ICNC Abstracts, ICNC 2018

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Anti-NMDAR Encephalitis in Children ; a Malaysian Experience
Balakrishnan Priyalatha, Tajudin Tajul Arifin, Vigneswari G

Last modified: 2018-09-09

Abstract


Introduction:Anti-NMDAR encephalitis is a newly recognized encephalitis presenting with movement disorders, seizures, cognitive decline, psychiatric and autonomic symptoms.

Aim:To describe the clinical characteristics, management and outcome of paediatric patients with anti-NMDAR encephalitis.

Methodology:A retrospective review of case notes of 20 patients managed at two paediatric neurology centres from 2014 to 2018 were conducted.

Results:Twenty patients (F:M=10:10) were identified with a median age of 9 years at presentation (range 1.1-13.9 yrs old). Majority of them (n=15, 75%) presented with prodrome of fever, headache, respiratory or gut symptoms. At presentation, fourteen (70%) patients had seizures and eighteen (90%) had movement disorders which comprised of chorea (n=12,67%), stereotypy (n=8,44%), dystonia (n=8,44%), tics (n=1,6%), tremor (n=1,6%), myoclonus (n=1,6%) and ataxia (n=1,6%). All (n=20,100%) had psychiatric or behavioral symptoms. Seventeen (85%) demonstrated autonomic disturbances. Interestingly, three patients presented atypically with optic neuritis, epileptic encephalopathy and brainstem syndrome respectively. Electroencephalogram was abnormal in all. The main observations were background slowing (n=17,85%) and focal epileptiform discharges (n=13,65%). Focal rhythmic delta activities were noted in some. MRI brain was done for all and fourteen (70%) were abnormal with predominant gray matter involvement. All received corticosteroids and intravenous immunoglobulin as first line treatment. Non-responders (n=10,50%) were treated with intravenous cyclophosphamide or rituximab. Twelve patients (60%) achieved good recovery, while seven patients had residual behavioral impairment and one suffered severe disability; while four relapsed.

Conclusion:Anti-NMDAR encephalitis is rare but potentially treatable condition and timely recognition with prompt treatment may yield a favourable outcome.

 

 


Keywords


Anti-NMDAR Encephalitis

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