Differential diagnosis of neuromyelitis optica spectrum disorders

Sung Min Kim, Seong Joon Kim, Haeng Jin Lee, Hiroshi Kuroda, Jacqueline Palace, Kazuo Fujihara

Research output: Contribution to journalReview articlepeer-review

57 Citations (Scopus)


Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory disorder of the central nervous system (CNS) mostly manifesting as optic neuritis and/or myelitis, which are frequently recurrent/bilateral or longitudinally extensive, respectively. As the autoantibody to aquaporin-4 (AQP4-Ab) can mediate the pathogenesis of NMOSD, testing for the AQP4-Ab in serum of patients can play a crucial role in diagnosing NMOSD. Nevertheless, the differential diagnosis of NMOSD in clinical practice is often challenging despite the phenotypical and serological characteristics of the disease because: (1) diverse diseases with autoimmune, vascular, infectious, or neoplastic etiologies can mimic these phenotypes of NMOSD; (2) patients with NMOSD may only have limited clinical manifestations, especially in their early disease stages; (3) test results for AQP4-Ab can be affected by several factors such as assay methods, serologic status, disease stages, or types of treatment; (4) some patients with NMOSD do not have AQP4-Ab; and (5) test results for the AQP4-Ab may not be readily available for the acute management of patients. Despite some similarity in their phenotypes, these NMOSD and NMOSD-mimics are distinct from each other in their pathogenesis, prognosis, and most importantly treatment. Understanding the detailed clinical, serological, radiological, and prognostic differences of these diseases will improve the proper management as well as diagnosis of patients.

Original languageEnglish
Pages (from-to)265-289
Number of pages25
JournalTherapeutic Advances in Neurological Disorders
Issue number7
Publication statusPublished - 2017 Jul 1


  • Devic's disease
  • aquaporin-4 antibody
  • differential diagnosis
  • longitudinally extensive transverse myelitis
  • multiple sclerosis
  • myelin oligodendrocyte glycoprotein antibody
  • neuromyelitis optica spectrum disorders
  • optic neuritis

ASJC Scopus subject areas

  • Pharmacology
  • Neurology
  • Clinical Neurology


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