CLIPPERS-Syndrom: Eine Erkrankung mit vielen Gesichtern – ein Fallbericht

Zusammenfassung

Das CLIPPERS-Syndrom ist ein chronisch entzündliches Krankheitsbild des ZNS unklarer Ätiologie, das erstmals 2010 durch die Arbeitsgruppe um Pittock beschrieben wurde. Es ist gekennzeichnet durch typische kernspintomographische Veränderungen, vorwiegend im Hirnstamm, mit entzündlichen perivaskulären, lymphohistiozytären Infiltraten und gutem Ansprechen auf eine Glukokortikoidtherapie. Hier möchten wir den Fall eines damals 40-jährigen Patienten vorstellen, der sich initial mit den typischen klinischen und bildmorphologischen Zeichen eines CLIPPERS-Syndroms vorstellte und unter immunsuppressiver Therapie zunächst eine komplette Remission erzielte. Zwei Jahre später erfolgte eine erneute Vorstellung wegen starker Kopfschmerzen. Das durchgeführte cMRT zeigte erneute, nun konfluierende Läsionen im Kleinhirn. Die erste Hirnbiopsie zeigte eine Lymphoproliferation, erst eine erneute Hirnbiopsie konnte schließlich die vermutete Diagnose eines primären ZNS-Lymphoms stellen.

Abstract

The CLIPPERS syndrome is a chronic, inflammatory disorder of the central nervous system of unknown etiology, which was first described in 2010 by Pittock and colleagues. It is characterized by typical magnetic resonance imaging (MRI) changes with lesions mainly in the brainstem, a perivascular, lymphohistiocytic inflammatory process and significant improvement under glucocorticoid therapy. Here we describe the case of a 40-year-old male who presented initially with typical clinical and radiological signs of CLIPPERS syndrome and who achieved complete remission under immunosuppressive therapy. Two years later, he presented with severe headaches. The MRI showed a reappearance of the lesion in the cerebellum, but now with a confluent character. The first brain biopsy showed lymphoproliferation. A second brain biopsy could finally confirm the suspected diagnosis of a primary CNS lymphoma.

Schlüsselwörter

CLIPPERS - ZNS-Lymphom - Hirnbiopsie

Key words

CLIPPERS - CNS lymphoma - brain biopsy

• Literatur

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