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

Mov Disord. 1998 May;13(3):576-81. doi: 10.1002/mds.870130335.

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.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Cerebellar Ataxia / diagnosis
  • Cerebellar Ataxia / pathology
  • Cerebellar Diseases / diagnosis*
  • Cerebellar Diseases / pathology
  • Cerebellar Nuclei / pathology
  • Cerebellum / pathology*
  • Diagnosis, Differential
  • Follow-Up Studies
  • Granuloma / diagnosis*
  • Granuloma / pathology
  • Histiocytosis, Langerhans-Cell / diagnosis*
  • Histiocytosis, Langerhans-Cell / pathology
  • Humans
  • Lipomatosis / diagnosis*
  • Lipomatosis / pathology
  • Magnetic Resonance Imaging
  • Male
  • Medulla Oblongata / pathology
  • Pons / pathology*