Zusammenfassung
Neurologische paraneoplastische Syndrome sind seltene, meist immunvermittelte Begleiterscheinungen
von bösartigen Tumoren. Unter den zahlreichen Tumoren sind das Ovarial- und das Mammakarzinom
eine häufige Ursache und damit eine Herausforderung an den Gynäkologen. Das typische
Erscheinungsbild besteht in der raschen Entwicklung einer schweren neurologischen
Behinderung bei einem bisher nicht bekannten und lokal begrenzten Tumor. Neben der
paraneoplastischen zerebellären Degeneration mit einer schweren Ataxie als häufigstem
Erscheinungsbild sind die limbische Enzephalitis, die Hirnstammenzephalitis, die sensorische
Neuronopathie, das Stiff-Person-Syndrom, die Dermatomyositis und die paraneoplastische
Retinopathie beschrieben. Der Meilenstein für die Diagnose sind onkoneurale Antikörper
im Serum und Liquor, die sowohl an Tumorzellen als auch an Nervenzellen binden. Neurologische
paraneoplastische Syndrome bei Frauen mit Ovarial- und Mammakarzinomen haben immer
eine ungünstige Prognose. Die meisten Patienten bleiben trotz einer effektiven Antitumortherapie,
die Voraussetzung für eine wirksame Immuntherapie ist, schwer behindert und die Mortalität
ist hoch.
Abstract
Paraneoplastic neurologic disorders are rare manifestations of a malignant neoplasm
and mostly immune-mediated. Out of a broad spectrum of tumors ovarian and breast carcinomas
are frequently the underlying cause presenting a challenge for the gynecologist. The
combination of a rapidly progressive severe neurologic disability and a small, as
yet unknown tumor is the typical clinical presentation. In addition to the most frequent
paraneoplastic cerebellar degeneration resulting in severe ataxia, limbic encephalitis,
brain stem encephalitis, sensory neuropathy, stiff-person syndrome, dermatomyositis
and paraneoplastic retinopathy have been described. The cornerstone for the diagnosis
are onconeural antibodies in serum and cerebrospinal fluid reacting with both the
nervous system and the underlying cancer. Paraneoplastic neurologic syndromes in women
with ovarian or breast cancer imply a grave prognosis. Although antitumor therapy
may be effective in many cases and are essential for the success of additional immunotherapy,
most patients remain severely disabled and mortality is remarkably high.
Schlüsselwörter
Paraneoplastische Syndrome - gynäkologische Tumoren - Neurologische Symptomatik -
onkoneurale Antikörper
Key words
Paraneoplastic syndromes - gynecologic tumors - neurologic signs - onconeural antibodies
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Prof. Dr. Roland Besser
Neurologische Klinik
Klinikum Krefeld
Lutherplatz 40
47805 Krefeld
Email: neurologie.besser@klinikum-krefeld.de
