Related Experiment Video
Updated: Feb 18, 2026
Visualizing Impairment of the Endothelial and Glial Barriers of the Neurovascular Unit during Experimental Autoimmune Encephalomyelitis In Vivo
Published on: March 26, 2019
Progressive multifocal leukoencephalopathy in the absence of immunosuppression
Benjamin E Zucker1,2, Sybil R L Stacpoole3,4
1Imperial College School of Medicine, South Kensington Campus, London, SW7 2AZ, UK.
Abstract:
A 69-year-old woman presented with a cortical hand syndrome progressing over several weeks. MRI brain showed characteristic appearances of progressive multifocal leukoencephalopathy (PML), confirmed by detection of the JC virus in CSF, despite the absence of any evidence of immunosuppression. Treatment with mirtazapine, mefloquine and cidofovir did not affect the progression of the disease, which was fatal within 7 months of presentation. This report adds to the small case literature that suggests that PML can occur in immunocompetent people, albeit extremely rarely.
Insights
Progressive multifocal leukoencephalopathy (PML) is extremely rare in immunocompetent individuals. This case highlights a fatal PML progression in a 69-year-old woman without signs of immunosuppression.
Area of Science:
- Neurology
- Virology
- Neuroimmunology
Background:
- Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating disease of the central nervous system.
- PML is typically associated with severe cellular immunodeficiency, particularly in individuals with advanced HIV infection or those on immunosuppressive therapies.
Observation:
- A 69-year-old immunocompetent woman presented with a weeks-long progression of cortical hand syndrome.
- Cerebral MRI revealed findings characteristic of PML.
- JC virus was detected in cerebrospinal fluid (CSF), confirming the diagnosis.
Findings:
- Despite treatment with mirtazapine, mefloquine, and cidofovir, the patient's condition rapidly deteriorated.
- The disease proved fatal within seven months of initial presentation.
- This case underscores the possibility of PML occurring in the absence of detectable immunosuppression.
Implications:
- This case contributes to the limited literature suggesting PML can occur in immunocompetent individuals, though exceedingly rare.
- It prompts consideration of PML in the differential diagnosis of neurological syndromes even in the absence of known risk factors for immunosuppression.
- Further research is needed to understand the mechanisms and potential management strategies for PML in immunocompetent hosts.
