title: Sudden unexpected death associated with HHV-6 in an adolescent with tuberous sclerosis.
abstract: A 14-year-old female with tuberous sclerosis and history of seizures was found dead in bed at home 3 days after she had been assessed as doing well at a routine neurology clinic appointment. She had been treated with an antiepileptic drug, felbamate, for 36 months and had been seizure-free except for one seizure episode 5 months before death. Postmortem examination revealed cerebral edema, with uncal and tonsillar herniation, and pulmonary edema, consistent with seizure-induced apnea. Multiple microglial nodules with mature perivascular lymphocytic cuffing and diffuse infiltrates were identified around subependymal tuberous sclerosis giant cell nodules. Immunostaining and electron microscopy revealed human herpesvirus-6-infected macrophages, astrocytes, lymphocytes, and endothelial cells in the subependymal tuberous sclerosis lesions and choroid plexus. Subacute human herpesvirus-6 encephalitis is postulated to have precipitated a seizure and thus sudden unexpected death in epilepsy in this otherwise stable adolescent patient.
authors.affiliation: Department of Pathology, Harbor-UCLA Medical Center, University of California Los Angeles, School of Medicine, Torrance, 90509, USA.
authors.forename: J
authors.initials: J
authors.lastname: Wang
authors.forename: K
authors.initials: K
authors.lastname: Huff
authors.forename: R
authors.initials: R
authors.lastname: McMasters
authors.forename: M E
authors.initials: ME
authors.lastname: Cornford
mesh_terms: D000293:Adolescent; D003645:Death, Sudden / Q000209:etiology*; D018792:Encephalitis, Viral / Q000150:complications* / Q000821:virology; D005260:Female; D006566:Herpesviridae Infections / Q000150:complications* / Q000821:virology; D015654:Herpesvirus 6, Human / Q000302:isolation & purification*; D006801:Humans; D014402:Tuberous Sclerosis / Q000150:complications* / Q000188:drug therapy