Cortical liquefaction in severe human herpesvirus 6 encephalopathy

Abstract

Human herpesvirus 6 (HHV6) is the causative agent of the common childhood infectious disease exanthema subitum. Primary infection of CNS by HHV6 can cause seizures and encephalitis/encephalopathy.1 We report a 9-month-old girl with severe HHV6 encephalopathy, in whom a subacute MRI showed an unusual appearance of the cerebral cortex suggesting cortical liquefaction. A previously healthy 9-month-old Japanese girl was admitted because of status epilepticus for 1 hour following a 1-day prodromal illness consisting of fever (around 38.5 °C for 3 days) and vomiting. After her seizure was controlled, she was in a comatose state and required intubation. Laboratory examination revealed elevated serum liver enzyme activities. CSF showed normal cell count (1/mm3), protein level (18 mg/dL), and glucose level (122 mg/dL). EEG showed polyspikes in bilateral centroparietal regions. HHV6 was later isolated from her throat swab and serum, and a diagnosis of HHV6 encephalopathy was established. Clinical symptoms or laboratory data suggesting hemorrhagic shock and encephalopathy syndrome (shock, diarrhea, disseminated intravascular …