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Nervenheilkunde 2016; 35(10): 665-672
DOI: 10.1055/s-0037-1616438
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Schattauer GmbH

Myasthenie und Thymus

Myasthenia gravis and thymus

Authors

  • B. Jordan

    1   Klinik für Neurologie der Martin-Luther-Universität Halle, Halle/Saale

  • S. Zierz

    1   Klinik für Neurologie der Martin-Luther-Universität Halle, Halle/Saale
Further Information

Publication History

eingegangen am: 05 June 2016

angenommen am: 13 June 2016

Publication Date:
10 January 2018 (online)

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Zusammenfassung

Die Thymuspathologie bei der Myasthenia gravis (MG) umfasst sowohl die lymphofollikuläre Hyperplasie (LFH) bei der Frühmanifestation der Azetylcholinrezeptorantikörper (AChR-AK) positiven Form als auch Thymome. Die Diagnostik schließt die Erhebung des Antikörperstatus ein, um die Indikation zur Thymektomie bei der klassischen AChR-Antikörper positiven Form prüfen zu können. Bei radiologischem Verdacht einer mediastinalen Raumforderung kann nuklearmedizinisch die Bindung von Somatostatinrezeptoren (DOTA-TOC-PET) bei Thymomen visualisiert werden. Die Thymektomie mit Thymomresektion ist unter onkologischem Aspekt grundsätzlich indiziert. Die Diagnose der paraneoplastischen MG besitzt somit eine besondere Indikatorfunktion für den weiteren Verlauf der Thymomerkrankung.

Wenngleich die thymomassoziierte MG sich im klinischen Verlauf nicht von der klassischen Myasthenie unterscheidet, bedarf die Betreuung dieser Patienten besonderer Sorgfalt. Meist ist die Fortführung der Immunsuppression erforderlich. Gleichzeitig sind dabei die erhöhte Infektneigung, das erhöhte Risiko eines Zweitmalignoms als auch eines Thymomrezidivs zu beachten.

Summary

In Myasthenia gravis (MG) with positive acetylcholin (AChR) receptor antibodies thymic pathology includes lymphofollicular hyperplasia (LFH) in early onset disease as well as thymomas. Indication of thymectomy in generalized MG is based on measurement of pathogenic AChR antibodies. Nuclear based imaging of somatostatin receptors in thymomas may be useful in case of unknown mediastinal lesion. Indication of thymomectomy is based on an oncological treatment approach. Diagnosis of paraneoplastic myasthenia is frequently indicating the presence of a thymoma and therefore its prognostic relevance is of utmost importance.

Paraneoplastic disease resembles classical MG in further disease course and proceeded immunosuppression is necessary. However, special attention is needed in patients with thymoma due to the increased risk of infections, risk of second malignancies and thymoma recurrence.

Schlüsselwörter

Myasthenia gravis - Thymom - Lymphofollikuläre Hyperplasie - Antikörper - Somatostatin

Keywords

Myasthenia gravis - lymphofollicular hyperplasia - thymoma - somatostatin receptor - antibodies
 

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