First reported cases of anti-NMDA receptor encephalitis in Vietnamese adolescents and adults
Nguyen Thi Hoang M., Nguyen Hoan P., Le Van T., McBride A., Ho Dang Trung N., Tran Tan T., Nguyen Thi Thu H., Heemskerk D., Day J., Vincent A., Nguyen Van Vinh C., Thwaites G.
© 2017Introduction Anti-NMDA receptor encephalitis is increasingly recognised as an important differential diagnosis in patients with encephalitis of unknown aetiology. We report the first case series of patients diagnosed in Vietnam. Methods Samples of CSF from patients with presumed encephalitis but negative microbiological investigations, who exhibited dyskinesia, autonomic instability or psychosis were tested for antibodies against the NR1 subunit of the glutamate (type-NMDA) receptor using an indirect immunofluorescence assay. Results Of 99 patients admitted with all-cause encephalitis over an 18 month period, 9.1% (n = 9 patients, 5 female, median age 28 years) had confirmed NMDAR encephalitis. All patients were admitted from one mental health hospital, and the incidence may therefore be an underestimate. Common features included reduction in speech (n = 9), catatonia (n = 9), convulsions (n = 7), dyskinesia (n = 9), rigidity (n = 9) and autonomic dysfunction (n = 7). Aside from a modest lymphocytic pleocytosis, routine CSF analysis was usually normal. No female patient had ovarian teratoma detected by abdominal ultrasound. Most patients were treated with high dose corticosteroids, and one patient received intravenous immunoglobulin. The median duration of hospitalization was 75 days and no patient died during admission. Conclusions Anti-NMDA receptor encephalitis is an important differential diagnosis to consider for patients presenting with acute onset psychiatric symptoms, who develop ensuing seizures, movement or autonomic disorder in Vietnam. A stronger evidence base for management and access to second line immunotherapy agents may help to reduce morbidity from this disease.