Pediatric anti-NMDAR encephalitis with demyelination on brain MRI: A single center study
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Yuhang Li1, Hanyu Luo1, Yaxin Zheng1
1Department of Neurology, Children's Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing Key Laboratory of Paediatrics, 2 Zhongshan Rd, Chongqing 400013, China.
Objective:
To explore the clinical characteristics, immunotherapy response, and prognosis of pediatric anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis associated with demyelination on brain magnetic resonance (MRI).
Methods:
We retrospectively reviewed the medical records of children diagnosed with anti-NMDAR encephalitis in our hospital between January 2016 and December 2021. All children with evidence of demyelination on brain MRI were included.
Results:
A total of 183 anti-NMDAR encephalitis children were included; 8.7 % (16/183) of them had demyelination on brain MRI. Nine were positive for myelin oligodendrocyte glycoprotein (MOG)-IgG, while two were positive for both MOG-IgG and glial fibrillary acidic protein (GFAP)-IgG. Four patients had a history of acquired demyelinating syndromes and encephalitis, respectively, while nine (56.3 %) had atypical symptoms of anti-NMDAR encephalitis. All children had supratentorial demyelination on brain MRI; four of them had additional infratentorial lesions. All children received first-line immunotherapy; four were administered repeated first-line immunotherapy and/or rituximab because of poor initial response. During the follow-up, 37.5 % (6/16) of the children relapsed, but all responded well to immunotherapy. There were no significant differences in mRS score before immunotherapy, response to first-line immunotherapy, and long-term prognosis between anti-NMDAR encephalitis children with and without demyelination. However, patients with demyelination were more likely to have a history of acquired demyelinating syndromes or unexplained cortical encephalitis and to relapse.
Conclusion:
Pediatric anti-NMDAR encephalitis can co-occur with demyelination and has a high rate of MOG-IgG positivity. A history of acquired demyelinating syndromes or unexplained cortical encephalitis and atypical symptoms may indicate demyelination in children with anti-NMDAR encephalitis. Pediatric anti-NMDAR encephalitis with demyelination is more likely to relapse and needs a closer follow-up. However, it remains unknown whether more intensive immunotherapy is required in these patients.
Insights
Pediatric anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis can present with demyelination, often associated with myelin oligodendrocyte glycoprotein (MOG)-IgG. These cases may relapse more frequently, necessitating closer monitoring.
Area of Science:
- Neurology
- Immunology
- Pediatrics
Background:
- Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a severe autoimmune neurological disorder.
- Demyelination can occur in various neurological conditions, and its association with anti-NMDAR encephalitis in children is not fully understood.
Purpose of the Study:
- To investigate the clinical features, treatment response, and outcomes of pediatric anti-NMDAR encephalitis cases with concurrent demyelination.
- To identify potential indicators of demyelination in children diagnosed with anti-NMDAR encephalitis.
Main Methods:
- Retrospective review of medical records of children diagnosed with anti-NMDAR encephalitis between January 2016 and December 2021.
- Inclusion criteria: diagnosis of anti-NMDAR encephalitis and evidence of demyelination on brain magnetic resonance imaging (MRI).
- Analysis of clinical characteristics, immunotherapy response, relapse rates, and long-term prognosis.
Main Results:
- 8.7% of pediatric anti-NMDAR encephalitis patients showed demyelination on MRI.
- Nine patients tested positive for myelin oligodendrocyte glycoprotein (MOG)-IgG; two were positive for both MOG-IgG and glial fibrillary acidic protein (GFAP)-IgG.
- Patients with demyelination were more likely to have a history of acquired demyelinating syndromes or unexplained cortical encephalitis and exhibited a higher relapse rate, though all responded to immunotherapy.
Conclusions:
- Pediatric anti-NMDAR encephalitis can co-occur with demyelination, frequently associated with MOG-IgG positivity.
- A history of demyelinating syndromes or atypical encephalitis symptoms may suggest co-existing demyelination.
- While demyelination increases relapse risk, requiring closer follow-up, the need for more intensive immunotherapy remains uncertain.
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