Highlights
- •Anti-NMDAR encephalitis and MOG ab related disease can occur independently or simultaneously.
- •Children with typical anti-NMDAR encephalitis can combine with positive MOG ab only.
- •More clinical overlaps exist between NMDAR ab(+)/MOG ab(+) group and NMDAR ab(+)/MOG ab(-) group.
- •More MRI overlaps exist between NMDAR ab(+)/MOG ab(+) group and MOG ab(+)/NMDAR ab(-) group.
- •MOG ab is associated with relapse, and NMDAR ab is associated with neurological sequelae.
Abstract
Objective
To analyze the clinical features in children with anti-NMDAR encephalitis combined
with myelin oligodendrocyte glycoprotein antibody (MOG ab).
Methods
Clinical data of 7 children with anti-NMDAR encephalitis combined with MOG ab(+) were
collected in Guangzhou Women and Children's Medical Center from January, 2016 to June,
2019. Children with NMDAR ab(+)/MOG ab(-) and MOG ab(+)/NMDAR ab(-) were randomly
selected as controls.
Results
Onset age was 6.0 (IQR 5.0–7.0) years old, male to female was 2:5. Prominent symptoms
include abnormal mental behavior (7/7), sleep disorder (6/7), speech disorder (6/7),
involuntary movement (4/7) and paralysis (4/7). There were significant differences
between NMDAR ab(+)/MOG ab(+) group versus MOG ab(+)/NMDAR ab(-) and NMDAR ab(+)/MOG
ab(-) group versus MOG ab(+)/NMDAR ab(-) group (P< 0.0167, Fisher exact tests) in abnormal mental behavior, sleep disorder, speech
disorder and involuntary movement. 1 case developed anti-NMDAR encephalitis 1 year
after recovery from MOG ab related acute disseminated encephalomyelitis (ADEM). 4
cases developed anti-NMDAR encephalitis and MOG ab related ADEM simultaneously, with
2 cases relapsed. 2 cases were anti-NMDAR encephalitis with only MOG ab positive.
In terms of MRI, there were differences in subcortical white matter, basal ganglia
and brainstem (P < 0.0167, Fisher exact tests) between NMDAR ab(+)/MOG ab(+) group versus NMDAR ab(+)/MOG
ab(-) (P < 0.0001) and NMDAR ab(+)/MOG ab(-) group versus MOG ab(+)/NMDAR ab(-) group(P<0.0001). There were significant differences in MOG antibody titer (Z = −=2.03, P = 0.042) and duration (Z = −1.97, P = 0.049) between relapsed and non-relapsed patients. 3 cases had neurological sequelae.
The differences of NMDAR antibody titer (Z = −2.22, P = 0.026) and duration (Z = −2.18, P = 0.029) were significant between patients with and without neurological sequelae.
Conclusion
NMDAR and MOG antibodies can coexist in children with autoimmune encephalitis. Double
antibody positive subjects had more overlaps in clinical manifestations with NMDAR
encephalitis, and more overlaps in MRI changes with MOG ab related disease. Higher
persistent MOG antibody titer may indicate recurrence, while higher persistent NMDAR
antibodies titer may cause neurological sequelae.
Keywords
Abbreviations:
Ab (antibody), ADEM (acute disseminated encephalomyelitis), AQP4 (aquaporin-4), CNS (central nervous system), CSF (cerebrospinal fluid), EEG (electroencephalogram), IVIG (intravenous immunoglobulin), IVMP (intravenous methylprednisolone), MOG (myelin oligodendrocyte glycoprotein), MRI (magnetic resonance imaging), mRS (modified Rankin score), MDEM (multiphasic disseminated encephalomyelitis), NMDAR (N-methyl-d-aspartate receptor), NMOSD (optic myelomyelitis spectrum disorder), Pro (protein), WBC (white blood cell)To read this article in full you will need to make a payment
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Article info
Publication history
Published online: February 22, 2020
Accepted:
February 21,
2020
Received in revised form:
February 7,
2020
Received:
December 8,
2019
Identification
Copyright
© 2020 Elsevier B.V. All rights reserved.