and Nerve Pub Date : 2024-05-01 DOI: 10.11477/mf.1416202650
Shigeaki Suzuki
{"title":"[Peripheral Neuropathy and Muscle Disorders as Immune-Related Adverse Events].","authors":"Shigeaki Suzuki","doi":"10.11477/mf.1416202650","DOIUrl":"10.11477/mf.1416202650","url":null,"abstract":"<p><p>Neurological immune-related adverse events (irAEs) associated with cancer treatment with immune checkpoint inhibitors (ICI) present diverse clinical characteristics. Neurological irAEs affect the peripheral nervous system and muscles more than they affect the central nervous system. Among the various subsets of peripheral neuropathies, polyradiculoneuropathy, which includes Guillain-Barre syndrome and chronic inflammatory demyelinating polyneuropathy, stands out as the most severe form, leading to significant muscle weakness. ICIs can induce dysautonomia, including autoimmune autonomic ganglionopathy. Autonomic neuropathy represents a neurological irAE. Neurological irAEs of neuromuscular junctions include myasthenia gravis (MG) and Lambert-Eaton myasthenic syndrome (LEMS). Diagnosing MG or myositis independently can be challenging when they occur as irAEs. Myocarditis is sometimes observed as an irAE in patients with MG and can cause both severe heart failure and lethal arrhythmias, resulting in fatal outcomes. Anti-Kv1.4 antibodies are biomarkers of the severe form of MG and myocarditis. The administration of ICI in patients with small cell lung cancer increases the risk of LEMS. The distinction between LEMS is an irAE or a manifestation of paraneoplastic neurological syndrome is unclear as both conditions share common immunological mechanisms.</p>","PeriodicalId":52507,"journal":{"name":"Brain and Nerve","volume":"76 5","pages":"605-611"},"PeriodicalIF":0.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140917469","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
[Typical CIDP: Update of the Pathogenesis, Diagnosis, and Treatment]. [典型 CIDP：发病机制、诊断和治疗的最新进展]。
Brain and Nerve Pub Date : 2024-05-01 DOI: 10.11477/mf.1416202637
Satoshi Kuwabara
{"title":"[Typical CIDP: Update of the Pathogenesis, Diagnosis, and Treatment].","authors":"Satoshi Kuwabara","doi":"10.11477/mf.1416202637","DOIUrl":"10.11477/mf.1416202637","url":null,"abstract":"<p><p>Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is the most common chronic immune-mediated demyelinating neuropathy and includes several clinical subtypes. The major phenotype is \"typical CIDP,\" which is characterized by symmetric polyneuropathy and \"proximal and distal\" muscle weakness. In typical CIDP, the nerve roots and distal nerve terminals, where the blood-nerve barrier is anatomically deficient, are preferentially affected, and therefore antibody-mediated immune pathogenesis is likely to have a major role. Currently, CIDP is considered a syndrome including typical CIDP and CIDP variants. In 2021, the European Academy of Neurology/Peripheral Nerve Society Guideline was published, whereas the Japanese CIDP/ Multifocal Motor Neuropathy Clinical Practice Guideline will be available in May 2024. This review article summarizes the immunopathogenesis, diagnosis, and treatment for typical CIDP.</p>","PeriodicalId":52507,"journal":{"name":"Brain and Nerve","volume":"76 5","pages":"515-519"},"PeriodicalIF":0.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140917477","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
[AChR Antibody-Positive Myasthenia Gravis]. [AChR 抗体阳性肌萎缩症]。
Brain and Nerve Pub Date : 2024-05-01 DOI: 10.11477/mf.1416202651
Hiroyuki Murai
{"title":"[AChR Antibody-Positive Myasthenia Gravis].","authors":"Hiroyuki Murai","doi":"10.11477/mf.1416202651","DOIUrl":"10.11477/mf.1416202651","url":null,"abstract":"<p><p>Herein, we describe the mechanisms, diagnostic procedures, and treatment options for acetylcholine receptor (AChR) antibody-positive myasthenia gravis (MG). The upstream pathomechanism of this condition involves AChR-sensitized T cell-dependent B cell proliferation and the subsequent production of pathogenic autoantibodies. Downstream molecules include AChR antibodies that activate complement pathways, resulting in the destruction of motor endplates. We further introduce newly-developed molecular targeted drugs for the treatment of MG that aims to secure patients' health-related quality of life.</p>","PeriodicalId":52507,"journal":{"name":"Brain and Nerve","volume":"76 5","pages":"613-620"},"PeriodicalIF":0.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140917462","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
