Updated estimate of AQP4-IgG serostatus and disability outcome in neuromyelitis optica

Yujuan Jiao, James P. Fryer, Vanda A. Lennon, Sarah M. Jenkins, Amy M L Quek, Carin Y. Smith, Andrew McKeon, Chiara Costanzi, Raffaele Iorio, Brian G. Weinshenker, Dean M. Wingerchuk, Elizabeth A. Shuster, Claudia F. Lucchinetti, Sean J. Pittock

Research output: Contribution to journalArticlepeer-review


Objective: To 1) determine, using contemporary recombinant antigen-based assays, the aquaporin-4 (AQP4)-immunoglobulin G (IgG) detection rate in sequential sera of patients assigned a clinical diagnosis of neuromyelitis optica (NMO) but initially scored negative by tissue-based indirect immunofluorescence (IIF) assay and 2) evaluate the impact of serostatus on phenotype and outcome. Methods: From Mayo Clinic records (2005-2011), we identified 163 patients with NMO 110 (67%) were seropositive by IIF and 53 (33%) were scored seronegative. Available stored sera from 49 " seronegative" patients were tested by ELISA, AQP4-transfected cell-based assay, and in-house fluorescence-activated cell sorting assay. Clinical characteristics were compared based on final serostatus. Results: Thirty of the 49 IIF-negative patients (61%) were reclassified as seropositive, yielding an overall AQP4-IgG seropositivity rate of 88% (i.e., 12% seronegative). The fluorescence-activated cell sorting assay improved the detection rate to 87%, cell-based assay to 84%, and ELISA to 79%. The sex ratio (female to male) was 1:1 for seronegatives and 9:1 for seropositives (p , 0.0001). Simultaneous optic neuritis and transverse myelitis as onset attack type (i.e., within 30 days of each other) occurred in 32% of seronegatives and in 3.6% of seropositives (p , 0.0001). Relapse rate, disability outcome, and other clinical characteristics did not differ significantly. Conclusions: Serological tests using recombinant AQP4 antigen are significantly more sensitive than tissue-based IIF for detecting AQP4-IgG. Testing should precede immunotherapy. If negative later-drawn specimens should be tested. aqp4-igg-seronegative nmo is less frequent than previously reported and is clinically similar to aqp4-igg-seropositive NMO.

Original languageEnglish
Pages (from-to)1197-1204
Number of pages8
Issue number14
Publication statusPublished - Oct 1 2013

ASJC Scopus subject areas

  • Clinical Neurology
  • Arts and Humanities (miscellaneous)


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