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10. Kongress für Infektionskrankheiten und Tropenmedizin (KIT 2010)

Deutsche Gesellschaft für Infektiologie,
Deutsche AIDS-Gesellschaft,
Deutsche Gesellschaft für Tropenmedizin und Internationale Gesundheit,
Paul-Ehrlich-Gesellschaft für Chemotherapie

23.06. - 26.06.2010, Köln

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Progressive Multifocal Leukoencephalopathy (PML) in Common Variable Immunodeficiency (CVID) with general T-cell dysfunction and reduction

Progressive Multifokale Leukoenzephalopathie bei variablen Immundefekt (CVID) und genereller T-Zelldysfunktion und -reduktion

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  • N.T. Baerlecken - Medizinische Hochschule Hannover, Klinik für Immunologie und Rheumatologie, Hannover, Germany
  • R. Jacobds - Medizinische Hochschule Hannover, Klinik für Immunologie und Rheumatologie, Hannover, Germany
  • T. Witte - Medizinische Hochschule Hannover, Klinik für Immunologie und Rheumatologie, Hannover, Germany
  • S. Niescken - Medizinische Hochschule Hannover, Klinik für Nieren- und Hochdruckerkrankungen, Hannover, Germany
  • R.E. Schmidt - Medizinische Hochschule Hannover, Klinik für Immunologie und Rheumatologie, Hannover, Germany
  • M. Stoll - Medizinische Hochschule Hannover, Klinik für Immunologie und Rheumatologie, Hannover, Germany

10. Kongress für Infektionskrankheiten und Tropenmedizin (KIT 2010). Köln, 23.-26.06.2010. Düsseldorf: German Medical Science GMS Publishing House; 2010. DocP105

doi: 10.3205/10kit160, urn:nbn:de:0183-10kit1604

Published: June 2, 2010

© 2010 Baerlecken et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by-nc-nd/3.0/deed.en). You are free: to Share – to copy, distribute and transmit the work, provided the original author and source are credited.

Outline

Text

We present a patient with progressive multifocal leukoencephalopathy and common variable immunodeficiency. Progressive multifocal leukoencephalopathy (PML) based on common variable immunodeficiency (CVID) has been described five times since 1996. None of these patients has survived longer than 12 months.

Our patient showed besides low levels of immunoglobulin, particularly type IgG, -M, -A a reduction of T-cell including CD4-cells and reduction of B-cells, and dysfunction of T-cells. PML occured 2 years later after CVID was diagnosed. Magnetic resonance imaging (MRI) revealed extensive T2-weighted hyper-intensity and T1-weighted hypo-intensity of white matter of the right pre- and post-central region with reduction of the cortical mass, which proceeded from fissura longitudinalis to the mesencephalon in right parietofrontal. In addition, a neurosurgical biopsy was performed and it revealed progressive multifocal leukoencephalopathy (PML) with positivity for JC-Virus (JCV).

Diagnostics of the underlying immunodeficiency and its consequence for its therapy are described. Experimental attempts of antiviral treatment with cidofovir, mefloquine and 5HT(2a) receptor antagonists were not able to avert a fatal outcome.