1086 Anti-NMDA-receptor encephalitis: Case report

Sunday, 7 December 2014
Exhibition Hall-Poster Area (Sul America)

Estela Risso , Alergy, Hospital Do Servidor Público Estadual De São Paulo, são paulo, Brazil

Mariana Monteiro , Alergy, Hospital Do Servidor Público Estadual De São Paulo, Sao Paulo, Brazil

João Ferreira De Mello , Hospital Do Servidor Público Estadual De São Paulo, Sao Paulo, Brazil

Tatianna Saraiva , Allergy and Immunology, Hospital Do Servidor Público Estadual, Brazil

Adriana Teixeira Rodrigues , Division of Clinical Immunology and Allergy, Hospital Das Clinicas, University of São Paulo, School of Medicine, Sao Paulo, Brazil

Erica Sbrissa , Alergy, Hospital Do Servidor Público Estadual De São Paulo, Sao Paulo, Brazil

Sonia Sawaya , Pediatria, Hospital Do Servidor Público Estadual De São Paulo, Brazil

Background: Anti N-methyl-d-aspartate (NMDA) receptor encephalitis is a severe autoimmune neurological disorder. Although most described in young women, is increasingly seen in adult men and children of both sexes, with or without cancer. It is characterized by psychosis, emotional and behavioral disorders, seizures and language deterioration, abnormal movements and even autonomic instability. Cognitive impairment, psychomotor regression and amnesia are common. In children, we observed milder or incomplete forms.

Methods: We report a case of anti NMDA receptor encephalitis in a child with subacute encephalopathy and psychiatric manifestations without malignancy.

Results: Female, 15 years old, healthy, 15 days ago with confusion, behavior and mood disorders, memory loss and agitation worsened progressively and 2 episodes of seizure. On examination: sleepy, confused and disoriented, no segmental deficits or meningeal signs.
Normal serum laboratory tests. Analysis of cerebrospinal fluid (CSF) showed an increase of leukocytes (41/mm3) with a predominance of lymphocytes (98%); immunology negative for syphilis and cysticercosis in CSF. Search oligoclonal bands: normal IgG in serum and CSF. Normal magnetic resonance imaging of skull. Electroencephalogram disorganization and diffuse slowing of brain electrical activity.
after another seizure, the patient had a decreased level of consciousness, speech impairment, movement disorders and muscle clonus, without motor response to stimuli (Glasgow 6). Received corticosteroid pulse therapy with 1 g / day for 3 days, human immunoglobulin 40 mg / day intravenously for 3 days and 6 sessions of plasmapheresis with mild clinical improvement.
After the detection of anti NMDA receptor antibody, began treatment with rituximab with good evolution. Showed no hemodynamic instability and no malignancies were found. At discharge, kept rituximab every 3 weeks and anticonvulsants. After 20 months of diagnosis, showed no change in behavior, motor or cognitive.

Conclusions: Anti NMDA receptor encephalitis is a rare disease. Up until 2012, only 3 cases of the disease were reported in Brazil. Often confused with psychiatric disorders or infectious encephalitis is still underdiagnosed and should always be considered. A retrospective study of adults with unknown etiology encephalitis demonstrated anti NMDA receptor antibodies in 1% of patients admitted to an intensive care unit.