We report a rare case of Erdheim-Chester disease (ECD) presenting as a progressive cerebellar syndrome and diabetie insipidus. On magnetic resonance imaging, a 7-mm extraaxial, enhancing mass was seen enveloping the right vertebral artery and was confirmed at autopsy to represent an adventitial xanthoma with lipid-laden, foamy histiocytes. The cerebellar syndrome most likely resulted from extensive histiocytic infiltration of the pons, particularly the basis pontis and middle cerebellar peduncles.
ASJC Scopus subject areas
- Clinical Neurology