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LATEST DEVELOPMENTS: Emphasize serum neurofilament light chain as a biomarker of active axonal injury rate and growing use of rituximab instead of cyclophosphamide for induction in ANCA-associated vasculitis; highlight imaging such as magnetic resonance neurography and FDG-PET for biopsy targeting and monitoring. ESSENTIAL POINTS: Describe immune-mediated spectra, diagnostic workflow with electrodiagnostics and biopsy, and stepwise follow-up focused on preventing relapses using induction corticosteroids and maintenance steroid-sparing agents.",{"@graph":69,"@context":122},[70,84,105],{"@type":71,"itemListElement":72},"BreadcrumbList",[73,77,79,82],{"item":74,"name":75,"@type":76,"position":8},"https://docshare.wps.com","Home","ListItem",{"item":78,"name":9,"@type":76,"position":14},"https://docshare.wps.com/document/",{"item":80,"name":35,"@type":76,"position":81},"https://docshare.wps.com/document/healthcare/",3,{"item":83,"name":65,"@type":76,"position":19},"https://docshare.wps.com/document/inflammatory-and-autoimmune-axonal-neuropathies/542091/",{"url":83,"name":65,"@type":85,"image":86,"author":91,"headline":65,"publisher":94,"fileFormat":97,"inLanguage":63,"description":67,"dateModified":98,"datePublished":99,"encodingFormat":97,"isAccessibleForFree":100,"interactionStatistic":101},"DigitalDocument",{"url":87,"@type":88,"width":89,"height":90},"https://docshare.wps.com/thumbnails/inflammatory-and-autoimmune-axonal-neuropathies/542091.png","ImageObject",300,407,{"name":92,"@type":93},"Chumphorn","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-10-17","2026-10-10",true,{"@type":102,"interactionType":103,"userInteractionCount":81},"InteractionCounter",{"@type":104},"ViewAction",{"@type":106,"mainEntity":107},"FAQPage",[108,114,118],{"name":109,"@type":110,"acceptedAnswer":111},"What conditions are included under inflammatory and autoimmune axonal neuropathies?","Question",{"text":112,"@type":113},"They encompass a spectrum of immune-mediated disorders presenting with mononeuritis multiplex, asymmetric sensorimotor polyneuropathy, sensory ataxia, or plexopathy. The article stresses immune mechanisms and clinical presentation patterns.","Answer",{"name":115,"@type":110,"acceptedAnswer":116},"How are diagnosis and axonal pattern confirmation approached?",{"text":117,"@type":113},"Diagnosis requires high clinical suspicion, detailed electrodiagnostic evaluation, and consideration of systemic features. Nerve biopsy can help confirm the diagnosis and guide immunotherapy despite limited patchy yield.",{"name":119,"@type":110,"acceptedAnswer":120},"What are the key treatment principles and follow-up elements?",{"text":121,"@type":113},"Treatment aims to halt inflammation and prevent relapses, using corticosteroids as first-line induction and steroid-sparing agents for maintenance. A structured follow-up with standardized neurologic exams, functional assessments, and ancillary testing is essential to track progression and response.","https://schema.org",{"og:url":83,"og:type":124,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":126,"canonical":83},"index,follow",{"doc_id":128,"site_id":62},542091,1792171168,{"code":4,"msg":5,"data":131},{"doc_id":128,"user_id":132,"nickname":92,"user_avatar":133,"doc_module":4,"category_id":34,"category_name":35,"doc_title":65,"doc_description":67,"doc_content":134,"file_id":135,"file_url":136,"file_type":137,"file_size":138,"view_count":81,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":139,"language":140,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":141,"faqs":142,"seo_title":143,"seo_description":67,"update_tm":144,"read_time":145},2336475401981,"https://ap-avatar.wpscdn.com/avatar/22000c94efd8d5204d?x-image-process=image/resize,m_fixed,w_180,h_180&k=1786935347598174694","Downloaded from [https://continuum.aan.com by Universit](https://continuum.aan.com by Universit)é degli Studi di Napoli Federico II on 5 October 2026  \nInfl ammatory and Autoimmune Axonal Neuropathies  \nBy Qihua Fan, MD; Vinay Chaudhry, MD, FAAN  \nABSTRACT  \nOBJECTIVE: This article outlines the clinical patterns, diagnostic strategies, and treatment principles for patients with inflammatory and autoimmune axonal neuropathies.  \nLATEST 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.  \nESSENTIAL 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.  \nINTRODUCTION  \nI  \nnflammatory and autoimmune axonal polyneuropathies require a high degree of suspicion for diagnosis, rigorous evaluation, iterative evaluation of treatment response, and reassessment of differential diagnoses in cases where there is no pathologically definite confirmation. This article focuses on a bedside approach to the recognition, diagnosis, and treatment of  \ninflammatory axonal neuropathies. For information on axonal variants of Guillain-Barr syndrome, refer to the article “Guillain-Barr Syndrome” by Mamatha Pasnoor, MD, FAAN,1 in this issue of Continuum.  \nAs with other peripheral neuropathies, inflammatory axonal neuropathiescan be characterized by tempo, anatomic distribution, affected fiber type,  \nREVIEW ARTICLE  \nCONTINUUM AUDIO INT ERVIEW AVAI LABLEON LINE  \nCONTINUUM ALOUD RECORDING AVAI LABLEON LINE  \nC ITE AS :  \nCONTINUUM (MIN NEA P M INN) 2026;32(5, PERIPHERAL NERVE AND MOTOR NEURON DISORDERS):1365–1391.  \nAddress correspondence to Dr Qihua Fan, 1101 E Marshall St, 6th Floor, Richmond, VA 23298, [qihua.fan@](qihua.fan@)[ ](qihua.fan@)[vcuhealth.org](vcuhealth.org).  \nRELATIONSHIP DISCLOSURE: Dr Fan has received personal compensation in the range of $500 to $4999 for serving as a consultant for argenx and on a speakers bureau for Alnylam Pharmaceuticals, Inc, and AstraZeneca. Dr Chaudhry has received intellectual property interests from discoveries or technologies relating to health care.  \nUNLABELED USE OF PRODUCTS/ INVESTIGATIONAL USE DISCLOSURE:  \nDrs Fan and Chaudhry report no disclosures.  \nAll cases in this article are hypothetical or composite.  \n© 2026 American Academy of Neurology.  \nCONTINUUM . AAN . COM 1365  \nCopyright © 2026 American Academy of Neurology. Unauthorized reproduction of this article is prohibited.  \nINFLAMMATORY AND AUTOIMMUNE AXONAL NEUROPATHIES  \n1366  \nDownloaded from [https://continuum.aan.com by Universit](https://continuum.aan.com by Universit)é degli Studi di Napoli Federico II on 5 October 2026  \nelectrophysiologic features, and laboratory studies including antibodies (TABLE 4-1) . The namesake provides a helpful framework: an axonal pattern should be confirmed by electrodiagnostic testing, and an inflammatory etiology should follow an inflammatory mechanism or show treatment responsiveness to approp","cbCaiqCXGOF5aVbT","https://ap.wps.com/l/cbCaiqCXGOF5aVbT","pdf",916523,27,"English","# Abstract\n# Introduction\n# Clinical Presentation\n## Mononeuritis multiplex or multiple mononeuropathies\n## Asymmetric distal sensorimotor axonal polyneuropathy\n## Sensory ataxia\n## Lumbosacral or cervicobrachial radiculoplexus neuropathy","[{\"question\":\"What conditions are included under inflammatory and autoimmune axonal neuropathies?\",\"answer\":\"They encompass a spectrum of immune-mediated disorders presenting with mononeuritis multiplex, asymmetric sensorimotor polyneuropathy, sensory ataxia, or plexopathy. The article stresses immune mechanisms and clinical presentation patterns.\"},{\"question\":\"How are diagnosis and axonal pattern confirmation approached?\",\"answer\":\"Diagnosis requires high clinical suspicion, detailed electrodiagnostic evaluation, and consideration of systemic features. Nerve biopsy can help confirm the diagnosis and guide immunotherapy despite limited patchy yield.\"},{\"question\":\"What are the key treatment principles and follow-up elements?\",\"answer\":\"Treatment aims to halt inflammation and prevent relapses, using corticosteroids as first-line induction and steroid-sparing agents for maintenance. A structured follow-up with standardized neurologic exams, functional assessments, and ancillary testing is essential to track progression and response.\"}]","Inflammatory and Autoimmune Axonal Neuropathies | PDF",1791670256,68]