Neurologische paraneoplastische Syndrome bei gynäkologischen Malignomen

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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