Auto-immune anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis: three case reports. (3rd July 2017)

• Record Type: Journal Article
• Authors: Bashiri, Fahad A.; Al-Rasheed, Abdulrahman A.; Hassan, Saeed M.; Hamad, Muddathir H. A.; El Khashab, Heba Y.; Kentab, Amal Y.; AlBadr, Fahad B.; Salih, Mustafa A.
• Is Part Of: Paediatrics and international child health. Volume 37:Number 3(2017)
• Page Start: 222
• Page End: 226
• Publication Date: 2017-07-03
• Subjects: Anti-NMDAR encephalitis -- Children -- Auto-antibodies -- Movement disorder -- Immunomodulation therapy; Pediatrics -- Developing countries -- Periodicals; Children -- Health and hygiene -- Developing countries -- Periodicals; Children -- Diseases -- Developing countries -- Periodicals; 618.920009172405
• DOI: 10.1080/20469047.2016.1191852
• Languages: English
• ISSNs: 2046-9047

Abstract

Abstract : Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a recently identified auto-immune disorder characterised by severe memory deficit, a decreased level of consciousness, seizures, autonomic dysfunction and movement disorders. Three girls with the disorder are reported; they were aged 4 years, 5 years and 10 months. The 10-month-old infant who is one of the youngest patients reported with anti-NMDAR encephalitis worldwide, had MRI features suggestive of herpes simplex encephalitis (known to trigger anti-NMDAR encephalitis), but CSF PCR for herpes simplex was negative. All the patients presented with seizures, behavioural change, regression of speech, dystonia and choreo-athetosis. Anti-NMDAR antibodies were detected in all patients' sera and cerebrospinal fluid (CSF). Intravenous immunoglobulin, corticosteroids and rituximab were administered at different intervals. Cases 1 and 2 made a full recovery, but case 3 has mild motor and speech delay. Patients who present with encephalopathy, seizures and movement disorders should be tested for anti-NMDAR antibodies in serum and CSF in addition to being screened for herpes simplex encephalitis.