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

doi:10.1002/mds.870130335

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

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21 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 language	English (US)
Pages (from-to)	576-581
Number of pages	6
Journal	Movement Disorders
Volume	13
Issue number	3
DOIs	https://doi.org/10.1002/mds.870130335
State	Published - May 1998

Keywords

Ataxia
Erdheim-Chester
Histiocytosis
Lipogranulomatosis

ASJC Scopus subject areas

Neurology
Clinical Neurology

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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 H.} and Caterina Giannini and Conley, {Christopher R.} and Parisi, {Joseph E.} and Fletcher, {Geoffrey P.}",

year = "1998",

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

AU - Evidente, Virgilio Gerald H.

AU - Adler, Charles H.

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

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

Abstract

Keywords

ASJC Scopus subject areas

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