Infection with JC virus and possible dysplastic ganglion-like transformation of the cerebral cortical neurons in a case of progressive multifocal leukoencephalopathy.
Infection of the cerebral cortical neurons with JC virus (JCV) with possible dysplastic ganglion-like alteration of the infected neurons found in a case of progressive multifocal leukoencephalopathy (PML) is described. The patient was a 21-year-old man with common variable immunodeficiency who died of PML after a 9-month clinical course. At autopsy, the white matter of the cerebrum, brainstem, cerebellum, and spinal cord exhibited extensive demyelination and necrosis. Numerous inclusion-bearing oligodendrocytes and bizarre astrocytes were found. In the occipital and temporal cortex, thick band-like aggregates of dysplastic ganglion-like cells (DGLCs) were found. These DGLCs showed immunohistochemical properties of neurons, and nuclei of some DGLCs were immunoreactive for large T antigen of SV40/JCV and p53, but not for capsid protein JCV VP1. In situ hybridization for mRNA of JCV large T antigen revealed positive signals in the nuclei of some DGLCs. These results indicate that JCV infected neurons and it is suggested that binding of the large T antigen with cellular proteins could have resulted in the dysplastic, ganglion cell-like change of the infected neurons, although the possibility that the aggregates of DGLCs represent a pre-existent malformative lesion of the cortex cannot be excluded completely.
Shintaku
M
M
Department of Pathology, Osaka Red Cross Hospital, Tennoji, Japan.
Matsumoto
R
R
Sawa
H
H
Nagashima
K
K
D000328:Adult; D000952:Antigens, Polyomavirus Transforming / Q000032:analysis / Q000235:genetics; D000956:Antigens, Viral / Q000032:analysis; D002213:Capsid / Q000032:analysis / Q000235:genetics; D002467:Cell Nucleus / Q000473:pathology / Q000821:virology; D002540:Cerebral Cortex / Q000473:pathology* / Q000821:virology; D017809:Fatal Outcome; D006801:Humans; D017403:In Situ Hybridization; D007181:Inclusion Bodies, Viral / Q000737:chemistry / Q000473:pathology; D007577:JC Virus; D007968:Leukoencephalopathy, Progressive Multifocal / Q000473:pathology*; D008297:Male; D009336:Necrosis; D009474:Neurons / Q000473:pathology / Q000821:virology; D009836:Oligodendroglia / Q000473:pathology / Q000821:virology; D012333:RNA, Messenger / Q000032:analysis; D012367:RNA, Viral / Q000032:analysis
D000952:Antigens, Polyomavirus Transforming; D000956:Antigens, Viral; D012333:RNA, Messenger; D012367:RNA, Viral