Evaluation of the 2015 diagnostic criteria for neuromyelitis optica spectrum disorder

Neurology. 2016 May 10;86(19):1772-9. doi: 10.1212/WNL.0000000000002655. Epub 2016 Apr 13.


Objective: To evaluate the application of the 2015 International Panel for NMO Diagnosis (IPND) criteria to consecutive cases of neuromyelitis optica spectrum disorder (NMOSD) in a large cohort of individuals with CNS inflammatory diseases.

Methods: In total, 594 patients with CNS inflammatory diseases were included. Rigorous confirmation of the patients' aquaporin-4 immunoglobulin G antibodies (AQP4-IgG) status throughout the disease duration (mean 9.2 ± 5.7 years) using repeated assays, including ELISA and cell-based assay, was performed.

Results: A total of 252 patients fulfilled the IPND criteria (AQP4-IgG positive: 226 [90%], AQP4-IgG negative: 26 [10%]). Of these, 136 (54%) patients met the 2006 neuromyelitis optica criteria. When we assumed an unknown AQP4-IgG status in the confirmed NMOSD group with AQP4-IgG, 162 of 226 (72%) patients with AQP4-IgG were classified as having NMOSD by the IPND criteria. The majority of patients were diagnosed with NMOSD within 2 years of onset (73%) or after a second attack (72%). Acute myelitis (83%) and optic neuritis (65%) were the most common clinical features throughout the disease duration. Optic neuritis (42%) was the most common initial manifestation, followed by acute myelitis (38%) and area postrema syndrome (14%).

Conclusions: The IPND criteria well-reflected the broader clinical spectrum of NMOSD and markedly improved the diagnostic yield compared to the previous criteria, even in patients with an unknown AQP4-IgG status.

Publication types

  • Evaluation Study

MeSH terms

  • Adult
  • Aquaporin 4 / immunology
  • Diagnosis, Differential
  • Enzyme-Linked Immunosorbent Assay
  • Female
  • Follow-Up Studies
  • Humans
  • Immunoglobulin G / metabolism
  • Male
  • Neuromyelitis Optica / diagnosis*
  • Neuromyelitis Optica / physiopathology
  • Retrospective Studies


  • AQP4 protein, human
  • Aquaporin 4
  • Immunoglobulin G