Progressive Multifocal Leukoencephalopathy—A Case Report in an Immunocompetent Patient

Abstract

Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the central nervous system due to the reactivation of the JC virus, which usually occurs in immunocompromised patients and is a major opportunistic infection associated with HIV infection. We report a case of a previously healthy patient who was diagnosed with PML.

Progressive Multifocal Leukoencephalopathy Presenting as Primary CNS Malignancy in an Immunocompetent Patient

“…Our review of the literature has shown that there are only 23 reported cases of PML in immunocompetent patients [2][3][4][5][6][7][8][9][10][11]. A previous review of the literature performed by Gheuens et al yielded an additional 32 cases [9].…”

“…Identifying the reason for JCV reactivation through a thorough review of immunological studies and whole exome sequencing is especially important when faced with PML in an immunocompetent patient, as it can lend insight into the mechanism of reactivation and potential novel treatment options [ 16 ]. Finally, previous cases of PML in immunocompetent patients offered the possibility that the patient was transiently immunodeficient at some point in their health history [ 2 - 5 ]. We cannot exclude that possibility in this case.…”

“…Immunocompromised patients, those with known malignancies, and patients undergoing immunotherapy are at risk for reactivation of the virus and subsequent PML [1]. It is quite rare for a patient to present with primary PML in an immunocompetent state [2][3][4][5][6][7][8][9][10][11]. Because of this, the patient requires extensive diagnostic evaluation, leading to diagnostic brain biopsy.…”