[Advancements in Magnetic Resonance Neurography: Techniques, Sequences, and Standardization]. [磁共振神经成像的进展：技术、序列和标准化]。
Brain and Nerve Pub Date : 2024-05-01 DOI: 10.11477/mf.1416202633
Hajime Yokota
{"title":"[Advancements in Magnetic Resonance Neurography: Techniques, Sequences, and Standardization].","authors":"Hajime Yokota","doi":"10.11477/mf.1416202633","DOIUrl":"10.11477/mf.1416202633","url":null,"abstract":"<p><p>Magnetic resonance neurography requires varying imaging techniques based on the site of imaging and anticipated disease. In assessing the brachial and lumbosacral plexus, a three-dimensional (3D) spin echo method, such as 3D-short tau inversion recovery imaging, is frequently employed. It's beneficial to familiarize oneself with the imaging sequence and understand the appearance of normal images in advance. The imaging parameters used in our institute are provided below as a reference. When interpreting the images, pay close attention to nerve thickening, signal intensity changes, asymmetry between the left and right sides, and irregularities in nerve caliber. Efforts are underway to standardize qualitative assessments and quantify signals through technological advancements.</p>","PeriodicalId":52507,"journal":{"name":"Brain and Nerve","volume":"76 5","pages":"481-486"},"PeriodicalIF":0.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140917463","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
[POEMS Syndrome]. [POEMS 综合症]。
Brain and Nerve Pub Date : 2024-05-01 DOI: 10.11477/mf.1416202642
Tomoki Suichi, Sonoko Misawa
{"title":"[POEMS Syndrome].","authors":"Tomoki Suichi, Sonoko Misawa","doi":"10.11477/mf.1416202642","DOIUrl":"10.11477/mf.1416202642","url":null,"abstract":"<p><p>POEMS syndrome is a multisystem disorder associated with monoclonal plasma cell proliferation and the overproduction of vascular endothelial growth factors. The prognosis of POEMS syndrome has significantly improved owing to anti-myeloma treatments such as thalidomide and autologous stem cell transplantation. Therefore, early diagnosis and appropriate treatment are becoming increasingly important. A thorough and comprehensive evaluation of both systemic symptoms and laboratory abnormalities associated with the disease is essential for early diagnosis. The collaboration between neurology and hematology is indispensable to ensure proper treatment.</p>","PeriodicalId":52507,"journal":{"name":"Brain and Nerve","volume":"76 5","pages":"547-554"},"PeriodicalIF":0.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140917471","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
[Neuromuscular Ultrasound: Diagnosis and Evaluation in Neuromuscular Diseases]. [神经肌肉超声：神经肌肉疾病的诊断和评估]。
Brain and Nerve Pub Date : 2024-05-01 DOI: 10.11477/mf.1416202634
Yu-Ichi Noto
{"title":"[Neuromuscular Ultrasound: Diagnosis and Evaluation in Neuromuscular Diseases].","authors":"Yu-Ichi Noto","doi":"10.11477/mf.1416202634","DOIUrl":"https://doi.org/10.11477/mf.1416202634","url":null,"abstract":"<p><p>Neuromuscular ultrasound has become an integral part of the diagnostic workup of neuromuscular diseases in neurology. Neuromuscular ultrasound can detect nerve enlargement, selective muscle damage, and fasciculation easily and non-invasively, which allows differentiation between auto-immune/inflammatory and degenerative/hereditary diseases. It is significant and essential for all neurologists to master the neuromuscular ultrasound technique.</p>","PeriodicalId":52507,"journal":{"name":"Brain and Nerve","volume":"76 5","pages":"487-496"},"PeriodicalIF":0.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140917441","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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