HUMAN HERPESVIRUS-6 (HHV-6)-ASSOCIATED NECROTIZING ENCEPHALITIS IN GRISCELLIS-SYNDROME

We report a male caucasian German pediatric patient of no Arab or Medi terranean ancestry with virus associated CNS lesions in Griscelli's sy ndrome (GS; McKusick No. 214450). The boy presented with recurrent inf ections, and meningitis with subsequent progressive signs of increased intracranial pressure leading to death at 32 weeks of age. At autopsy , various sites of the CNS revealed necroses in gray and white matter. CNS histology revealed numerous and massive predominantly perivascula r CD8 positive lymphohistiocytic infiltrates. These findings were asso ciated strictly with the presence of human herpesvirus-8 (HHV-6) genom e or the HHV-6 specific late antigen H-AR 3, found in neurons, oligode ndrocytes, and astrocytes. The search for HHV-6 replication dependent antigen, HHV-7 DNA, CMV, adenovirus, Coxsackie B1, B2, and B4-antigens , and mycobacteria was not successful. Detection of viruses was attemp ted using immunohistochemistry, in situ hybridization or nested polyme rase chain reaction, respectively. Lymphocyte typing was carried out i mmunohistochemically. In GS, virus induced CNS damage does not seem to require necessarily active virus replication. It may also appear as a consequence of an immune reaction triggered by antigen expression. (C ) 1997 Wiley-Liss, Inc.