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"Burnt-out" progressive multifocal leukoencephalopathy in idiopathic CD4+ lymphocytopenia
Tatsuya Fukumoto1,2, Yasuhiro Sakashita3, Fumiaki Katada2
1Department of Neurology, Tokushima University Graduate School of Biomedical Sciences, Tokushima, Japan.
Abstract:
Progressive multifocal leukoencephalopathy (PML) is a fatal disease caused by John Cunningham virus (JCV) infection; however, a growing number of PML patients now survive longer and achieve remission, largely due to the advent of combination antiretroviral therapy. Several reports have suggested that the pathology in such patients presents only chronic demyelination without characteristic cellular changes, being referred to as "burnt-out" PML. On the other hand, our knowledge of "burnt-out" PML is still substantially limited, especially in patients with non-human immunodeficiency virus infection. Here, we report a case of PML associated with idiopathic CD4+ lymphocytopenia (ICL) who presented with spontaneous remission and survived for 11 years after onset. Notably, postmortem examination revealed surprisingly broad "burnt-out" lesions lacking the classic histopathological findings. However, pathogenic JCV-specific DNA sequences was still present in the autopsied brain tissue. This case suggests that complete remission can be achieved with a persistent presence of JCV-specific pathogenic sequences, even after a catastrophic infection. Considering that there have been a few reported cases of PML with ICL with long survival, the long-term survival of our case may share a favorable immunological response that is unique to a subgroup of ICL.
Insights
Progressive multifocal leukoencephalopathy (PML) can now be survived longer, with some patients achieving remission. This case study highlights long-term survival in PML with idiopathic CD4+ lymphocytopenia, showing remission is possible even with persistent JCV DNA.
Area of Science:
- Neurovirology
- Immunology
Background:
- Progressive multifocal leukoencephalopathy (PML) is a fatal demyelinating disease caused by John Cunningham virus (JCV).
- Advances in combination antiretroviral therapy have improved survival rates and remission in PML patients, particularly those with human immunodeficiency virus.
- Limited understanding exists regarding "burnt-out" PML, especially in non-HIV infected individuals.
Observation:
- A case of PML associated with idiopathic CD4+ lymphocytopenia (ICL) is presented, demonstrating spontaneous remission and an 11-year survival post-onset.
- Postmortem examination revealed extensive "burnt-out" lesions without typical histopathological signs of active PML.
- Pathogenic JCV-specific DNA sequences were detected in the brain tissue despite the absence of classic cellular changes.
Findings:
- Complete remission in PML can occur despite the persistent presence of JCV-specific pathogenic sequences.
- The case suggests that long-term survival in PML with ICL may be linked to a unique and favorable immunological response within a specific ICL subgroup.
Implications:
- This case expands the understanding of PML outcomes, particularly in non-HIV settings.
- It suggests that "burnt-out" PML lesions can harbor JCV DNA and still be associated with long-term remission.
- Further research into the immunological mechanisms underlying favorable responses in ICL patients with PML is warranted.
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