Abstract:
:We report a male caucasian German pediatric patient of no Arab or Mediterranean ancestry with virus associated CNS lesions in Griscelli's syndrome (GS; McKusick No. 214450). The boy presented with recurrent infections, 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 perivascular CD8 positive lymphohistiocytic infiltrates. These findings were associated strictly with the presence of human herpesvirus-6 (HHV-6) genome or the HHV-6 specific late antigen H-AR 3, found in neurons, oligodendrocytes, 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 attempted using immunohistochemistry, in situ hybridization or nested polymerase chain reaction, respectively. Lymphocyte typing was carried out immunohistochemically. 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.
journal_name
J Med Viroljournal_title
Journal of medical virologyauthors
Wagner M,Müller-Berghaus J,Schroeder R,Sollberg S,Luka J,Leyssens N,Schneider B,Krueger GRdoi
10.1002/(sici)1096-9071(199711)53:3<306::aid-jmv21subject
Has Abstractpub_date
1997-11-01 00:00:00pages
306-12issue
3eissn
0146-6615issn
1096-9071pii
10.1002/(SICI)1096-9071(199711)53:3<306::AID-JMV21journal_volume
53pub_type
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