CONTINUUM Lifelong Learning in Neurology最新文献

Neurology's Action Potential: Holding Together the House of Neurology. 神经病学的动作电位：团结神经病学之家。

CONTINUUM Lifelong Learning in Neurology Pub Date : 2026-10-01 Epub Date: 2026-10-02 DOI: 10.1212/cont.0000000000001787

引用次数: 0

List of Abbreviations. 缩略语列表。

CONTINUUM Lifelong Learning in Neurology Pub Date : 2026-10-01 Epub Date: 2026-10-02 DOI: 10.1212/cont.0000000000001756

引用次数: 0

Small Fiber Neuropathy. 小纤维神经病。

CONTINUUM Lifelong Learning in Neurology Pub Date : 2026-10-01 Epub Date: 2026-10-02 DOI: 10.1212/cont.0000000000001763

Midori Eckenstein, Waqar Waheed

引用次数: 0

Hereditary Neuropathies. 遗传疾病。

CONTINUUM Lifelong Learning in Neurology Pub Date : 2026-10-01 Epub Date: 2026-10-02 DOI: 10.1212/cont.0000000000001749

Mario A Saporta, Stephan Züchner

{"title":"Hereditary Neuropathies.","authors":"Mario A Saporta, Stephan Züchner","doi":"10.1212/cont.0000000000001749","DOIUrl":"10.1212/cont.0000000000001749","url":null,"abstract":"Objective: This article describes the clinical features, genetic causes, and diagnostic strategies of inherited peripheral neuropathies, and highlights how recent developments are reshaping the management of these disorders.Latest developments: Multiple new genetic causes of hereditary neuropathies have been identified, deepening insight into peripheral nerve biology and enabling the first clinical trial targeting a recessive form of genetic neuropathy. Concurrent advances in next-generation sequencing have substantially improved diagnostic yield. In parallel, several disease-modifying therapeutic approaches are being evaluated in human studies. Progress in targeted therapies, particularly for familial amyloid neuropathy, provides a framework for the development of innovative treatments across the spectrum of genetic neuropathies.Essential points: Inherited peripheral neuropathies are among the most common genetic neuromuscular disorders worldwide, yet diagnosis remains challenging because of marked genetic and phenotypic heterogeneity. Charcot-Marie-Tooth disease is now linked to pathogenic variants in more than 100 genes encoding proteins essential for Schwann cell biology and peripheral axonal function. Additional inherited neuropathies present as multisystem disorders, including familial amyloid neuropathy and neuropathies associated with metabolic or other genetic conditions. Advances in sequencing technologies and refined genotype-phenotype correlations are transforming the diagnosis of inherited neuropathies.","PeriodicalId":52475,"journal":{"name":"CONTINUUM Lifelong Learning in Neurology","volume":"32 5","pages":"1486-1502"},"PeriodicalIF":0.0,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149118434","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

Diagnostic Approach to Neuropathy. 神经病变的诊断方法。

CONTINUUM Lifelong Learning in Neurology Pub Date : 2026-10-01 Epub Date: 2026-10-02 DOI: 10.1212/cont.0000000000001743

Reza Sadjadi

引用次数: 0

Inflammatory and Autoimmune Axonal Neuropathies. 炎性和自身免疫性轴突神经病。

CONTINUUM Lifelong Learning in Neurology Pub Date : 2026-10-01 Epub Date: 2026-10-02 DOI: 10.1212/cont.0000000000001746

