Seminars in NeurologyPub Date : 2025-04-01Epub Date: 2025-05-08DOI: 10.1055/a-2566-0796
David M Greer
{"title":"David Burkholder, MD, and Elaine Wirrell, MD.","authors":"David M Greer","doi":"10.1055/a-2566-0796","DOIUrl":"https://doi.org/10.1055/a-2566-0796","url":null,"abstract":"","PeriodicalId":49544,"journal":{"name":"Seminars in Neurology","volume":"45 2","pages":"177"},"PeriodicalIF":1.8,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144044486","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Seminars in NeurologyPub Date : 2025-04-01Epub Date: 2025-03-12DOI: 10.1055/a-2525-3511
Lisa Gillinder, Jeffrey W Britton
{"title":"Seizures Associated with Autoimmune Disorders - Current Treatment Approaches.","authors":"Lisa Gillinder, Jeffrey W Britton","doi":"10.1055/a-2525-3511","DOIUrl":"10.1055/a-2525-3511","url":null,"abstract":"<p><p>Autoimmune-associated seizures and epilepsy are increasingly recognized in clinical practice and can arise in the setting of acute encephalitis but in some cases may present with chronic focal epilepsy. These conditions are usually resistant to antiseizure therapy but may respond definitively to timely immunotherapy. Early diagnosis and treatment are critical to minimize neural injury and optimize outcomes. Treatment is guided by consensus opinion because definitive trials are currently lacking. The initial management approach usually involves first-line agents such as corticosteroids, intravenous immunoglobulin (IVIg), or plasma exchange, with second-line agents like rituximab or cyclophosphamide. Maintenance therapy is considered to prevent relapses, which occur in up to 35% of patients. Relapse management requires careful differentiation from postencephalitic epilepsy, which in the absence of active inflammation does not respond to immunotherapy. This review discusses treatment strategies for autoimmune-associated seizure disorders, including acute symptomatic seizures and epilepsy. We discuss expected outcomes on the basis of the underlying pathogenesis including cases mediated by autoantibodies targeting specific neuronal surface/synaptic antigens, and intracellular epitopes, and for cases lacking defined biomarkers. Specific approaches are outlined for disorders such as anti-LGI1, anti-NMDAR, anti-GABA-BR, and anti-GAD65 encephalitides, emphasizing tailored immunotherapy based on pathophysiology and clinical context.</p>","PeriodicalId":49544,"journal":{"name":"Seminars in Neurology","volume":" ","pages":"275-286"},"PeriodicalIF":1.8,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143617820","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Seminars in NeurologyPub Date : 2025-04-01Epub Date: 2025-03-19DOI: 10.1055/a-2562-1964
Shirin Jamal Omidi, Brian Nils Lundstrom
{"title":"Invasive Neurostimulation for the Treatment of Epilepsy.","authors":"Shirin Jamal Omidi, Brian Nils Lundstrom","doi":"10.1055/a-2562-1964","DOIUrl":"10.1055/a-2562-1964","url":null,"abstract":"<p><p>Although electricity has been used in medicine for thousands of years, bioelectronic medicine for treating epilepsy has become increasingly common in recent years. Invasive neurostimulation centers primarily around three approaches: vagus nerve stimulation (VNS), responsive neurostimulation (RNS), and deep brain stimulation (DBS). These approaches differ by target (e.g., cranial nerve, cortex, or thalamus) and stimulation parameters (e.g., triggered stimulation or continuous stimulation). Although typically noncurative, these approaches can dramatically reduce the seizure burden and offer patients new treatment options. There remains much to be understood about optimal targets and individualized stimulation protocols. Objective markers of seizure burden and biomarkers that quickly quantify neural excitability are still needed. In the future, bioelectronic medicine could become a curative approach that remodels neural networks to reduce pathological activity.</p>","PeriodicalId":49544,"journal":{"name":"Seminars in Neurology","volume":" ","pages":"252-263"},"PeriodicalIF":1.8,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12064384/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143665200","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Seminars in NeurologyPub Date : 2025-02-01Epub Date: 2024-10-21DOI: 10.1055/s-0044-1791721
Nuri Jacoby, Yaacov Anziska
{"title":"A Practical Approach to Diagnosing Peripheral Neuropathies.","authors":"Nuri Jacoby, Yaacov Anziska","doi":"10.1055/s-0044-1791721","DOIUrl":"10.1055/s-0044-1791721","url":null,"abstract":"<p><p>Polyneuropathies are common, with the incidence increasing with older age. The causes of polyneuropathies are diverse and numerous, and it can be challenging for clinicians to determine the etiology of a particular patient's neuropathy. In this article, we systematically detail a practical approach to polyneuropathies, beginning with the most important aspects of the workup, the history and physical. We then discuss the limited diagnostic approach required for patients who present with a distal symmetric polyneuropathy and the more comprehensive approach for patients who present with other neuropathy subtypes.</p>","PeriodicalId":49544,"journal":{"name":"Seminars in Neurology","volume":" ","pages":"4-12"},"PeriodicalIF":1.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142478984","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Seminars in NeurologyPub Date : 2025-02-01Epub Date: 2024-10-17DOI: 10.1055/s-0044-1791579
