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Paediatr Int Child Health · Aug 2017
Case ReportsAuto-immune anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis: three case reports.
- Fahad A Bashiri, Abdulrahman A Al-Rasheed, Saeed M Hassan, Muddathir H A Hamad, Heba Y El Khashab, Amal Y Kentab, Fahad B AlBadr, and Mustafa A Salih.
- a Division of Pediatric Neurology.
- Paediatr Int Child Health. 2017 Aug 1; 37 (3): 222-226.
AbstractAnti-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.
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