Neues bei paraneoplastischen Syndromen in der Neurologie

Zusammenfassung

In nur wenigen Jahren hat sich das Spektrum und damit das Wissen um paraneoplastische Syndrome in der Neurologie deutlich erweitert. Insbesondere hat die Beschreibung neuer fakultativ paraneoplastischer Subgruppen von Enzephalitiden mit gutem Ansprechen auf immunmodulatorische Therapien das klinische und wissenschaftliche Interesse verstärkt. Das Erkennen dieser Subgruppen mittels neuartiger Autoantikörper hat unmittelbare therapeutische Konsequenzen. Die fundierte Kenntnis paraneoplastischer Syndrome in der Neurologie ist daher äußerst wichtig für den Kliniker. Neue wissenschaftliche Erkenntnisse beleuchten interessante Aspekte der Pathophysiologie paraneoplastischer Prozesse, ihrer möglichen Reversibilität und Initiierung. Neue Registerdaten helfen die Epidemiologie paraneoplastischer neurologischer Syndrome besser einzuschätzen. Der zunehmende Einsatz von „Biologika“ in der Neurologie eröffnet zukünftig neue Therapiechancen. Allerdings führt die mittlerweile entstandene Fülle an neuen Syndromen und Autoantikörpern auch vielfach zu Verwirrung und Unsicherheit. Diese Übersichtsarbeit fasst daher neue und wichtige ältere Aspekte paraneoplastischer neurologischer Syndrome, ihrer Klassifikation, Pathophysiologie und Behandlung zusammen.

Abstract

Recent years have witnessed a growing awareness of new aspects of paraneoplastic processes in neurological diseases. The discovery of new subgroups of paraneoplastic encephalitis syndromes with remarkably good response to immune therapy has spawned renewed clinical and scientific interest. Knowledge of these subgroups and associated autoantibodies is of direct therapeutic consequence and thus of the utmost importance for clinicians. New scientific insights into these syndromes shed light on issues concerning their reversibility, initiation and on paraneoplastic processes in general. Recently published data from a large European registry offer new information on epidemiology. Furthermore, the increasing utility of monoclonal antibodies in neurology is likely to offer new therapeutic avenues for the future. Nevertheless, the abundance of new autoantibodies and syndromes leads to confusion and uncertainty. This review article summarises new and important well-established aspects of paraneoplastic neurological syndromes, their new classification, pathophysiology and treatment.

Schlüsselwörter

Paraneoplastische Syndrome - Enzephalitis - Tumor

Keywords

paraneoplastic diseases - encephalitis - tumours

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