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Anti-NMDAR encephalitis secondary to acute necrotizing encephalopathy caused by herpes simplex virus infection in
Jia Zhang1, Jinfeng Liu1, Jianjun Wang1
1Department of Pediatrics, West China Second University Hospital, Sichuan University, Chengdu 610041, China; Key Laboratory of Obstetrics & Gynecologic and Pediatric Diseases and Birth Defects of the Ministry of Education, Sichuan University, Chengdu, Sichuan, China; Key Laboratory of Development and Maternal and Child Diseases of Sichuan Province, China.
Insights
Herpes simplex virus encephalitis in infants can lead to acute necrotizing encephalopathy and secondary anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis. Early immunotherapy is crucial for improved outcomes in these cases.
Area of Science:
- Neurology
- Pediatric Neurology
- Infectious Diseases
Background:
- Herpes simplex virus (HSV) encephalitis is a severe neurological infection in infants.
- Acute necrotizing encephalopathy (ANE) is a rare but devastating complication of HSV encephalitis.
- Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is an autoimmune disorder affecting the central nervous system.
Observation:
- Four infants with HSV-1 encephalitis presented with fever, seizures, and altered consciousness.
- Clinical course included progressive neurological deterioration, elevated CSF protein, and necrotizing encephalopathy on MRI.
- Initial treatments with acyclovir, dexamethasone, and immunoglobulins showed limited efficacy.
Findings:
- CSF analysis revealed positive anti-NMDAR IgG antibodies weeks to months after initial infection.
- High-dose methylprednisolone therapy led to gradual clinical improvement.
- This suggests a secondary autoimmune process triggered by HSV encephalitis.
Implications:
- Recognition of secondary anti-NMDAR encephalitis in infants with HSV-associated ANE is critical.
- Prompt immunotherapy, after excluding active CNS infection, can improve patient outcomes.
- This highlights the complex interplay between viral infections and autoimmune neurological disorders in children.
Background:
To describe the clinical characteristics of anti-NMDAR encephalitis secondary to acute necrotizing encephalopathy caused by herpes simplex virus encephalitis in infants, and aid in its early recognition, diagnosis and treatment.
Case Presentation:
A total of 4 infants were included; all presented with fever, seizures, and progressive disturbances of consciousness and were diagnosed with herpes simplex virus (HSV-1) encephalitis. Cerebrospinal fluid (CSF) protein levels progressively increased, and the head MRI showed necrotizing encephalopathy. There was no significant improvement or recurrence after treatment with acyclovir, dexamethasone, or immunoglobulins. CSF reexamination at 3 weeks to 3 months showed positive anti-NMDAR IgG antibodies and gradual improvement after high-dose methylprednisolone therapy.
Conclusion:
Infants with ANE associated with HSV can develop secondary anti-NMDAR encephalitis, recognition of which is critical to ensure the appropriate institution of immunotherapy after active CNS infection has been ruled out.
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