“Burnt‐out” progressive multifocal leukoencephalopathy in idiopathic CD4+ lymphocytopenia

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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.

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