1 - Unidade de Infeciologia Pediátrica, Hospital de Dona Estefânia
2 - Serviço de Pediatria, Centro Hospitalar de Leiria
3 - Unidade de Neurologia Pediátrica, Hospital de Dona Estefânia
4 - Unidade de Reumatologia Pediátrica, Hospital de Dona Estefânia
- 37th Meeting of the European Society for Paediatric Infectious Diseases (ESPID)
Background: Anti-NMDA receptor encephalitis is the most common autoimmune encephalitis. 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 2018 was conducted. Demographic, clinical, complications data, treatment and outcome were analysed
Results: We report 9 cases, six females and three males, with a median age of 14,5 years (min-15days, max-15years). Most common manifestations were behaviour changes (8/9), psychiatric disorders (7/9), movement disorders (7/9), insomnia (5/9) and seizures (4/9). NMDA antibody was positive in CSF (9) and in serum (5). An infectious agent was identified in four cases: HSV1 (1), HSV2 (1), Mycoplasma pneumoniae and HHV7 (1) and adenovirus (1). In one patient ovarian teratoma was identified and the other four cases were considered cryptogenic. All cryptogenic cases were female between 14-15 years old and infections were investigated only in two. MRI and CSF were normal in most patients (6), the abnormal MRIs had changes related to the infectious disease. Electroencephalography was abnormal in six patients, most of them showing slow activity. Treatment included immunoglobulin in all patients, intravenous methylprednisolone (6), rituximab (7) and plasmapheresis (3). Cyclophosphamide (2) was used in patients refractory to previous treatments. Sequelae were reported more frequently in post-infectious cases (4/6): spastic tetraplegia, behavioural disorders and learning disability.
Conclusions: Infectious agents, such as HSV and Mycoplasma, should be investigated in all patients with 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 a trigger to autoimmunity against NMDA receptors in predisposed patients.
Palavras Chave: Encefalite auto-imune; Recetores NMDA