Abstract
IntroductionAnti-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. MethodsSamples 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. ResultsOf 99 patients admitted with all-cause encephalitis over an 18month period, 9.1% (n=9 patients, 5 female, median age 28years) 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 75days and no patient died during admission. ConclusionsAnti-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.
Highlights
Anti-NMDA receptor encephalitis is increasingly recognised as an important differential diagnosis in patients with encephalitis of unknown aetiology
Since its first discovery in 2007 as a phenomenon associated with underlying ovarian teratoma, the epidemiology of anti-NMDA receptor encephalitis has shifted substantially; it has more often been reported in female patients without tumor, males and children in recent years [8]
At an infectious diseases referral hospital in southern Vietnam, we investigated whether anti-NMDA receptor encephalitis was prevalent in patients with suggestive clinical features, in whom conventional microbiological testing had not identified an infectious cause
Summary
Anti-NMDA receptor encephalitis is increasingly recognised as an important differential diagnosis in patients with encephalitis of unknown aetiology. Likewise in a recent cohort of 291 adults with presumed viral encephalitis admitted to our hospital, only 32% of patients had a microbiologically confirmed infection [2]. Over the past 10 years, autoimmune encephalitides, especially antiN-methyl D aspartate (NMDA) receptor encephalitis, have been increasingly recognised as important differential diagnoses for viral encephalitis, in young adults and children [4,5,6]. Since its first discovery in 2007 as a phenomenon associated with underlying ovarian teratoma, the epidemiology of anti-NMDA receptor encephalitis has shifted substantially; it has more often been reported in female patients without tumor, males and children in recent years [8]. There are few reports from resource-limited settings [9]
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