Clinical analysis of anti-NMDAR encephalitis combined with MOG antibody in children
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Chi Hou1, Wenlin Wu1, Yang Tian1
1Department of Neurology, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, The 9# Jin Sui Road, Guangzhou, Guangdong 510623, China.
Insights
Children with autoimmune encephalitis can have both N-methyl-D-aspartate receptor (NMDAR) and myelin oligodendrocyte glycoprotein (MOG) antibodies. Co-infection presents unique clinical and MRI features, with higher MOG antibody titers potentially indicating recurrence and higher NMDAR titers linked to sequelae.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Autoimmune Encephalitis
Background:
- Autoimmune encephalitis is a group of disorders characterized by inflammation of the brain due to autoimmune processes.
- Antibodies targeting N-methyl-D-aspartate receptors (NMDAR) and myelin oligodendrocyte glycoprotein (MOG) are significant causes of autoimmune encephalitis in children.
- Co-occurrence of NMDAR and MOG antibodies presents a complex diagnostic and therapeutic challenge.
Purpose of the Study:
- To investigate the clinical characteristics and outcomes of pediatric patients with coexisting anti-NMDAR and MOG antibodies.
- To compare the clinical and MRI findings in children with dual antibody positivity versus those with single antibody positivity.
Main Methods:
- A retrospective analysis of clinical data from 7 children with anti-NMDAR encephalitis and MOG antibodies (MOG ab(+)) was conducted.
- Control groups included children with NMDAR antibodies only (NMDAR ab(+)/MOG ab(-)) and MOG antibodies only (MOG ab(+)/NMDAR ab(-)).
- Clinical symptoms, MRI findings, antibody titers, and outcomes were analyzed using Fisher exact tests and other statistical methods.
Main Results:
- Children with coexisting NMDAR and MOG antibodies exhibited prominent symptoms including abnormal mental behavior, sleep disturbances, speech disorders, and involuntary movements.
- Significant differences in clinical manifestations and MRI findings (subcortical white matter, basal ganglia, brainstem) were observed between the dual-positive group and single-positive control groups.
- Higher MOG antibody titers were associated with disease recurrence, while elevated NMDAR antibody titers correlated with neurological sequelae.
Conclusions:
- NMDAR and MOG antibodies can coexist in pediatric autoimmune encephalitis, leading to overlapping clinical and radiological features.
- Dual antibody positivity shows a higher overlap in clinical symptoms with NMDAR encephalitis and in MRI findings with MOG antibody-associated disease.
- Persistent high titers of MOG antibodies may predict relapse, whereas persistent high NMDAR antibody titers are linked to neurological sequelae.
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.
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