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Autopsy case of the cerebellar form of progressive multifocal leukoencephalopathy without immunodeficiency
Yoshifumi Arai1, Yoshihiro Tsutsui, Kazuo Nagashima
1Second Department of Pathology, Hamamatsu University School of Medicine, Japan.
Abstract:
A case of the cerebellar form of progressive multifocal leukoencephalopathy (PML) without remarkable immune depression or immune deficiency is reported here. The patient was a 74-year-old-woman who had complications of chronic renal failure and renal anemia for several years. Seven months before her death she had symptoms of general fatigue, gait disturbance and articulation disorder. During her hospitalization period her neurological disorder gradually progressed irreversibly with failure of consciousness and she died of respiratory failure. She did not have remarkable clinical signs of immunodeficiency nor did she receive immunosuppressive therapy. Clinically she had not been diagnosed with PML. At the post-mortem examination different degrees of demyelination were observed in the brain white matter: diffuse and severe in the cerebellum, moderate and coalescent in the brainstem, and light and patchy in the cerebrum. JC virus antigen-positive cells were frequently observed in the demyelinated lesions in the cerebrum and sometimes observed in the brainstem, but were rarely found in the cerebellum. These findings suggest that PML lesions may be present with different degrees of demyelination that are inversely correlated with the number of JC virus-infected cells. This fact should be considered when evaluating the brain biopsies of PML patients.
Insights
A rare cerebellar form of progressive multifocal leukoencephalopathy (PML) occurred in an elderly woman without significant immune deficiency. Post-mortem analysis revealed varying demyelination inversely correlated with JC virus presence.
Area of Science:
- Neurology
- Virology
- Pathology
Background:
- Progressive multifocal leukoencephalopathy (PML) is typically associated with severe immune deficiency.
- This case presents a rare cerebellar form of PML in a patient without overt immunodeficiency.
Observation:
- A 74-year-old woman with chronic renal failure and anemia developed progressive neurological symptoms including fatigue, gait disturbance, and articulation disorder.
- Clinical presentation did not suggest immunodeficiency or PML, and she did not receive immunosuppressive therapy.
- Post-mortem examination revealed diffuse demyelination in the cerebellum, moderate in the brainstem, and mild in the cerebrum.
Findings:
- JC virus antigen was frequently found in demyelinated lesions in the cerebrum and brainstem, but rarely in the cerebellum.
- The degree of demyelination in different brain regions was inversely correlated with the number of JC virus-infected cells.
- These observations suggest variability in demyelination severity relative to viral presence in PML.
Implications:
- PML can manifest with varying degrees of demyelination, not always directly correlating with JC virus detection in all affected areas.
- Consideration of inverse correlation between demyelination and JC virus load is crucial for interpreting brain biopsies in suspected PML cases.
- This case highlights the importance of considering atypical presentations of PML, even in the absence of profound immunosuppression.