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

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

doi:10.1016/j.jneuroim.2018.04.016

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

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

Research output: Contribution to journal › Article › peer-review

31 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 language	English (US)
Pages (from-to)	157-163
Number of pages	7
Journal	Journal of neuroimmunology
Volume	321
DOIs	https://doi.org/10.1016/j.jneuroim.2018.04.016
State	Published - Aug 15 2018

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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10.1016/j.jneuroim.2018.04.016

Cite this

@article{7481ecb8aa024af98a52b05440924634,

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.",

keywords = "Autoimmune, Cerebrospinal fluid, Glial fibrillary astrocytic protein, Meningoencephalomyelitis, Pediatric",

author = "Divyanshu Dubey and Hinson, {Shannon R.} and Jolliffe, {Evan A.} and Anastasia Zekeridou and Flanagan, {Eoin P.} and Pittock, {Sean J.} and Eati Basal and Drubach, {Daniel A.} and Lachance, {Daniel H.} and Lennon, {Vanda A.} and Andrew McKeon",

note = "Publisher Copyright: {\textcopyright} 2018 Elsevier B.V.",

year = "2018",

month = aug,

day = "15",

doi = "10.1016/j.jneuroim.2018.04.016",

language = "English (US)",

volume = "321",

pages = "157--163",

journal = "Journal of neuroimmunology",

issn = "0165-5728",

publisher = "Elsevier",

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TY - JOUR

T1 - Autoimmune GFAP astrocytopathy

T2 - Prospective evaluation of 90 patients in 1 year

AU - Dubey, Divyanshu

AU - Hinson, Shannon R.

AU - Jolliffe, Evan A.

AU - Zekeridou, Anastasia

AU - Flanagan, Eoin P.

AU - Pittock, Sean J.

AU - Basal, Eati

AU - Drubach, Daniel A.

AU - Lachance, Daniel H.

AU - Lennon, Vanda A.

AU - McKeon, Andrew

PY - 2018/8/15

Y1 - 2018/8/15

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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U2 - 10.1016/j.jneuroim.2018.04.016

DO - 10.1016/j.jneuroim.2018.04.016

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AN - SCOPUS:85047067413

SN - 0165-5728

VL - 321

SP - 157

EP - 163

JO - Journal of neuroimmunology

JF - Journal of neuroimmunology

ER -

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

Abstract

Keywords

ASJC Scopus subject areas

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Cite this

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

Abstract

Keywords

ASJC Scopus subject areas

Access to Document

Other files and links

Fingerprint

Cite this

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