Cerebral Erdheim-Chester disease: case report and review of the literature
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Erdheim-Chester disease is a rare systemic non-Langerhans histiocytosis of unknown etiology that affects multiple organ systems. Cerebral involvement is most often caused by extra-axial masses of foamy histiocytes, whereas intraparenchymal manifestations are less frequent. Review of the literature yielded a total of seven patients with increased signal intensities on T2-weighted images in both dentate nuclei and the peridentate regions. We report on a 44-year-old man with biopsy-proven Erdheim-Chester disease and slowly progressive cerebellar dysfunction. MRI showed additional symmetrical hyperintense signal changes in the superior cerebellar peduncle as well as in the trigonum lemnisci on coronal FLAIR images. The widespread neurological manifestations of cerebral Erdheim-Chester disease and differential diagnosis are discussed.
KeywordsErdheim-Chester disease Cerebral involvement Magnetic resonance imaging
The authors would like to thank Prof. Dr. J. Freyschmidt, Department of Radiology, Central Hospital of Bremen, Germany, and Prof. Dr. H. Ostertag, Institute of Pathology, Nordstadt Hospital of Hannover, Germany, for setting the diagnosis in this patient, and Marina Heibel for her technical assistance.
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