Elizabeth M Monohan, Thomas H Brannagan
{"title":"Immune-Mediated Neuropathies: Top 10 Clinical Pearls.","authors":"Elizabeth M Monohan, Thomas H Brannagan","doi":"10.1055/s-0044-1791579","DOIUrl":"10.1055/s-0044-1791579","url":null,"abstract":"<p><p>Immune-mediated neuropathies encompass a range of neurological disorders, including chronic inflammatory demyelinating polyradiculoneuropathy, Guillain-Barré syndrome, multifocal motor neuropathy, autoimmune autonomic neuropathies, and paranodal nodopathies. Recognizing clinical patterns is key to narrowing the broad range of differential diagnoses in immune-mediated neuropathies. Electrodiagnostic testing is a useful tool to support the diagnosis of immune-mediated neuropathies. Our understanding of autoimmune demyelinating neuropathies is rapidly advancing, particularly with the discovery of nodal and paranodal antibodies. Recent advances in neuropathy treatment include the utilization of neonatal Fc receptors to reduce antibody recycling, and the development of complement inhibitors to reduce inflammatory damage, offering promising new therapeutic avenues. Timely identification of immune-mediated neuropathies is imperative as delay in diagnosis and treatment may lead to irreversible disability.</p>","PeriodicalId":49544,"journal":{"name":"Seminars in Neurology","volume":" ","pages":"122-131"},"PeriodicalIF":1.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142478987","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Seminars in NeurologyPub Date : 2025-02-01Epub Date: 2024-10-11DOI: 10.1055/s-0044-1791693
Mitali Mehta, Jessica Robinson-Papp
{"title":"Infectious Neuropathies.","authors":"Mitali Mehta, Jessica Robinson-Papp","doi":"10.1055/s-0044-1791693","DOIUrl":"10.1055/s-0044-1791693","url":null,"abstract":"<p><p>This review explores diverse infectious etiologies of peripheral nervous system (PNS) dysfunction, spanning sensory and motor neurons, nerves, and associated structures. Progress in viral and bacterial infections reveals multifaceted mechanisms underlying neuropathies, including viral neurotoxicity and immune-mediated responses. Latest diagnostic advances facilitate early PNS complication detection, with ongoing research offering promising treatment avenues. Emerging pathogens like severe acute respiratory syndrome coronavirus 2, Zika virus, and EV-D68 highlight the evolving infectious neuropathy paradigm. Recognizing characteristic patterns and integrating clinical factors are pivotal for precise diagnosis and tailored intervention. Challenges persist in assessment and management due to varied pathogenic mechanisms. Advancements in understanding pathogenesis have improved targeted therapies, yet gaps remain in effective treatments. Ongoing research is crucial for optimizing approaches and improving patient outcomes.</p>","PeriodicalId":49544,"journal":{"name":"Seminars in Neurology","volume":" ","pages":"63-74"},"PeriodicalIF":1.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142407102","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Seminars in NeurologyPub Date : 2025-02-01Epub Date: 2024-10-15DOI: 10.1055/s-0044-1791520
Ruchee Patel, Bipasha Mukherjee-Clavin
{"title":"Top 10 Clinical Pearls in Inherited Neuropathies.","authors":"Ruchee Patel, Bipasha Mukherjee-Clavin","doi":"10.1055/s-0044-1791520","DOIUrl":"10.1055/s-0044-1791520","url":null,"abstract":"<p><p>The inherited neuropathies are a clinically and genetically heterogeneous collection of neuropathies that neurologists, particularly neuromuscular specialists, must be familiar with. They include Charcot-Marie-Tooth disease, which is common yet currently lacks targeted treatment, and hATTRV polyneuropathy, which is rare but has disease-modifying gene therapies. With a focus on emerging new genes and treatments, this article offers a recent update on clinical diagnosis and management of inherited neuropathies.</p>","PeriodicalId":49544,"journal":{"name":"Seminars in Neurology","volume":" ","pages":"88-98"},"PeriodicalIF":1.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142478992","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Seminars in NeurologyPub Date : 2025-02-01Epub Date: 2024-10-17DOI: 10.1055/s-0044-1791769
Benjamin Becker, Amro Stino
