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SSPE-like inclusion body disorder in treated childhood leukemia
Abstract:
Clinico-pathological report on a boy with cytostatically treated leukemia, dying with cerebral symptoms after passing clinical measles 10 weeks before death. At autopsy, numerous nuclear inclusion bodies in glial and nerve cells were found. By electron microscopy, nuclear inclusions appeared as loosely arranged smooth tubules, corresponding to paramyxovirus nucleocapsids. Frequently, cytoplasmic changes appeared too, consisting of incomplete tubular structures and an abundant dense "fuzzy" material. No regular tubuli of the coated granular type were present, as in common measles virus infection, nor any mature viral structures or differentiation of the surface membrane. The lack of maturation in cytoplasm together with a predominance of nuclear changes suggested a slow type of measles virus infection, while the particular cytoplasmic changes suggested a defect in synthesis of granular nucleocapsids, possibly a basic factor for the slow type of the viral infection. Possible pathogenetic factors are discussed.
Insights
This report details a rare, slow measles virus infection in a boy with leukemia, characterized by unusual nuclear and cytoplasmic changes. The findings suggest a defect in viral nucleocapsid synthesis contributing to the infection
Area of Science:
- Virology
- Neuropathology
- Pediatric Oncology
Background:
- Measles virus (paramyxovirus) typically causes acute infection with characteristic cytopathic effects.
- Leukemia patients undergoing cytostatic treatment may have altered immune responses, potentially influencing viral infections.
Purpose of the Study:
- To investigate the clinico-pathological findings of a fatal measles virus infection in a child with leukemia.
- To characterize the ultrastructural features of measles virus replication in the central nervous system.
Main Methods:
- Clinico-pathological examination of autopsy tissues.
- Electron microscopy to analyze viral morphology and cellular changes in glial and nerve cells.
Main Results:
- Autopsy revealed numerous nuclear inclusion bodies in glial and nerve cells.
- Electron microscopy showed nuclear inclusions consistent with paramyxovirus nucleocapsids.
- Cytoplasmic changes included incomplete tubular structures and dense "fuzzy" material, differing from typical measles virus infections.
Conclusions:
- The observed pathology suggests a slow-onset measles virus infection.
- Defects in the synthesis of granular nucleocapsids may underlie the atypical viral replication and slow infection type.
- Altered host immune status in leukemia patients could be a contributing factor.