Autoimmune GFAP astrocytopathy: Prospective evaluation of 90 patients in 1 year

Divyanshu Dubey, Shannon R. Hinson, Evan A. Jolliffe, Anastasia Zekeridou, Eoin Flanagan, Sean J Pittock, Eati Basal, Daniel Drubach, Daniel H Lachance, Vanda A Lennon, Andrew McKeon

Research output: Contribution to journalArticle

13 Scopus citations

Abstract

In this prospective evaluation of serum and CSF samples, all but two CSF GFAPα-IgG positive patients had autoimmune meningoencephalomyelitis while serum GFAPα-IgG positivity alone was less specific. Phenotypes were diverse among patients that were serum positive only. Adult and pediatric clinical presentations were similar. Most patients were immunotherapy responsive. Co-existing NMDA-R-IgG and cancer were associated with lack of response to first-line immunotherapy. Among patients with follow-up information, 18% had relapses. This study demonstrates CSF GFAPα-IgG is a specific autoimmune meningoencephalomyelitis biomarker, with favorable corticosteroid response. Lack of response should prompt evaluation for co-existing NMDA-R-IgG or malignancy.

Original languageEnglish (US)
JournalJournal of Neuroimmunology
DOIs
StateAccepted/In press - Jan 1 2018

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Keywords

  • Autoimmune
  • Cerebrospinal fluid
  • Glial fibrillary astrocytic protein
  • Meningoencephalomyelitis
  • Pediatric

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology
  • Neurology
  • Clinical Neurology

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