Difficulties Diagnosing Progressive Multifocal Leukoencephalopathy in Patients Infected with the Human Immunodeficiency Virus – Case Reports

Czech version

Authors: H. Rozsypal ¹; D. Jilich ¹; P. Hubáček ²; M. Holub ¹
Authors‘ workplace: Klinika infekčních a tropických nemocí 1. LF UK a Nemocnice Na Bulovce, Praha ¹; Laboratoř molekulární genetiky, Ústav lékařské mikrobiologie a Klinika dětské hematologie a onkologie 2. LF UK a FN v Motole, Praha ²
Published in: Cesk Slov Neurol N 2013; 76/109(4): 501-507
Category: Case Report

Práce vznikla s podporou výzkumného záměru PRVOUK P2/ 1LF/ 3, podpořeno projektem MZČR koncepčního rozvoje FN Motol 00064203 a v rámci specifického vysokoškolského výzkumu SVV‑ 2012- 264506.

Autoři děkují MU Dr. Miroslavu Kašparovi, CSc., za poskytnutí CT a MR snímků.

Overview

Progressive Multifocal Leukoencephalopathy (PML) is a focal inflammatory process of white brain matter induced by polyomaviral infection that affects HIV‑ positive individuals, patients with hematologic malignancies and recently also patients on a biological treatment. PML is a typical opportunistic infection of patients with AIDS. Examination with magnetic resonance imaging (MRI) and detection of JC virus (JCV) DNA in the cerebrospinal fluid (CSF) are the most important approaches to the diagnosis of PML. Case reports document that PML diagnosis is not easy. The disease may develop before the number of CD4+ lymphocytes drops below 100 cells/ µl. Appearance of lesions on a CT scan and MRI is not completely specific and may be mistaken for brain toxoplasmosis, an ischemic lesion or other intracranial processes; sometimes it can start with one lesion only, suggesting a unifocal process. The paper draws a reader’s attention to the use of molecular biological diagnostics. It describes initial experience with JCV detection in the CSF using PCR in the Czech Republic and, at the same time, it discusses limited sensitivity of the method. PML prognosis is poor but disease progression can be slowed down by well managed combined antiretroviral therapy (cART). The initiation of cART in persons with severe immunodeficiency may in rare cases lead to paradoxical development of PML as a sign of immune reconstitution inflammatory syndrome (IRIS).

Key words:
progressive multifocal leukoencephalopathy – human immunodeficiency virus – acquired immunodeficiency syndrome – JC virus

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