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2020

ANUÁRIO DO HOSPITAL
DONA ESTEFÂNIA

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ANTI-NMDA RECEPTOR ENCEPHALITIS: CHALLENGES IN DIAGNOSIS AND TREATMENT

Joana Pais de Faria1; Rita Lopes Silva2; Catarina Santos3; Flora Candeias1

1 - Unidade de Infecciologia Pediátrica, Área de Pediatria Médica, Hospital Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central, Lisboa
2 - Serviço de Neurologia Pediátrica, Área de Pediatria Médica, Hospital Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central, Lisboa
3 - Serviço de Psiquiatria da Infância e Adolescência, Hospital Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central, Lisboa

- 11th Excellence in Pediatrics Conference. Copenhaga, Dinamarca, 6 de dezembro de 2019. Comunicação oral

Introduction: Anti-Nmethyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder that is being recognized more often, especially in children and young adults. However, challenges remain in early diagnosis and treatment.
Case description: Previously healthy 11-year-old female presented with tonic-clonic seizure, followed by focal seizures, controlled with intravenous diazepam and levetiracetam. Initial CSF analysis showed lymphocytic pleocytosis (WBC 22cells/mm). Electroencephalogram and brain imaging (CT and MRI) were normal. Acyclovir and ceftriaxone were started empirically. After five days (D5), showed asymmetric choreatic movements predominantly at the distal segment of the right upper limb. Further investigations revealed evidence of past streptococcal infection (anti-streptolysin O 1220UI/mL; Anti-DNase B 737U/mL), rheumatic fever was excluded. PCR in respiratory secretions was positive for adenovirus. At D9 were noticed acute behavioral changes, psychomotor agitation and disfluency. A second lumbar puncture was performed. Anti-NMDAR autoantibodies were positive in CSF (titer 4, reference <1). Intrathecal synthesis and oligoclonal bands were negative. Started methylprednisolone pulses (30mg/kg) for 5 days, followed by oral prednisolone (2mg/kg), combined with rituximab and high dose intravenous immunoglobulin (2g/kg), following the International Inflammatory Brain Disease Outcome Study treatment protocol. Nevertheless, psychomotor agitation persisted, associated with extreme emotional lability, obsessive-compulsive behaviors and insomnia, requiring child and adolescent psychiatrist collaboration for therapeutic optimization. To reduce the risk of relapse started monthly injections of cyclophosphamide. After 83 days, the patient was discharged, keeping multidisciplinary follow-up. At 6 months follow-up, patient’s behavior had improved, presenting without changes in neurologic exam or memory deficits.
Conclusion: The differential diagnosis of autoimmune and infectious encephalitis is notoriously difficult. Anti-NMDAR encephalitis is a potential lethal condition; early identification and treatment are crucial. In the absence of prospective and randomized data, treatment decisions should be individualized and take into consideration patient age, the presence or absence of a tumor, and symptom severity. Further studies in both pharmacologic approaches and non-pharmacologic interventions are critical in improving patient care and outcomes.

Palavras Chave: autoimmune disorder; NMDAR encephalitis;