1 - Unidade de Infeciologia Pediátrica
2 - Serviço de Neurologia Pediátrica
3 - Serviço de Pedopsiquiatria
4 - Unidade de Cuidados Intensivos: Hospital Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central.
- Poster, ESPID 2022
BACKGROUND: Anti-NMDAr encephalitis is an immune-mediated disease. A typical presentation includes acute neuropsychiatric features, abnormal behavior, movement disorder, and autonomic instability. Autoantibodies against NMDA receptors in the brain can be created following triggering mechanisms such as infectious agents and tumors.
CASE PRESENTATION SUMMARY: A 17-year-old teenager with neuropsychiatric disorder, seizures, fluctuating consciousness and dysautonomia, required intensive care and mechanical ventilation. It was detected anti-NMDAr in the blood and cerebrospinal fluid. An abdominal ultrasound identified a left ovarian teratoma. A surgical excision was performed, and the first line treatment of Brain Works protocol was started with intravenous immunoglobulin, corticotherapy and plasmapheresis. Despite this, there was no clinical improvement. On the 13th day of hospitalization, the condition was complicated by Acinetobacter baumannii pneumonia, which motivated a delay of second line therapy with rituximab, that was started only on 38th day. The clinical response was slow and after the fourth dose there was a partial clinical improvement and cyclophosphamide was started. After the first cycle, it was observed leukopenia (290/ul) with severe neutropenia (69/uL) complicated by nosocomial pneumonia, and after the second cycle, alopecia, dysgeusia, constipation, macrocytosis and sepsis without an identified agent. Therefore, cyclophosphamide was suspended, maintaining monthly immunoglobulin and daily prednisolone. After 153 days of treatment, the patient revealed partial clinical improvement with multiple neurological sequelae.
LEARNING POINTS/ DISCUSSION: The neurological benefit of immunomodulatory therapy must be balanced with the risk of opportunistic infections and noninfectious complications. There are limited data for the management of this therapy, wherefore its maintenance must be integrated into a multidisciplinary discussion, involving the patient and his family.