Qihua Fan, Vinay Chaudhry

{"title":"Inflammatory and Autoimmune Axonal Neuropathies.","authors":"Qihua Fan, Vinay Chaudhry","doi":"10.1212/cont.0000000000001746","DOIUrl":"https://doi.org/10.1212/cont.0000000000001746","url":null,"abstract":"Objective: This article outlines the clinical patterns, diagnostic strategies, and treatment principles for patients with inflammatory and autoimmune axonal neuropathies.Latest developments: Recent studies highlight the role of serum neurofilament light chain as a biomarker of the rate of active axonal injury and the increasing use of rituximab over cyclophosphamide for induction in neutrophil cytoplasmic antibody-associated vasculitis. Imaging modalities, such as magnetic resonance neurography and fludeoxyglucose positron emission tomography (FDG-PET), aid in identifying biopsy targets and monitoring disease activity.Essential points: Inflammatory axonal neuropathies encompass a spectrum of immune-mediated disorders presenting with mononeuritis multiplex, asymmetric sensorimotor polyneuropathy, sensory ataxia, or plexopathy. The diagnosis requires a high degree of clinical suspicion, detailed electrodiagnostic evaluation, and consideration of systemic features. Nerve biopsy, although limited by patchy yield, can confirm the diagnosis and guide immunotherapy decisions. Treatment focuses on halting inflammation and preventing relapses, with corticosteroids as first-line induction and steroid-sparing agents for maintenance. A structured follow-up approach, including standardized neurologic examinations, functional assessments, and ancillary testing, is essential for tracking disease progression and treatment response.","PeriodicalId":52475,"journal":{"name":"CONTINUUM Lifelong Learning in Neurology","volume":"32 5","pages":"1365-1391"},"PeriodicalIF":0.0,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149118426","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

Paraproteinemic Neuropathies. Paraproteinemic神经病变。

CONTINUUM Lifelong Learning in Neurology Pub Date : 2026-10-01 Epub Date: 2026-10-02 DOI: 10.1212/cont.0000000000001748

Amro M Stino, Benjamin E Becker

{"title":"Paraproteinemic Neuropathies.","authors":"Amro M Stino, Benjamin E Becker","doi":"10.1212/cont.0000000000001748","DOIUrl":"https://doi.org/10.1212/cont.0000000000001748","url":null,"abstract":"Objective: Paraproteinemic neuropathies present with a myriad of phenotypes and associated underlying plasma cell dyscrasias. This article provides a phenotype-driven approach to the evaluation of paraproteinemic neuropathies, guided by an alignment of clinical, electrodiagnostic, and serologic features.Latest developments: The advent of targeted immunotherapies has improved outcomes for patients with plasma cell dyscrasia. While no US Food and Drug Administration (FDA)-approved therapies exist for the disabling paraproteinemic neuropathies discussed in this article, such as anti-myelin-associated glycoprotein neuropathy or polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes (POEMS), increased understanding of these neuropathies' pathomechanisms and natural history is paving the way for newer, promising therapies. There are multiple ongoing trials, such as using Bruton tyrosine kinase inhibitors for the treatment of anti-myelin-associated glycoprotein neuropathy.Essential points: The evaluating clinician needs to properly order and interpret screening tests for paraproteinemic neuropathy. Red flag features beyond the typical distal symmetric polyneuropathy phenotype, namely, the presence of symmetric distal or proximal weakness, ataxia, autonomic deficits, ophthalmoplegia, demyelination on nerve conduction studies, and IgM or lambda gammopathy (particularly when combined with any of the above features), should prompt added scrutiny and phenotype-driven testing. Early diagnosis can streamline the administration of potentially effective immunotherapies and avoid unnecessary and inappropriate therapies and cost.","PeriodicalId":52475,"journal":{"name":"CONTINUUM Lifelong Learning in Neurology","volume":"32 5","pages":"1440-1460"},"PeriodicalIF":0.0,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149118595","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

Amyloid Neuropathies. 淀粉样疾病。

CONTINUUM Lifelong Learning in Neurology Pub Date : 2026-10-01 Epub Date: 2026-10-02 DOI: 10.1212/cont.0000000000001752

