Abstract Ref Number = APCP1111
OPERCULAR SYNDROME AS A RARE MANIFESTATION IN PEDIATRIC HERPES SIMPLEX ENCEPHALITIS
Stephanie Amanda Hatibie,Agatha F Christie,Irawan Mangunatmadja
RSCM FKUI RSCMFKUI
Background : Herpes simplex virus (HSV) is the most common cause of encephalitis in children with incidence of 1: 250.000-500.000 per year. Focal seizure and neurologic dysfunction with progressive deterioration are the main characteristics. Opercular syndrome is a severe and rare (< 1:1.000.000 cases) clinical manifestation of HSV encephalitis (HSE), characterized by the loss of the voluntary control of facial-lingual-glossopharyngeal and masticatory muscles due to bilateral lesions of the anterior opercular region. Even with appropriate treatment, HSE mortality rates can reach up to 30%. About 20-40% patients recovered with sequelae. Here we present our case to raise awareness about the rare clinical manifestation of HSE.
Case Presentation Summary : A ten-year-old boy was referred with confusion, dysphagia, speech and mouth-closing difficulties. He was previously hospitalized for 3 weeks in rural hospital with four days of fever, headache, hemiparese, and progressive behavioral change. Serological marker showed reactive IgM and IgG HSV antibodies. Electroencephalogram (EEG) showed asymmetric wave with periodic lateralizing epileptiform discharged (PLED) and frontotemporal focal decelaration. Cranial MRI images showed multiple lesion with rim enhancement in cortical, subcortical and white-matter of right temporal, parieto-occipital bilateral, and operculum area. Thus,he was diagnosed as severe HSE and 60 mg/kg/day intravenous (IV) acyclovir treatment was initiated. He was able to cry, blink, and yawn. On the basis of source limitation, IV acyclovir was switched to oral after 14 days given. On the 19th day of therapy, patients were discharged due to parents concerned. Oral acyclovir were continued completing total 21 days therapy. Two weeks following discharged, patient could obey command and speak few words. Feeding tube was no longer needed, his swallowing functions were regenerated, and motoric function was improved.
Learning Points/Discussion : Opercular Syndrome is severe clinical manifestation of pediatric HSE. By being aware of this atypical presentation of symptoms, earlier adequate treatment can be initiated and could prevent mortality and neurological complications.
Keywords: operculum herpes simplex encephalitis severe