Die progressive multifokale Leukoenzephalopathie (PML) - Diagnose, Pathogenese und Therapieoptionen

 

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Zusammenfassung

Das humane Polyomavirus JC (JCV) infiziert mehr als 80 % der Menschheit in den ersten fünf bis zehn Lebensjahren. Kommt es, vor allem bei immunsupprimierten Patienten, insbesondere bei HIV-infizierten, zu einer Vermehrung des Virus in Oligodendrozyten, so entwickelt sich die nahezu immer tödlich verlaufende Entmarkungskrankheit progressive multifokale Leukoenzephalopathie (PML). Neben dieser Erkrankung steht das JCV im Verdacht, an der Entstehung einer Reihe von Tumoren, insbesondere von Hirntumoren wie dem Medulloblastom aber auch von Kolonkarzinomen, beteiligt zu sein. Eine gesicherte und wirksame Behandlung der PML ist nicht bekannt, allerdings ist seit der Einführung der hochaktiven antiretroviralen Therapie (HAART) eine deutliche Verlängerung der Überlebenszeit bei HIV-Infizierten von etwa 6 Monaten auf 3,4 - 4,5 Jahre beobachtet worden.

Abstract

The human polyomavirus JC (JCV) infects during the first five to ten years more than 80 % of the human population. Replication of JCV in oligodendrocytes in immunosuppressed patients, in particular in HIV infected patients, causes the almost always-fatal demyelinating disorder progressive multifocal leukoencephalopathy (PML). Besides this disorder, JCV appears to be connected with the development of several tumor types, in particular with certain brain tumors such as medulloblastoma but also with that of colon carcinomas. A proven and effective therapy of PML is not known. Since the introduction of highly active antiretroviral therapy, however, the average time of survival in HIV infected people has risen from about 6 months to 3.4 to 4.5 years.

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Dr. med. Frank Weber

Sektion Neurologie · Max-Planck-Institut für Psychiatrie

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80804 München

Email: fweber@mpipsykl.mpg.de