Abstract
BackgroundProgressive multifocal leukoencephalopathy (PML) is caused by an opportunistic infection with JC polyoma virus (JCPyV) and mainly affects immunocompromised patients. It leads to pronounced demyelination of the central nervous system (CNS) resulting in severe disability or even death. Detection of JCPyV DNA in the cerebrospinal fluid (CSF) is usually accepted as proof for the diagnosis of PML. Routine CSF parameters, like CSF cell count, protein concentration, Qalbumin, or intrathecal immunoglobulin synthesis are mostly considered normal. However, this has not been investigated systematically.MethodsWe analyzed routine CSF parameters in a cohort of 108 PML patients that were treated at four different neurological centers in Germany. The patients exhibited different underlying conditions with natalizumab-treated multiple sclerosis (n = 54) and human immunodeficiency virus (HIV)-infection (n = 25) being the most frequent. The data were collected at the respective centers in accordance with local requirements and then jointly analyzed. The total PML cohort was compared with a control group of patients with normal pressure hydrocephalus (NPH) and idiopathic intracranial hypertension (IIH). Multiple sclerosis and HIV patients were additionally compared with their own non-PML control groups.ResultsThe PML group showed an elevated cell count (p < 0.001) compared to the control group, however, this effect was mainly driven by HIV-PML patients. This subgroup also demonstrated a significantly higher proportion of patients with a disturbed blood-CSF-barrier function.ConclusionsThis comprehensive, retrospective study on CSF diagnostic analysis in PML patients provides insight into the CSF of those patients. It demonstrates that CSF composition in PML patients may be specific for the underlying condition that predisposes for the development of PML and thus data have to be interpreted in this context.
Highlights
Progressive multifocal leukoencephalopathy (PML) is caused by an opportunistic infection with JC polyoma virus (JCPyV) and mainly affects immunocompromised patients
The underlying diagnoses included multiple sclerosis treated with natalizumab (MS/NTZ, n = 54), human immunodeficiency virus (HIV) infection (n = 25), and hematological diseases (n = 19) such as B-cell non-Hodgkin lymphoma (n = 10), multiple myeloma (n = 2), chronic lymphocytic leukemia (n = 6), and acute myeloid leukemia (n = 1)
One patient each suffered from bronchial carcinoma, sarcoidosis, microscopic polyangiitis and common variable immunodeficiency (CVID)
Summary
Progressive multifocal leukoencephalopathy (PML) is caused by an opportunistic infection with JC polyoma virus (JCPyV) and mainly affects immunocompromised patients. It leads to pronounced demyelination of the central nervous system (CNS) resulting in severe disability or even death. Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the central nervous system (CNS) caused by reactivation of JC polyoma virus (JCPyV) and leading to a predominant destruction of oligodendrocytes [1]. Favorable outcomes have been reported in PML patients treated with immune checkpoint inhibitor therapies [12,13,14] and infusion of virus-specific T cells [15]. We report the results of routine CSF parameters from 108 PML patients that were treated at four University hospitals in Germany
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