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

被引：125

作者：

Dubey, Divyanshu ^{[1
]}

Hinso, Shannon R. ^{[2
,3
]}

Jolliffe, Evan A. ^{[1
]}

Zekeridou, Anastasia ^{[1
]}

Flanagan, Eoin P. ^{[1
]}

Pittock, Sean J. ^{[1
,2
,3
]}

Basal, Eati ^{[2
,3
]}

Drubach, Daniel A. ^{[1
]}

Lachance, Daniel H. ^{[1
]}

Lennon, Vanda A. ^{[1
,2
,3
,4
]}

McKeon, Andrew ^{[1
,2
,3
]}

机构：

[1] Mayo Clin, Dept Neurol, Rochester, MN 55905 USA

[2] Mayo Clin, Dept Lab Med, Rochester, MN 55905 USA

[3] Mayo Clin, Dept Pathol, Rochester, MN 55905 USA

[4] Mayo Clin, Dept Immunol, Rochester, MN 55905 USA

来源：

JOURNAL OF NEUROIMMUNOLOGY | 2018年 / 321卷

关键词：

Autoimmune; Meningoencephalomyelitis; Glial fibrillary astrocytic protein; Pediatric; Cerebrospinal fluid; ACIDIC PROTEIN ASTROCYTOPATHY; INFECTIOUS ENCEPHALITIS; EPIDEMIOLOGY; DIAGNOSIS;

D O I：

10.1016/j.jneuroim.2018.04.016

中图分类号：

R392 [医学免疫学]; Q939.91 [免疫学];

学科分类号：

100102 ;

摘要：

In this prospective evaluation of serum and CSF samples, all but two CSF GFAP alpha-lgG positive patients had autoimmune meningoencephalomyelitis while serum GFAP alpha-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 GFAP alpha-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.

引用

收藏

页码：157 / 163

页数：7

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