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Progressive multifocal leukoencephalopathy and gliomas in a HIV-negative patient
F Gullotta1, T Masini, G Scarlato
1Institut für Neuropathologie, Universität Münster, FRG.
Abstract:
A case of progressive multifocal leukoencephalopathy (PML) is reported, detected at autopsy of a 30-year-old patient. The clinical picture was characterized by a progressive course of mental deterioration and ingravescent neurological symptoms. The patient was HIV-negative. He died of bronchopneumonia, after a clinical course of 13 months. Autopsy disclosed pulmonary tuberculosis with involvement of regional lymph nodes. In the brain, besides numerous PML-foci of varying age and structure, a pleomorphic astrocytoma was found in the white matter of the right parietal lobe. In the brain stem glial proliferation resembling diffuse gliomatosis was also present. In situ hybridization revealed Papova-virus (JCV) in oligoglial nuclei, but not in neoplastic astrocytes. This is the third report on the concomitant occurrence of PML and glioma in man.
Insights
This case report details a rare instance of progressive multifocal leukoencephalopathy (PML) co-occurring with glioma in an HIV-negative patient. Autopsy revealed both conditions, highlighting an unusual association in the brain.
Area of Science:
- Neuropathology
- Neurovirology
- Oncology
Background:
- Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating disease of the central nervous system.
- PML is typically associated with severe immunosuppression, most commonly in individuals with Human Immunodeficiency Virus (HIV).
- The co-occurrence of PML and primary brain tumors like glioma is exceptionally rare.
Observation:
- A case of a 30-year-old, HIV-negative patient with a 13-month history of progressive neurological decline and mental deterioration is presented.
- Autopsy revealed pulmonary tuberculosis and, in the brain, multiple PML lesions of varying ages and a pleomorphic astrocytoma in the right parietal lobe.
- Glial proliferation resembling diffuse gliomatosis was also noted in the brainstem.
Findings:
- In situ hybridization confirmed the presence of Papova-virus (JCV) within the nuclei of oligodendrocytes in PML lesions.
- JCV was notably absent in the neoplastic astrocytes of the pleomorphic astrocytoma.
- This represents the third reported case of concomitant PML and glioma in a human patient.
Implications:
- The findings suggest that PML and glioma can occur together, even in the absence of HIV-related immunosuppression.
- Further research is needed to understand the potential pathogenetic mechanisms linking JCV infection and gliomagenesis.
- This case underscores the importance of considering diverse etiologies in patients presenting with neurological deterioration and coexisting brain lesions.