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Atypical MR presentation of Lhermitte-Duclos disease (Dysplastic gangliocytoma of the cerebellum)

Atypisches Signalverhalten von morbus lhermitte-duclos (dysplastisches gangliozytom des kleinhirns) in der MRT. Kasuistik mit literaturübersicht

Case report and review of the literature

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Summary

A 1-year-old girl presented with symptoms of nausea and vomiting, torticollis and paralysis of abducens and facial nerve. MR examination showed on Tl-weighted images a slightly hypointense mass lesion in the right cerebellar hemisphere and a moderately high signal on T2-weighted images, with parallel linear striations on the surface of the lesion. Administration of Gd-DTPA revealed an inhomogeneous, but marked enhancement of the lesion, suggesting the diagnosis of a posterior fossa neoplasm. A subtotal resection was performed. Histologic examination resulted in the diagnosis of Lhermitte-Duclos disease (LDD) (dysplastic gangliocytoma of the cerebellum). Short-term postoperative follow-up MRI showed slight but steady progression of the lesion. Review of literature revealed that this is a unique case of histologically proven LDD in a child showing contrast enhancement after administration of Gd-DTPA and lesion progression within a short postoperative interval.

Zusammenfassung

Ein einjähriges Mädchen wurde mit Kopfschmerzen, Erbrechen, Kopfschiefhaltung, Abduzens- und Fazialisparese vorgestellt. Die MRT ergab eine Raumforderung in der rechten Kleinhirnhemisphäre, hyperintens in den T2-gewichteten Sequenzen mit prominenter paralleler Streifung der Oberfläche. Nach Gd-DTPA-Gabe zeigte sich ein inhomogenes, aber deutliches Enhancement. Unter der Verdachtsdiagnose eines malignen Tumors wurde eine subtotale Resektion durchgeführt, die die Diagnose eines dysplastischen Gangliozytoms des Kleinhirns (Morbus Lhermitte-Duclos) ergab. Bei dieser kleinen Patientin mit einem histologisch gesicherten Morbus Lhermitte-Duclos zeigte sich nach Kontrastverstärkung ein deutliches Enhancement und damit, anders als in der Literatur beschrieben, ein „atypisches” Signalverhalten. Der Morbus Lhermitte-Duclos sollte daher als seltene Ursache einer intraaxialen Raumforderung der hinteren Schädelgrube differentialdiagnostisch berücksichtigt werden. Prominente Kleinhirnfoliae, hyperintens in den T2-gewichteten Aufnahmen, und möglicherweise ein Enhancement nach Kontrastmittelgabe sollten zusätzlich an die Möglichkeit eines Morbus Lhermitte-Duclos denken lassen. Wegen der Möglichkeit der Tumorprogression nach subtotaler Exstirpation ist postoperativ ein engmaschiges magnetresonanztomographisches Follow-up notwendig.

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Authors and Affiliations

  1. Abteilung Klinische Neuroradiologie der Universität, Im Neuenheimer Feld 400, D-69120, Heidelberg

    A. Dörfler, M. Forsting, T. Egelhof & K. Sartor

  2. Neurosurgery, University of Heidelberg, Heidelberg, Deutschland

    F. K. Albert

  3. Neuropathology, University of Heidelberg, Heidelberg, Deutschland

    C. Sommer

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Dörfler, A., Forsting, M., Egelhof, T. et al. Atypical MR presentation of Lhermitte-Duclos disease (Dysplastic gangliocytoma of the cerebellum). Klinische Neuroradiologie 7, 40–49 (1997). https://doi.org/10.1007/BF03043994

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