1 - Pediatric Infectious Diseases Unit, Hospital Dona Estefânia. CHLC-EPE Lisboa. Portugal
2 - Pediatric Neurology Unit, Hospital Dona Estefânia. CHLC-EPE Lisboa. Portugal
3 - Pediatric Rheumatology Unit, Hospital Dona Estefânia. CHLC-EPE Lisboa. Portugal
- reunião internacional – ESPID 2021
Background Anti-NMDAr encephalitis is an immune-mediated disorder. A typical presentation includes acute neuropsychiatric features, abnormal behaviour, movement disorder and autonomic instability. Self-antibodies against NMDA receptors in the brain can be created after trigger mechanisms such as infectious agents and tumours.
Methods A retrospective study between 2012 and 2020 was conducted. Demographic, clinical, complications data, treatment and outcome were analysed
Results We report 12 cases, nine females and three males, with a median age of 8,7 years (min-17 days, max-16 years). Most common manifestations were behaviour changes (8/12), psychiatric disorders (8/12), movement disorders (10/12), insomnia (8/12) and seizures (5/12). NMDA antibody was positive in CSF (12) and in serum (10). An infectious agent was identified in 5 cases: HSV1 (2), HSV2 (1), Mycoplasma pneumoniae (1), HHV7 (2) and adenovirus (1). In one case patient was identified ovarian teratoma and the other four cases were considered cryptogenic, however infections were investigated only in two of these cases. All these patients were female between 14-15 years old. MRI and CSF was normal in most cases (6), the altered MRIs have abnormalities related to the infectious disease. Electroencephalography was abnormal in 9 patients, most of them showing slower activity. Treatment included immunoglobulin in all patients, intravenous methylprednisolone (10) and rituximab (9) and plasmapheresis (4). Cyclophosphamide (2) was used with refractory response to the previous treatment. Sequalae was reported more frequently in post-infection cases (4/7): spastic tetraplegic cerebral palsydiagnosis, behavioural disorders and learning difficulties.
Conclusions Infectious agent, such as HSV and Mycoplasma, should be investigated in all NMDAR encephalitis. It is important an early suspicion and recognition of this disease. Presenting this case review, the authors intend to raise the discussion about infection as trigger to autoimmune response against to NMDA receptors in predisposed patients.
Palavras Chave: Anti-NMDAr encephalitis; autoimmune encephalitis