| Literature DB >> 27329512 |
Fahad A Bashiri1, Abdulrahman A Al-Rasheed2, Saeed M Hassan1, Muddathir H A Hamad1, Heba Y El Khashab1, Amal Y Kentab1, Fahad B AlBadr3, Mustafa A Salih1.
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.Entities:
Keywords: Anti-NMDAR encephalitis; Auto-antibodies; Children; Immunomodulation therapy; Movement disorder
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Year: 2016 PMID: 27329512 DOI: 10.1080/20469047.2016.1191852
Source DB: PubMed Journal: Paediatr Int Child Health ISSN: 2046-9047 Impact factor: 1.990