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Erdheim‐chester disease with extensive intraaxial brain stem lesions presenting as a progressive cerebellar syndrome
Author(s) -
Evidente Virgilio Gerald H.,
Adler Charles H.,
Giannini Caterina,
Conley Christopher R.,
Parisi Joseph E.,
Fletcher Geoffrey P.
Publication year - 1998
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.870130335
Subject(s) - medicine , pathology , erdheim–chester disease , cerebellar diseases , cerebellum , disease , neuroscience , psychology , histiocytosis
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.