[P1-110] Case Series of Anti-N-Methyl-D-aspartate receptor (NMDAR) Encephalitis in Children In Hong Kong
[Introduction]
Anti-NMDAR encephalitis is a severe, but treatable disorder. We study the clinical features, investigation findings and treatment outcome of children and adolescents (<18 years) with anti-NMDAR encephalitis in Hong Kong.
[Results]
15 cases were identified from the paediatric units of 6 Hospital Authority clusters from 2009 to 2015. Female to male ratio was 2 to 1. The mean and median age of presentation was 9 years and 12 years (1-17 years) respectively. All patients developed a constellation of symptoms with time. The most common symptom was neuropsychiatric manifestations (15/15, 100%), followed by seizures (14/15, 93%), speech dysfunction (13/15, 87%), movement disorders (12/15, 80%), sleep disturbance (9/15, 60%) and autonomic dysfunction (5/15, 33%). All patients had serum anti-NMDAR antibody checked and 10 (67%) were positive. 13 patients had CSF anti-NMDAR antibody testing done and all were positive (100%). Imaging performed in all patients did not demonstrate any pelvic tumor. 13 patients were treated with both IVIG and steroid, while 1 was treated with IVIG only and 1 was treated with steroid only. 3 patients also required plasmaphresis and Rituximab. 10 patients recovered fully after treatment. 3 patients had residual behavioral problem. 1 patient who was also suffered from HSV encephalitis was complicated with epilepsy and cerebral palsy. 1 patient defaulted follow up and long term outcome was therefore unknown.
[Conclusion]
The presentation of anti-NMDAR encephalitis is variable, but usually consists of a constellation of symptoms. With immunotherapy, 2/3 patients recovered fully, while 1/3 patients had different degrees of neurological deficits.
Anti-NMDAR encephalitis is a severe, but treatable disorder. We study the clinical features, investigation findings and treatment outcome of children and adolescents (<18 years) with anti-NMDAR encephalitis in Hong Kong.
[Results]
15 cases were identified from the paediatric units of 6 Hospital Authority clusters from 2009 to 2015. Female to male ratio was 2 to 1. The mean and median age of presentation was 9 years and 12 years (1-17 years) respectively. All patients developed a constellation of symptoms with time. The most common symptom was neuropsychiatric manifestations (15/15, 100%), followed by seizures (14/15, 93%), speech dysfunction (13/15, 87%), movement disorders (12/15, 80%), sleep disturbance (9/15, 60%) and autonomic dysfunction (5/15, 33%). All patients had serum anti-NMDAR antibody checked and 10 (67%) were positive. 13 patients had CSF anti-NMDAR antibody testing done and all were positive (100%). Imaging performed in all patients did not demonstrate any pelvic tumor. 13 patients were treated with both IVIG and steroid, while 1 was treated with IVIG only and 1 was treated with steroid only. 3 patients also required plasmaphresis and Rituximab. 10 patients recovered fully after treatment. 3 patients had residual behavioral problem. 1 patient who was also suffered from HSV encephalitis was complicated with epilepsy and cerebral palsy. 1 patient defaulted follow up and long term outcome was therefore unknown.
[Conclusion]
The presentation of anti-NMDAR encephalitis is variable, but usually consists of a constellation of symptoms. With immunotherapy, 2/3 patients recovered fully, while 1/3 patients had different degrees of neurological deficits.