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The article reviews clinical and radiologic phenotypes, diagnostic and treatment approaches, and pathogenesis, including differential diagnoses and the role of novel biomarkers. Key advances include discovery of serum AQP4-targeting antibodies in most patients, development of dedicated clinical trials with new targeted drugs, and ongoing revision of diagnostic criteria.",{"@graph":69,"@context":123},[70,84,106],{"@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/neuromyelitis-optica-spectrum-disorder-review-article/424002/",{"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/neuromyelitis-optica-spectrum-disorder-review-article/424002.png","ImageObject",300,407,{"name":92,"@type":93},"Aria","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-10-30","2026-09-28",true,{"@type":102,"interactionType":103,"userInteractionCount":105},"InteractionCounter",{"@type":104},"ViewAction",12,{"@type":107,"mainEntity":108},"FAQPage",[109,115,119],{"name":110,"@type":111,"acceptedAnswer":112},"What is the main diagnostic basis for NMOSD?","Question",{"text":113,"@type":114},"NMOSD diagnosis relies on detecting serum antibodies targeting aquaporin-4 (AQP4), though a subgroup of patients may be seronegative.","Answer",{"name":116,"@type":111,"acceptedAnswer":117},"How have recent developments affected NMOSD treatment?",{"text":118,"@type":114},"Dedicated clinical trials have led to the approval of new targeted drugs, reinforcing the importance of prompt and accurate recognition of NMOSD.",{"name":120,"@type":111,"acceptedAnswer":121},"Why is recognizing rare phenotypes of NMOSD important?",{"text":122,"@type":114},"Correct identification of atypical or limited presentations supports appropriate diagnostic and treatment decisions, improving patient outcomes despite the condition’s rarity.","https://schema.org",{"og:url":83,"og:type":125,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":127,"canonical":83},"index,follow",{"doc_id":129,"site_id":62},424002,1790763135,{"code":4,"msg":5,"data":132},{"doc_id":129,"user_id":133,"nickname":92,"user_avatar":134,"doc_module":4,"category_id":34,"category_name":35,"doc_title":65,"doc_description":67,"doc_content":135,"file_id":136,"file_url":137,"file_type":138,"file_size":139,"view_count":105,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":140,"language":141,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":142,"faqs":143,"seo_title":144,"seo_description":67,"update_tm":145,"read_time":146,"preview_page_count":4},2336464648322,"https://ap-avatar.wpscdn.com/avatar/2200025388227c56fec?_k=1778556882303663488","Downloaded from [https://continuum.aan.com by Mahidol University on 20 June 2026](https://continuum.aan.com by Mahidol University on 20 June 2026)  \nNeuromyelitis Optica Spectrum Disorder  \nBy Sara Mariotto, MD, PhD; Romain Marignier, MD, PhD  \nABSTRACT  \nOBJECTIVE: This article reviews the clinical features of and diagnostic and treatment approaches to neuromyelitis optica spectrum disorder (NMOSD), with additional focus on rare phenotypes and specific contexts. Pathogenesis and pathology, differential diagnoses, and the potential use of novel biomarkers are also discussed.  \nLATEST DEVELOPMENTS: A major advancement in the characterization of NMOSD was the discovery of serum antibodies targeting the water channel aquaporin-4 in the majority of patients, which also improved the characterization of disease pathogenesis. A proper diagnosis is fundamental to differentiating NMOSD from other central nervous system inflammatory conditions and starting prompt treatment. The recent development of dedicated clinical trials led to the approval of new targeted drugs and further emphasized the relevance of properly recognizing this disease. The ongoing revision of NMOSD diagnostic criteria will provide additional guidance.  \nESSENTIAL POINTS: Although rare, recognizing NMOSD is crucial for improving patient outcomes through correct diagnostic and treatment approaches. Reports of atypical forms and increasing knowledge of clinical, imaging, and laboratory-specific features are fundamental for the accurate recognition of this condition. Research on targeted therapies and biomarkers measuring and predicting disease activity will improve NMOSD management.  \nINTRODUCTION  \nN  \neuromyelitis optica spectrum disorder (NMOSD) is a rare but distinct inflammatory central nervous system disorder with a relapsing disease course that is frequently associated with residual disability. NMOSD is usually characterized by optic nerve, spinal cord, or area postrema involvement associated with  \ncompatible radiologic lesions. The diagnosis is based on the detection of serum antibodies targeting the water channel aquaporin-4 (AQP4), which may be negative in a subgroup of patients. Prompt acute treatment and the administration of chronic immunosuppression improve patient outcomes. This article reviews the pathogenesis, epidemiology, clinical and radiologic phenotypes, and disease course of NMOSD. Diagnostic and treatment approaches are also discussed, with additional focus on novel biomarkers and  \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(2, MULTIPLE SCLEROSIS AND RELATED DISORDERS):513–540.  \nAddress correspondence to Dr Romain Marignier, Hopital Neurologique, 59 Boulevard Pinel 69677, Lyon, Bron Cedex, France, [romain.marignier@chu-lyon.fr](romain.marignier@chu-lyon.fr).  \nRELATIONSHIP DISCLOSURE: Dr Mariotto has received personal compensation in the range of $5000 to $9999 for serving on a speakers bureau for Alexion Pharmaceuticals, Inc, the American Academy of Neurology (AAN), Amgen Inc, Biogen, Brain Dynamics Academy, F. Hoffmann-La Roche Ltd, Novartis AG, Sanofi , TSF HealthScience, and UCBS.A.  \nContinued on page 540  \nUNLABELED USE OF PRODUCTS/ INVESTIGATIONAL USE DISCLOSURE:  \nDrs Mariotto and Marignier discuss the unlabeled use of azathioprine, methotrexate, mycophenolate mofetil, andrituximab for the treatment of neuromyelitis optica spectrum disorder.  \nAll cases in this article are hypothetical or composite.  \n© 2026 American Academy of Neurology.  \nCONTINUUM . AAN . COM 513  \nCopyright © 2026 American Academy of Neurology. Unauthorized reproduction of this article is prohibited.  \nNEUROMYELITIS OPTICA SPECTRUM DISORDER  \n514  \nDownloaded from [https://continuum.aan.com by Mahidol University on 20 June 2026](https://continuum.aan.com by Mahidol University on 20 June 2026)  \nred flags requiring a proper differential diagnosis. NMOSD occu","cbCaidXSttTPZttz","https://ap.wps.com/l/cbCaidXSttTPZttz","pdf",969892,28,"English","# Abstract\n# Introduction\n# History\n# Epidemiology\n# Essential Points\n# Latest Developments","[{\"question\":\"What is the main diagnostic basis for NMOSD?\",\"answer\":\"NMOSD diagnosis relies on detecting serum antibodies targeting aquaporin-4 (AQP4), though a subgroup of patients may be seronegative.\"},{\"question\":\"How have recent developments affected NMOSD treatment?\",\"answer\":\"Dedicated clinical trials have led to the approval of new targeted drugs, reinforcing the importance of prompt and accurate recognition of NMOSD.\"},{\"question\":\"Why is recognizing rare phenotypes of NMOSD important?\",\"answer\":\"Correct identification of atypical or limited presentations supports appropriate diagnostic and treatment decisions, improving patient outcomes despite the condition’s rarity.\"}]","Neuromyelitis Optica Spectrum Disorder - Review Article | PDF",1790632631,71]