Marcus V Pinto

{"title":"Amyloid Neuropathies.","authors":"Marcus V Pinto","doi":"10.1212/cont.0000000000001752","DOIUrl":"https://doi.org/10.1212/cont.0000000000001752","url":null,"abstract":"Objective: This article reviews the pathophysiology, epidemiology, and clinical features of amyloid neuropathies, with a focus on light chain (AL) amyloidosis and hereditary amyloidogenic transthyretin (ATTRv) amyloidosis, and provides a brief overview of the most common systemic amyloidoses associated with neuropathy.Latest developments: Minimally invasive biopsies, such as skin punch biopsy and fat aspirate, are reasonable first biopsy sites in the evaluation of amyloid neuropathy with sensitivities of 48% to 83% for amyloid deposits. In the United States, it is recommended that every patient with ATTRv amyloidosis with peripheral neuropathy should have tissue confirmation of amyloid positivity. With the development of daratumumab, a highly effective plasma cell-depleting therapy, the survival rate for AL amyloidosis has increased, and the first-line therapy no longer includes autologous stem cell transplantation. First-generation and second-generation gene silencers are now available for the treatment of ATTRv amyloidosis with peripheral neuropathy and are highly effective in halting disease progression.Essential points: The overwhelming majority of amyloid neuropathies are secondary to AL amyloidosis or ATTRv amyloidosis, both treatable disorders that must not be missed. Their progressive nature and associated autonomic dysfunction are the most important clinical features and should always raise the possibility of amyloid neuropathies. The initial diagnostic workup of progressive neuropathies of uncertain etiology should always include monoclonal protein studies (with free light chain assay) and a fat aspirate or skin biopsy.","PeriodicalId":52475,"journal":{"name":"CONTINUUM Lifelong Learning in Neurology","volume":"32 5","pages":"1461-1485"},"PeriodicalIF":0.0,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149118463","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

Chronic Inflammatory Demyelinating Polyradiculoneuropathy. 慢性炎性脱髓鞘性多根神经病变。

CONTINUUM Lifelong Learning in Neurology Pub Date : 2026-10-01 Epub Date: 2026-10-02 DOI: 10.1212/cont.0000000000001747

Arjun Seth, Jeffrey A Allen

{"title":"Chronic Inflammatory Demyelinating Polyradiculoneuropathy.","authors":"Arjun Seth, Jeffrey A Allen","doi":"10.1212/cont.0000000000001747","DOIUrl":"https://doi.org/10.1212/cont.0000000000001747","url":null,"abstract":"Objective: This article describes the cardinal clinical, electrophysiologic, and supportive features of typical CIDP and its variants, how to review and apply CIDP diagnostic criteria, objective measures that can be used in practice to follow patients with CIDP, and current and emerging treatment practices.Latest developments: The diagnostic criteria for CIDP have been simplified to a diagnosis of either CIDP or possible CIDP. Chronic inflammatory sensory polyradiculoneuropathy is no longer considered a variant of CIDP. Autoimmune nodopathies are considered a separate entity with some clinical and electrodiagnostic features similar to CIDP. Treatments include IV immunoglobulin (IVIg), subcutaneous immunoglobulin, corticosteroids, and plasmapheresis. Subcutaneous efgartigimod alfa, a neonatal Fc-receptor antagonist, was approved by the US Food and Drug Administration (FDA) in June 2024 for the treatment of CIDP, the first novel treatment by drug class for this condition. Complement inhibition is currently being explored in clinical trials for the treatment of CIDP.Essential points: CIDP is a rare, treatable neuropathy that is frequently misdiagnosed. As novel therapeutics are studied and approved for clinical use, special attention to the diagnostic criteria and the use of objective treatment outcome measures are essential to preventing misdiagnosis and ensuring that patients receive appropriate therapies.","PeriodicalId":52475,"journal":{"name":"CONTINUUM Lifelong Learning in Neurology","volume":"32 5","pages":"1416-1439"},"PeriodicalIF":0.0,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149118468","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

Nutritional and Toxic Neuropathies. 营养性和中毒性神经病。

CONTINUUM Lifelong Learning in Neurology Pub Date : 2026-10-01 Epub Date: 2026-10-02 DOI: 10.1212/cont.0000000000001745

Karissa L Gable

引用次数: 0