Auto-immune anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis: three case reports

• Published in: Paediatrics and international child health Vol. 37; no. 3; pp. 222 - 226
• Main 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.
• Format: Journal Article
• Language: English
• Published: England Taylor & Francis 03.07.2017
• Subjects: Adrenal Cortex Hormones - therapeutic use; Anti-N-Methyl-D-Aspartate Receptor Encephalitis - diagnosis; Anti-N-Methyl-D-Aspartate Receptor Encephalitis - drug therapy; Anti-N-Methyl-D-Aspartate Receptor Encephalitis - pathology; Anti-NMDAR encephalitis; Auto-antibodies; Autoantibodies - blood; Brain - diagnostic imaging; Child; Child, Preschool; Children; Diagnosis, Differential; Female; Humans; Immunoglobulins, Intravenous - therapeutic use; Immunologic Factors - therapeutic use; Immunomodulation therapy; Infant; Magnetic Resonance Imaging; Movement disorder; Receptors, N-Methyl-D-Aspartate - immunology; Rituximab - therapeutic use; Treatment Outcome; Immunomodulation therapy; Anti-NMDAR encephalitis; Children; Auto-antibodies; Movement disorder
• ISSN: 2046-9047; 2046-9055
• DOI: 10.1080/20469047.2016.1191852

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.