A Case of Progressive Multifocal Leukoencephalopathy in a Fumaric Acid-Treated Psoriasis Patient With Severe Lymphopenia Among Other Risk Factors

Sinah Engel; Lara S Molina Galindo; Stefan Bittner; Frauke Zipp; Felix Luessi

doi:10.1177/11795735211037798

A Case of Progressive Multifocal Leukoencephalopathy in a Fumaric Acid-Treated Psoriasis Patient With Severe Lymphopenia Among Other Risk Factors

J Cent Nerv Syst Dis. 2021 Dec 22:13:11795735211037798. doi: 10.1177/11795735211037798. eCollection 2021.

Authors

Sinah Engel¹, Lara S Molina Galindo¹, Stefan Bittner¹, Frauke Zipp¹, Felix Luessi¹

Affiliation

¹ Department of Neurology, Focus Program Translational Neuroscience (FTN), and Immunotherapy (FZI), Rhine-Main Neuroscience Network (rmn2), University Medical Center of the Johannes Gutenberg University, Mainz, Germany.

Abstract

Progressive multifocal leukoencephalopathy (PML) is a potentially fatal condition caused by a brain infection with JC polyomavirus (JCV), which occurs almost exclusively in immunocompromised patients. Modern immunosuppressive and immunomodulatory treatments for cancers and autoimmune diseases have been accompanied by increasing numbers of PML cases. We report a psoriasis patient treated with fumaric acid esters (FAEs) with concomitant hypopharyngeal carcinoma and chronic alcohol abuse who developed PML. Grade 4 lymphopenia at the time point of PML diagnosis suggested an immunocompromised state. This case underscores the importance of immune cell monitoring in patients treated with FAEs, even more so in the presence of additional risk factors for an immune dysfunction.

Keywords: carcinoma; fumaric acid esters; progressive multifocal leukoencephalopathy; psoriasis.

Publication types

Case Reports