Paraneoplastische neuromuskuläre Erkrankungen

Zusammenfassung

Paraneoplastische neuromuskuläre Erkrankungen sind tumor- assoziierte Syndrome, die nicht durch eine lokale Wirkung des Tumors oder seiner Metastasen verursacht sind. Diese Erkrankungsgruppe umfasst die Polymyositis / Dermatomyositis und einige seltene Muskelerkrankungen, die Übertragungsstörungen der neuromuskulären Synapse (Lambert-Eaton myasthenes Syndrom, Neuromyotonie) und die paraneoplastischen Polyneuropathien. Die Myasthenia gravis wird in einer anderen Leitlinie beschrieben. Pathogenetisch liegt eine tumor-induzierte Autoimmunreaktion gegen Strukturen von Nerv oder Muskel zugrunde. Während bei paraneoplastischen Polyneuropathien häufig spezifische antineuronale Antikörper nachweisbar sind, finden sich bei paraneoplastischen und nicht-paraneoplastischen neuromuskulären Übertragungsstörungen jeweils die gleichen Autoantikörper. Auch die paraneoplastischen Myositiden lassen sich bisher nicht durch die Autoimmundiagnostik erkennen. Da mehr als die Hälfte dieser Erkrankungen der Entdeckung des Tumors vorausgehen, ist eine sorgfältige Tumorsuche notwendig. Die neuromuskulären Übertragungsstörungen und die Polymyositis / Dermatomyositis sprechen häufig gut auf eine immunsuppressive Therapie an. Ein Teil der paraneoplastischen Polyneuropathien stabilisiert sich unter der Therapie des Tumors, in wenigen Fällen ist ein Ansprechen auf Immunsuppressiva möglich.

Summary

Paraneoplastic neuromuscular diseases are tumour-associated syndromes, which are not caused by a local effect of the tumour or its metastases. This includes polymyositis/dermatomyositis and some rare muscle diseases, disturbances of the neuromuscular junction (Myasthenia gravis, Lambert-Eaton myasthenic syndrome and neuromyotonia) and paraneoplastic neuropathies. Myasthenia gravis is described in a separate guideline. Most of these diseases are caused by a cross-reactive autoimmune process against tumour and nervous system structures. In patients with paraneoplastic neuropathies antineuronal autoantibodies can be found sometimes. In contrast, autoantibodies in diseases of the neuromuscular junction and in muscle diseases are detectable in both paraneoplastic and non-paraneoplastic forms. Because more than 50% of the paraneoplastic neuromuscular syndromes precede the diagnosis of the tumour, a careful screening for an underlying tumour is necessary. Diseases of the neuromuscular junction and polymyositis/dermatomyositis show a good response to immunosuppressive treatment, whereas only a small group of paraneoplastic neuropathies may stabilize under tumour therapy and/or immunosuppressants.

Schlüsselwörter

Paraneoplatische neurologisches Syndrom - Autoantikörper - neuromuskuläre Synapse - Behandlung - Diagnose

Schlüsselwörter

paraneoplastic neurological syndrome - autoantibody - neuromuscular junction - treatment - diagnosis

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