Clinical Presentation and Disease Course of 37 Consecutive Cases of Progressive Multifocal Leukoencephalopathy (PML) at a German Tertiary-Care Hospital: A Retrospective Observational Study.

Background: Progressive multifocal leukoencephalopathy (PML) caused by JCV is a rare but frequently fatal disease of the central nervous system, usually affecting immunocompromised individuals. Our study aims to expand the data on patient characteristics, diagnosis, clinical course, possible PML-directed treatment, and outcome of patients with PML at a German tertiary-care hospital. Methods: In this single-center observational cohort study, 37 consecutive patients with a confirmed diagnosis of PML seen at the University Medical Center Hamburg-Eppendorf from 2013 until 2019 were retrospectively analyzed by chart review with a special focus on demographics, risk factors, and clinical aspects as well as PML-directed treatment and survival. Results: We identified 37 patients with definite, probable, and possible PML diagnosis. 36 patients (97%) had underlying immunosuppressive disorders such as HIV/AIDS ( n = 17; 46%), previous treatment with monoclonal antibodies ( n = 6; 16%), hematological or oncological malignancies ( n = 6; 16%), sarcoidosis ( n = 5; 14%), solid organ transplantation ( n = 1; 3%), and diagnosis of mixed connective tissue disease ( n = 1; 3%). In only one patient no evident immunocompromised condition was detected ( n = 1; 3%). Treatment attempts to improve the outcome of PML were reported in 13 patients ( n = 13; 35%). Twenty seven percent of patients were lost to follow-up ( n = 10). Twenty four-month survival rate after diagnosis of PML was 56% ( n = 15). Conclusion: This interdisciplinary retrospective study describes epidemiology, risk factors, clinical course, and treatment trials in patients with PML at a German tertiary-care hospital. Acquired immunosuppression due to HIV-1 constituted the leading cause of PML in this monocenter cohort.
Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
(Copyright © 2021 Graf, Rosenkranz, Hölzemer, Hagel, Goebell, Jordan, Friese, Addo, Schulze zur Wiesch and Beisel.)