First reported cases of anti-NMDA receptor encephalitis in Vietnamese adolescents and adults

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 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.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.

Brain imaging in catatonia: systematic review and directions for future research

“…Encephalomalacia was identified in 7 cases (more frequently in patients with a history of head trauma or stroke), and multifocal lesions were seen in 3 patients (multiple sclerosis, encephalomyelitis, cerebral metastasis). In 2017, Nguien Thi Hoang published a study with 9 patients with catatonia associated with anti-NMDA receptor encephalitis (Nguyen Thi Hoang et al, 2017). Among them, 3 had a normal brain CT, and 6 a brain MRI, of which one was abnormal showing bilateral hyperintensity in the image contrast within the medial temporal lobes.…”

Disentangling etiologies of CNS infections in Singapore using multiple correspondence analysis and random forest

“…A prospective study from the UK identified 20% of cases with an immune-mediated cause, including ADEM, NMDAR encephalitis and VGKC complex encephalitis 31 ; another from Thailand, found 24% of patients with encephalitis were associated with immune encephalitis 23 . A recent retrospective cohort study in the United States also identified 20% with an autoimmune cause 32 ; another from Vietnam found 9.1% had NMDAR encephalitis 33 . A systematic approach to autoantibody testing in the assessment of acute encephalitis patients may be required as early and accurate diagnosis of immune-mediated etiology is important for appropriate immunotherapy rather than empirical administration of antimicrobials.…”

Finding the Cause of Psychosis: A Challenging Case of Anti-NMDAR Encephalitis

“…Our patient responded well to immunotherapy with methylprednisolone and IVIG. Although symptoms are usually severe, studies suggest that patients are usually highly responsive to therapy [ 9 ]. As per a reported case series of 99 patients with all-cause encephalitis, 9.1% were found to have anti-NMDAR encephalitis.…”

“…The mean age of these patients was 28 years, and 5 of 9 were females. Follow-up of these patients showed zero mortality and complete symptomatic recovery after treatment with high-dose corticosteroids and intravenous immunoglobulin [ 9 ]. Prompt diagnosis and early treatment will reduce mortality and ensure complete recovery.…”