title: Erdheim-Chester disease with extensive intraaxial brain stem lesions presenting as a progressive cerebellar syndrome.
abstract: We report a rare case of Erdheim-Chester disease (ECD) presenting as a progressive cerebellar syndrome and diabetes 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.
authors.affiliation: Department of Neurology, Mayo Clinic Scottsdale, Arizona 85259, USA.
authors.forename: V G
authors.initials: VG
authors.lastname: Evidente
authors.forename: C H
authors.initials: CH
authors.lastname: Adler
authors.forename: C
authors.initials: C
authors.lastname: Giannini
authors.forename: C R
authors.initials: CR
authors.lastname: Conley
authors.forename: J E
authors.initials: JE
authors.lastname: Parisi
authors.forename: G P
authors.initials: GP
authors.lastname: Fletcher
mesh_terms: D000368:Aged; D002524:Cerebellar Ataxia / Q000175:diagnosis / Q000473:pathology; D002526:Cerebellar Diseases / Q000175:diagnosis* / Q000473:pathology; D002529:Cerebellar Nuclei / Q000473:pathology; D002531:Cerebellum / Q000473:pathology*; D003937:Diagnosis, Differential; D005500:Follow-Up Studies; D006099:Granuloma / Q000175:diagnosis* / Q000473:pathology; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis* / Q000473:pathology; D006801:Humans; D008068:Lipomatosis / Q000175:diagnosis* / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008526:Medulla Oblongata / Q000473:pathology; D011149:Pons / Q000473:pathology*