Myelin oligodendrocyte glycoprotein and aquaporin-4 antibodies are highly specific in children with acquired demyelinating syndromes


AIM: Our objectives were to evaluate the utility of measuring myelin oligodendrocyte glycoprotein (MOG) and aquaporin-4 (AQP4) antibodies (Ab) in clinical practice and describe their associated neurological phenotypes in children. METHOD: Between 2012 and 2017, 371 children with suspected acquired demyelinating syndromes (ADS) seen in three tertiary centres were tested for MOG-Ab and AQP4-Ab. Medical notes were retrospectively reviewed, and clinical and demographic data compiled. Clinical phenotyping was performed blinded to the antibody results. RESULTS: After review, 237 of the 371 were diagnosed with ADS. Of these, 76 out of 237 (32.1%) were MOG-Ab positive and 14 out of 237 (5.9%) were AQP4-Ab positive. None were positive for both autoantibodies. All 134 patients with non-ADS were negative for MOG-Ab. MOG-Ab were identified in 45 out of 70 (64.3%) patients presenting with acute disseminated encephalomyelitis (ADEM) and in 24 out of 25 patients with relapsing ADEM. Thirty-six out of 75 (48%) MOG-Ab positive patients relapsed. Of the 33 children with neuromyelitis optic spectrum disorder, 14 were AQP4-Ab positive, 13 were MOG-Ab positive, and 6 were seronegative. Of the children with longitudinal samples, 8 out of 13 AQP4-Ab remained positive during the disease course compared to 35 out of 43 MOG-Ab (13/16 monophasic and 22/27 relapsing). INTERPRETATION: Myelin oligodendrocyte glycoprotein antibodies were identified in a third of children with ADS. Almost half of the MOG-Ab positive children relapsed and the majority of them remained antibody positive over 4-years follow-up. WHAT THIS PAPER ADDS: Myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) are highly specific for acquired demyelinating syndromes (ADS). Myelin oligodendrocyte glycoprotein antibodies are not identified in children with peripheral demyelination or genetic leukodystrophies/hypomyelination. Up to 48% of MOG-Ab ADS paediatric patients relapse, higher than previously thought. Seroconversion to MOG-Ab negative status is infrequent; patients may test MOG-Ab positive at follow-up sampling even when asymptomatic. Myelin oligodendrocyte glycoprotein antibodies status should only be used in conjunction with the clinical information to guide maintenance therapy.

Publication DOI:
Divisions: College of Health & Life Sciences > Aston Pharmacy School
College of Health & Life Sciences
Additional Information: This is the peer reviewed version of the following article: Duignan, S. , Wright, S. , Rossor, T. , Cazabon, J. , Gilmour, K. , Ciccarelli, O. , Wassmer, E. , Lim, M. , Hemingway, C. and Hacohen, Y. (2018), Myelin oligodendrocyte glycoprotein and aquaporin‐4 antibodies are highly specific in children with acquired demyelinating syndromes. Dev Med Child Neurol, 60: 958-962, which has been published in final form at  This article may be used for non-commercial purposes in accordance With Wiley Terms and Conditions for self-archiving.
Publication ISSN: 1469-8749
Last Modified: 26 Feb 2024 08:23
Date Deposited: 16 Mar 2018 14:05
Full Text Link: http://discover ...
Related URLs: http://onlineli ... 588BBE18.f03t02 (Publisher URL)
PURE Output Type: Article
Published Date: 2018-09-01
Published Online Date: 2018-02-22
Accepted Date: 2018-01-03
Authors: Duignan, Sophie
Wright, Sukhvir (ORCID Profile 0000-0002-5464-3779)
Rossor, Tom
Cazabon, John
Gilmour, Kimberly
Ciccarelli, Olga
Wassmer, Evangeline
Lim, Ming
Hemingway, Cheryl
Hacohen, Yael



Version: Accepted Version

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