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

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

Research output: Contribution to journalArticle

20 Citations (Scopus)

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 1998

Fingerprint

Erdheim-Chester Disease
Cerebellar Diseases
Brain Stem
Xanthomatosis
Adventitia
Histiocytes
Pons
Vertebral Artery
Autopsy
Magnetic Resonance Imaging
Lipids

Keywords

  • Ataxia
  • Erdheim-Chester
  • Histiocytosis
  • Lipogranulomatosis

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

Cite this

Erdheim-Chester disease with extensive intraaxial brain stem lesions presenting as a progressive cerebellar syndrome. / Evidente, Virgilio Gerald H; Adler, Charles Howard; Giannini, Caterina; Conley, Christopher R.; Parisi, Joseph E; Fletcher, Geoffrey P.

In: Movement Disorders, Vol. 13, No. 3, 05.1998, p. 576-581.

Research output: Contribution to journalArticle

Evidente, Virgilio Gerald H ; Adler, Charles Howard ; Giannini, Caterina ; Conley, Christopher R. ; Parisi, Joseph E ; Fletcher, Geoffrey P. / Erdheim-Chester disease with extensive intraaxial brain stem lesions presenting as a progressive cerebellar syndrome. In: Movement Disorders. 1998 ; Vol. 13, No. 3. pp. 576-581.
@article{4a6ce830b12d44ee83de16ebe016c4a2,
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 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.",
keywords = "Ataxia, Erdheim-Chester, Histiocytosis, Lipogranulomatosis",
author = "Evidente, {Virgilio Gerald H} and Adler, {Charles Howard} and Caterina Giannini and Conley, {Christopher R.} and Parisi, {Joseph E} and Fletcher, {Geoffrey P.}",
year = "1998",
month = "5",
doi = "10.1002/mds.870130335",
language = "English (US)",
volume = "13",
pages = "576--581",
journal = "Movement Disorders",
issn = "0885-3185",
publisher = "John Wiley and Sons Inc.",
number = "3",

}

TY - JOUR

T1 - Erdheim-Chester disease with extensive intraaxial brain stem lesions presenting as a progressive cerebellar syndrome

AU - Evidente, Virgilio Gerald H

AU - Adler, Charles Howard

AU - Giannini, Caterina

AU - Conley, Christopher R.

AU - Parisi, Joseph E

AU - Fletcher, Geoffrey P.

PY - 1998/5

Y1 - 1998/5

N2 - 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.

AB - 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.

KW - Ataxia

KW - Erdheim-Chester

KW - Histiocytosis

KW - Lipogranulomatosis

UR - http://www.scopus.com/inward/record.url?scp=0031899086&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0031899086&partnerID=8YFLogxK

U2 - 10.1002/mds.870130335

DO - 10.1002/mds.870130335

M3 - Article

C2 - 9613758

AN - SCOPUS:0031899086

VL - 13

SP - 576

EP - 581

JO - Movement Disorders

JF - Movement Disorders

SN - 0885-3185

IS - 3

ER -