{"title":"Top 10 Clinical Pearls in Paraproteinemic Neuropathies.","authors":"Benjamin Becker, Amro Stino","doi":"10.1055/s-0044-1791769","DOIUrl":"10.1055/s-0044-1791769","url":null,"abstract":"<p><p>Paraproteinemic neuropathies represent an important subset of peripheral neuropathies. Once identified, further evaluation into the paraproteinemic subtype, clinical exam pattern, and electrodiagnostic phenotype helps clarify if the paraproteinemia is coincidental or causal of the neuropathy, as <i>not all paraproteinemias cause neuropathy</i>. Of all paraproteinemias, immunoglobulin M (IgM)-associated peripheral neuropathy, or IgM neuropathy, is of particular importance as half of IgM neuropathies also harbor anti-myelin-associated glycoprotein antibodies, which produce a characteristic demyelinating pattern on nerve conduction testing. Immunoglobulin G and immunoglobulin A paraproteinemias are less strongly associated with peripheral neuropathy, except in the setting of multiple myeloma or osteosclerotic myeloma (POEMS syndrome), which have characteristic systemic features. In multiple myeloma, chemotherapy is more likely to result in neuropathy than the myeloma itself. Finally, the presence of systemic features (e.g., cardiomyopathy, nephropathy, recurrent carpal tunnel syndrome, and autonomic insufficiency) should raise concern for hereditary or acquired light (AL) chain amyloidosis. AL amyloidosis can occur in the setting of any light or heavy chain paraproteinemia. Central to the proper evaluation of paraproteinemic neuropathy is electrodiagnostic testing, which helps delineate axonal versus demyelinating paraproteinemic neuropathy, the latter often misdiagnosed as chronic inflammatory demyelinating polyradiculoneuropathy.</p>","PeriodicalId":49544,"journal":{"name":"Seminars in Neurology","volume":" ","pages":"99-111"},"PeriodicalIF":1.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142478993","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Seminars in NeurologyPub Date : 2025-02-01Epub Date: 2024-10-21DOI: 10.1055/s-0044-1791823
Grazia Devigili, Raffaella Lombardi, Giuseppe Lauria, Daniele Cazzato
{"title":"The Evolving Landscape of Small Fiber Neuropathy.","authors":"Grazia Devigili, Raffaella Lombardi, Giuseppe Lauria, Daniele Cazzato","doi":"10.1055/s-0044-1791823","DOIUrl":"10.1055/s-0044-1791823","url":null,"abstract":"<p><p>Small fiber neuropathy (SFN) belongs to a heterogeneous group of disorders in which thinly myelinated Aδ and unmyelinated C-fibers are primarily affected, leading to neuropathic pain and autonomic symptoms. SFN can be associated with systemic conditions such as diabetes, autoimmune diseases, exposure to drugs and toxins, and infection, with the list of associated diseases continuing to expand. Variants in the SCN9A, SCN10A, and SCN11A genes encoding Nav 1.7, Nav 1.8, and Nav 1.9 sodium channel subunits, as well as in the TRPA1 gene, have been found in SFN patients, expanding the spectrum of underlying conditions and enhancing our understanding of pathophysiological mechanisms. There is also growing interest in immune-mediated forms that could help identify potentially treatable subgroups. According to international criteria, diagnosis is established through clinical examination, the assessment of intraepidermal nerve fiber density, and/or quantitative sensory testing. Autonomic functional tests allow for a better characterization of dysautonomia in SFN, which can be subclinical. Other tests can support the diagnosis. Currently, the management of SFN prioritizes treating the underlying condition, if identified, within a multidisciplinary approach that combines symptomatic pain therapy, lifestyle changes, and biopsychological interventions. Emerging insights from the molecular characterization of SFN channelopathies hold promise for improving diagnosis, potentially leading to the discovery of new drugs and refining trial designs in the future. This article reviews the clinical presentation, diagnostic workup, and advancing knowledge of associated conditions and interventional management of SFN.</p>","PeriodicalId":49544,"journal":{"name":"Seminars in Neurology","volume":" ","pages":"132-144"},"PeriodicalIF":1.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142478989","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Seminars in NeurologyPub Date : 2025-02-01Epub Date: 2024-10-11DOI: 10.1055/s-0044-1791578
Angelica Cornejo, Mary L Vo
{"title":"Updates on Common Mononeuropathies.","authors":"Angelica Cornejo, Mary L Vo","doi":"10.1055/s-0044-1791578","DOIUrl":"10.1055/s-0044-1791578","url":null,"abstract":"<p><p>This article provides an overview of the most common mononeuropathies. It includes a description of the neuroanatomy and function of each nerve which allows clinical localization of the lesion. It also describes the clinical presentation, findings in electrodiagnostic studies, updates in imaging including neuromuscular ultrasound and magnetic resonance neurography, and recommended treatment. While mononeuropathies may be part of polyneuropathy, this scenario is beyond the scope of this article. The most common mononeuropathy is carpal tunnel syndrome. Its prevalence in the United States is estimated at 50 per 1,000. The second most common entrapment neuropathy is ulnar neuropathy at the elbow. The incidence was calculated as 20.9% in a 2005 study. The most common compressive neuropathy of the lower extremity is peroneal neuropathy. Other common mononeuropathies included in this article are radial neuropathy, tibial neuropathy, and femoral neuropathy.</p>","PeriodicalId":49544,"journal":{"name":"Seminars in Neurology","volume":" ","pages":"13-23"},"PeriodicalIF":1.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142407105","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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