Objective: The aim of this paper is to report 2 cases with overlapping syndromes in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy. Methods: Antibodies were detected by indirect immunofluorescence assay. Patient data were analyzed retrospectively. Results: One patient presented with overlapping neuromyelitis optica spectrum disorder (NMOSD) and positive GFAP-IgG and aquaporin-4-IgG. His main symptoms included vision loss, hiccups, fever, headache, and ataxia. High leukocyte count and protein levels were found in cerebrospinal fluid. Brain magnetic resonance imaging (MRI) revealed abnormalities in the hippocampus, midbrain, pons, medulla, and meninges. Characteristic radial enhancing patterns were seen. The other patient was a male with relapsing polychondritis (RP) and positive GFAP-IgG. His main manifestations were meningoencephalitis and dementia. MRI showed extensive abnormalities in the white matter around the ventricles, temporal lobe, and thalamus, with enhancement. Both patients responded well to the treatment with steroids and immunosuppressants. Conclusions: Although overlapping syndromes are rare, we report positive GFAP-IgG in 2 cases with NMOSD or RP. Both patients had clinical features of GFAP astrocytopathy, but diagnosis of the condition was very challenging because of the overlapping presentation.

1.
Fang
B
,
McKeon
A
,
Hinson
SR
,
Kryzer
TJ
,
Pittock
SJ
,
Aksamit
AJ
, et al
Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy: A Novel Meningoencephalomyelitis
.
JAMA Neurol
.
2016
Nov
;
73
(
11
):
1297
307
.
[PubMed]
2168-6149
2.
Flanagan
EP
,
Hinson
SR
,
Lennon
VA
,
Fang
B
,
Aksamit
AJ
,
Morris
PP
, et al
Glial fibrillary acidic protein immunoglobulin G as biomarker of autoimmune astrocytopathy: analysis of 102 patients
.
Ann Neurol
.
2017
Feb
;
81
(
2
):
298
309
.
[PubMed]
0364-5134
3.
Long
Y
,
Liang
J
,
Xu
H
,
Huang
Q
,
Yang
J
,
Gao
C
, et al
Autoimmune glial fibrillary acidic protein astrocytopathy in Chinese patients: a retrospective study
.
Eur J Neurol
.
2018
Mar
;
25
(
3
):
477
83
.
[PubMed]
1351-5101
4.
Iorio
R
,
Damato
V
,
Evoli
A
,
Gessi
M
,
Gaudino
S
,
Di Lazzaro
V
, et al
Clinical and immunological characteristics of the spectrum of GFAP autoimmunity: a case series of 22 patients
.
J Neurol Neurosurg Psychiatry
.
2018
Feb
;
89
(
2
):
138
46
.
[PubMed]
0022-3050
5.
Yang
X
,
Liang
J
,
Huang
Q
,
Xu
H
,
Gao
C
,
Long
Y
, et al
Treatment of Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy: Follow-Up in 7 Cases
.
Neuroimmunomodulation
.
2017
;
24
(
2
):
113
9
.
[PubMed]
1021-7401
6.
Yang
X
,
Xu
H
,
Ding
M
,
Huang
Q
,
Chen
B
,
Yang
H
, et al
Overlapping Autoimmune Syndromes in Patients With Glial Fibrillary Acidic Protein Antibodies
.
Front Neurol
.
2018
Apr
;
9
:
251
.
[PubMed]
1664-2295
7.
Liu
T
,
Chen
B
,
Yang
H
,
Huang
J
,
Liu
S
,
Yang
X
, et al
Screening for autoantibodies in inflammatory neurological syndrome using fluorescence pattern in a tissue-based assay: cerebrospinal fluid findings from 793 patients
.
Mult Scler Relat Disord
.
2019
Feb
;
28
:
177
83
.
[PubMed]
2211-0348
8.
Wingerchuk
DM
,
Banwell
B
,
Bennett
JL
,
Cabre
P
,
Carroll
W
,
Chitnis
T
, et al;
International Panel for NMO Diagnosis
.
International consensus diagnostic criteria for neuromyelitis optica spectrum disorders
.
Neurology
.
2015
Jul
;
85
(
2
):
177
89
.
[PubMed]
0028-3878
9.
Cantarini
L
,
Vitale
A
,
Brizi
MG
,
Caso
F
,
Frediani
B
,
Punzi
L
, et al
Diagnosis and classification of relapsing polychondritis
.
J Autoimmun
.
2014
Feb-Mar
;
48-49
:
53
9
.
[PubMed]
0896-8411
10.
Al-Tabbaa
MM
,
Habal
H
.
Relapsing Polychondritis with Meningoencephalitis Refractory to Immunosuppressant Therapy
.
Case Rep Neurol Med
.
2018
Oct
;
2018
:
1873582
.
[PubMed]
2090-6668
11.
Cao
J
,
Zhang
M
.
Pleocytosis in a patient with relapsing polychondritis accompanied by meningoencephalitis: a case report
.
BMC Neurol
.
2018
Apr
;
18
(
1
):
53
.
[PubMed]
1471-2377
12.
Jeon
CH
.
Relapsing Polychondritis with Central Nervous System Involvement: Experience of Three Different Cases in a Single Center
.
J Korean Med Sci
.
2016
Nov
;
31
(
11
):
1846
50
.
[PubMed]
1011-8934
13.
Tsai
M
,
Hu
M
,
Zussman
J
,
Worswick
S
.
Relapsing polychondritis with meningoencephalitis
.
Cutis
.
2017
Jan
;
99
(
1
):
43
6
.
[PubMed]
2326-6929
14.
Hwang
YP
,
Kuo
R
,
Chen
TL
,
Chen
PH
,
Cheng
SJ
.
Relapsing Polychondritis Presenting with Meningoencephalitis and Dementia: Correlation with Neuroimaging and Clinical Features
.
Acta Neurol Taiwan
.
2015
Mar
;
24
(
1
):
30
3
.
[PubMed]
1028-768X
15.
Wang
ZJ
,
Pu
CQ
,
Wang
ZJ
,
Zhang
JT
,
Wang
XQ
,
Yu
SY
, et al
Meningoencephalitis or meningitis in relapsing polychondritis: four case reports and a literature review
.
J Clin Neurosci
.
2011
Dec
;
18
(
12
):
1608
15
.
[PubMed]
0967-5868
16.
Brucki
SM
,
Rocha
MS
.
[Relapsing polychondritis associated with meningoencephalitis: case report]
.
Arq Neuropsiquiatr
.
2001
Sep
;
59
3-A
:
605
8
.
[PubMed]
0004-282X
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.