Auto-immune anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis: three case reports
Bashiri, F.A.; Al-Rasheed, A.A.; Hassan, S.M.; Hamad, M.H.A.; El Khashab, H.Y.; Kentab, A.Y.; AlBadr, F.B.; Salih, M.A.
Paediatrics and International Child Health 37(3): 222-226
2017
ISSN/ISBN: 2046-9047 PMID: 27329512 DOI: 10.1080/20469047.2016.1191852
Accession: 057270560
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.