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

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13 Citations (Scopus)

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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Immunoglobulin G
N-Methylaspartate
Immunotherapy
Serum
Neoplasms
Adrenal Cortex Hormones
Biomarkers
Pediatrics
Phenotype
Recurrence

Keywords

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

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology
  • Neurology
  • Clinical Neurology

Cite this

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title = "Autoimmune GFAP astrocytopathy: Prospective evaluation of 90 patients in 1 year",
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.",
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author = "Divyanshu Dubey and Hinson, {Shannon R.} and Jolliffe, {Evan A.} and Anastasia Zekeridou and Eoin Flanagan and Pittock, {Sean J} and Eati Basal and Daniel Drubach and Lachance, {Daniel H} and Lennon, {Vanda A} and Andrew McKeon",
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AU - Dubey, Divyanshu

AU - Hinson, Shannon R.

AU - Jolliffe, Evan A.

AU - Zekeridou, Anastasia

AU - Flanagan, Eoin

AU - Pittock, Sean J

AU - Basal, Eati

AU - Drubach, Daniel

AU - Lachance, Daniel H

AU - Lennon, Vanda A

AU - McKeon, Andrew

PY - 2018/1/1

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N2 - 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.

AB - 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.

KW - Autoimmune

KW - Cerebrospinal fluid

KW - Glial fibrillary astrocytic protein

KW - Meningoencephalomyelitis

KW - Pediatric

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