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Case report: rare progressive cavitary lesion in progressive multifocal leukoencephalopathy
1Department of Internal Medicine, University Hospitals Geauga Medical Center, 13207 Ravenna Rd, Chardon, OH, 44024, USA. mindy.hoang@uhhospitals.org.
Abstract:
Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating disease of the central nervous system caused by reactivation of John Cunningham (JC) virus in a subset of immunocompromised individuals. This case report describes a rare case of progressive cavitary lesion in PML with only one other documented case in the world. The patient is a 63-year old male with medical history of microscopic polyangiitis who had previously undergone cyclophosphamide treatment and was on chronic immunosuppression with mycophenolate and high dose prednisone who presented to the emergency department with stroke-like symptoms. A series of magnetic resonance imaging (MRI) demonstrated a non-enhancing multifocal lesion in his frontal lobe that progressed into a cavitary lesion. PML was diagnosed using imaging presence of periventricular white matter lesions and positive JC virus in the cerebrospinal fluid. Stereotactic biopsy followed by histological staining of the samples and immunohistochemistry confirmed the diagnosis. We emphasize cavitary lesions as an extremely rare, but potential development of PML that appears to correlate with aggressive neurocognitive decline and has no identified associations.
Insights
This case report details a rare progressive cavitary lesion in progressive multifocal leukoencephalopathy (PML), a central nervous system disease caused by John Cunningham (JC) virus reactivation. This finding correlates with rapid cognitive decline.
Area of Science:
- Neurology
- Infectious Diseases
- Radiology
Background:
- Progressive multifocal leukoencephalopathy (PML) is a rare, opportunistic demyelinating disease of the central nervous system.
- PML is caused by the reactivation of John Cunningham (JC) virus in immunocompromised individuals.
- Immunosuppression is often associated with conditions like microscopic polyangiitis, treated with agents such as cyclophosphamide, mycophenolate, and prednisone.
Purpose of the Study:
- To report an extremely rare case of progressive cavitary lesions in PML.
- To highlight the association between cavitary lesions and aggressive neurocognitive decline in PML.
- To contribute to the limited literature on this specific PML manifestation.
Main Methods:
- Case report of a 63-year-old male with microscopic polyangiitis on immunosuppressive therapy.
- Magnetic resonance imaging (MRI) to track lesion progression.
- Cerebrospinal fluid (CSF) analysis for JC virus detection.
- Stereotactic brain biopsy with histological staining and immunohistochemistry for diagnosis confirmation.
Main Results:
- The patient presented with stroke-like symptoms.
- MRI revealed a multifocal frontal lobe lesion that evolved into a cavitary lesion.
- PML diagnosis was confirmed by imaging, positive JC virus in CSF, and biopsy.
- Cavitary lesions in PML are exceptionally rare, with only one prior case documented.
Conclusions:
- Cavitary lesions represent an extremely rare but potential complication of PML.
- This manifestation appears linked to rapid neurocognitive deterioration.
- Further research is needed to understand the mechanisms and implications of cavitary PML.
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