Zusammenfassung
Das posteriore reversible Leukoenzephalopathiesyndrom (RPLS) ist eine neurologische Erkrankung, die zunehmende Aufmerksamkeit in der medizinischen Fachliteratur erfährt. Das zeigt sich deutlich in der Zunahme an Veröffentlichungen in den letzten zwei Jahren. Sie führt zu ödematösen Veränderungen der weißen Substanz besonders im posterioren Parietal- und Okzipitallappen, den Basalganglien und dem Kleinhirn. Die Erkrankung ist häufig durch neurologische Symptome wie Kopfschmerzen, zentrale Sehstörungen, Übelkeit mit Erbrechen, qualitativen und quantitativen Bewusstseinsstörungen, Orientierungsstörungen und Krampfanfällen gekennzeichnet. Das RPLS ist häufig mit einer plötzlichen hypertensiven Entgleisung assoziiert und betrifft meistens Patienten mit Eklampsie, Niereninsuffizienz und hypertensiver Enzephalopathie. Auch die Therapie mit Immunsuppressiva und Immunmodulatoren, wie Zyklosporin A, Tacrolimus, Interferon-α und Filgastrim kann ein RPLS auslösen. Eine besondere Form des RPLS ist die isolierte Leukoenzephalopathie des Hirnstammes, die v. a. durch die Diskordanz ausgeprägter kernspintomographischer Veränderungen und geringen klinischen Symptomen gekennzeichnet ist. Die Läsionen des RPLS lassen sich kernspintomographisch in der FLAIR- und T2-Gewichtung als diffuse Signalanhebungen der weißen Substanz darstellen. Die Diffusionsgewichtung ist eine wichtige differenzialdiagnostische Hilfe um das überwiegend vasogene Ödem des RPLS vom zytotoxischen Ödem einer akuten Ischämie zu unterscheiden. Die Therapie besteht in einer konsequenten und raschen Einstellung hypertoner Blutdruckwerte. Darunter ist die Symptomatik zumeist reversibel und die MRT-Veränderungen bilden sich zurück. Deshalb ist die schnelle Diagnosestellung beim RPLS besonders wichtig. Bei Verzögerung der richtigen Behandlung kann es zu bleibenden Schäden der betroffenen Hirnregionen kommen. Die vorliegende Übersichtarbeit soll den aktuellen Stand zur Pathophysiologie, Diagnostik und Therapie des RPLS darstellen. Zudem wird besonders auf die isolierte reversible Leukoenzephalopathie des Hirnstammes eingegangen.
Abstract
Reversible posterior leukoencephalopathy syndrome (RPLS) is a widely recognized neurological disorder, considering the increasing number of publications over the past two years. Oedematous cerebral white matter lesions particularly involve the posterior parietal and occipital lobes, but may also affect the brainstem, basal ganglia and cerebellum. This leads to characteristic neurological symptoms such as headache, visual disturbances, nausea and vomiting, altered mental status and seizures. This syndrome is often associated with an abrupt increase in blood pressure, mainly in patients with eclampsia, renal insufficiency and hypertensive encephalopathy. Immunosuppressive and immunomodulating drugs such as cyclosporine A, tacrolimus, interferone-α and filgastim may also lead to RPLS. A rare variant of RPLS is the isolated brainstem leukoencephalopathy, which is characterized by extensive MRI lesions associated with little clinical symptoms. The respective lesions are best visualized with FLAIR and T2-weighted magnetic resonance imaging. They show diffuse hyperintensity generally involving cerebral parieto-occipital regions, but may also selectively affect the brainstem without accompanying supratentorial lesions. Diffusion weighted imaging is an important diagnostic tool to differentiate the mainly vasogenic edema of RPLS from the cytotoxic edema of acute cerebral ischaemia. Appropriate therapy consists of rapid and sustained correction of hypertension. Symptoms can be completely reversible and MRI lesions may show complete remission. Early recognition of RPLS is extremely important, because of its benign prognosis under therapy. Delay of appropriate treatment, however, may lead to permanent damage of affected brain tissue. This review will give an overview of current knowledge of pathophysiology, diagnostic procedures and treatment options on RPLS. Special consideration will be given to reversible isolated brainstem leukoencephalopathy.
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Mark Obermann
Universitätsklinikum Essen · Klinik und Poliklinik für Neurologie
Hufelandstraße 55
45122 Essen
eMail: mark.obermann@uni-essen.de
