AUTHOR=Graf Lisa M. , Rosenkranz Sina C. , Hölzemer Angelique , Hagel Christian , Goebell Einar , Jordan Sabine , Friese Manuel A. , Addo Marylyn M. , Schulze zur Wiesch Julian , Beisel Claudia 

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

JOURNAL=Frontiers in Neurology

VOLUME=12

YEAR=2021

URL=https://www.frontiersin.org/journals/neurology/articles/10.3389/fneur.2021.632535

DOI=10.3389/fneur.2021.632535

ISSN=1664-2295

ABSTRACT=<p><bold>Background:</bold> 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.</p><p><bold>Methods:</bold>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.</p><p><bold>Results:</bold>We identified 37 patients with definite, probable, and possible PML diagnosis. 36 patients (97%) had underlying immunosuppressive disorders such as HIV/AIDS (<italic>n</italic> = 17; 46%), previous treatment with monoclonal antibodies (<italic>n</italic> = 6; 16%), hematological or oncological malignancies (<italic>n</italic> = 6; 16%), sarcoidosis (<italic>n</italic> = 5; 14%), solid organ transplantation (<italic>n</italic> = 1; 3%), and diagnosis of mixed connective tissue disease (<italic>n</italic> = 1; 3%). In only one patient no evident immunocompromised condition was detected (<italic>n</italic> = 1; 3%). Treatment attempts to improve the outcome of PML were reported in 13 patients (<italic>n</italic> = 13; 35%). Twenty seven percent of patients were lost to follow-up (<italic>n</italic> = 10). Twenty four-month survival rate after diagnosis of PML was 56% (<italic>n</italic> = 15).</p><p><bold>Conclusion:</bold> 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.</p>