Erdheim-Chester disease with extensive intraaxial brain stem lesions presenting as a progressive cerebellar syndrome

Virgilio Gerald H. Evidente, Charles H. Adler, Caterina Giannini, Christopher R. Conley, Joseph E. Parisi, Geoffrey P. Fletcher

Research output: Contribution to journalArticle

20 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)576-581
Number of pages6
JournalMovement Disorders
Volume13
Issue number3
DOIs
StatePublished - May 1 1998

Keywords

  • Ataxia
  • Erdheim-Chester
  • Histiocytosis
  • Lipogranulomatosis

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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