Infratentorielle Progressive Multifokale Leukenzephalopathie

Zusammenfassung

Die Progressive Multifokale Leukenzephalopathie (PML), Ausdruck einer JC-Virus-Reaktivierung, ist vorrangig als supratentorielle Entzündung bei kompromittiertem Immunstatus bekannt. Als Folge der AIDS-Pandemie, jedoch auch neuer immunsuppressiver Therapien, haben Bedeutung und Verständnis dieser oft fatal verlaufenden Erkrankung erheblich zugenommen. Da eine etablierte spezifische Therapie fehlt, richtet sich die Behandlung in erster Linie auf eine Verbesserung des Immunstatus. Ein 49-jähriger HIV-positiver Patient entwickelte eine rasch progrediente Hemiataxie und Dysarthrie. Die Diagnose einer PML konnte mithilfe von MRT und JC-Virus-PCR im Liquor cerebrospinalis gesichert werden, interessanterweise mit anfangs ausschließlich zerebellärer Manifestation. Trotz kombinierter antiretroviraler Therapie verlief die Erkrankung innerhalb weniger Wochen letal. Dieser Fallbericht soll die Möglichkeit einer selektiv infratentoriellen Manifestation der PML unterstreichen.

Abstract

Progressive multifocal leukoencephalopathy (PML), manifestation of a JC virus reactivation, is mainly known as supratentorial inflammation due to immunosuppression. The importance and comprehension of this disease, that is terminal in many cases, has increased over the past years as a consequence of new possibilities of medical immunosuppression and as a consequence of the AIDS pandemia. Treatment mainly aims at improving the patient’s immunostatus as there is no established specific therapy to date. A 49-year-old HIV-positive man presented with rapid progressive hemiataxia and dysarthria. The diagnosis of PML could be made using magnetic resonance imaging and JC virus PCR testing of cerebrospinal fluid, interestingly first concerning the cerebellum only. In spite of combined antiretroviral medication the disease was lethal within a few weeks. This case report is intended to underline the possibility of a selectively infratentorial manifestation of PML.

Schlüsselwörter

Progressive Multifokale Leukenzephalopathie - infratentoriell - AIDS - Ataxie

Keywords

progressive multifocal leukoencephalopathy - infratentorial - AIDS - ataxia

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