Document Detail


Erdheim-Chester disease with extensive intraaxial brain stem lesions presenting as a progressive cerebellar syndrome.
MedLine Citation:
PMID:  9613758     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
V G Evidente; C H Adler; C Giannini; C R Conley; J E Parisi; G P Fletcher
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Movement disorders : official journal of the Movement Disorder Society     Volume:  13     ISSN:  0885-3185     ISO Abbreviation:  Mov. Disord.     Publication Date:  1998 May 
Date Detail:
Created Date:  1998-08-10     Completed Date:  1998-08-10     Revised Date:  2005-11-17    
Medline Journal Info:
Nlm Unique ID:  8610688     Medline TA:  Mov Disord     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  576-81     Citation Subset:  IM    
Affiliation:
Department of Neurology, Mayo Clinic Scottsdale, Arizona 85259, USA.
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MeSH Terms
Descriptor/Qualifier:
Aged
Cerebellar Ataxia / diagnosis,  pathology
Cerebellar Diseases / diagnosis*,  pathology
Cerebellar Nuclei / pathology
Cerebellum / pathology*
Diagnosis, Differential
Follow-Up Studies
Granuloma / diagnosis*,  pathology
Histiocytosis, Langerhans-Cell / diagnosis*,  pathology
Humans
Lipomatosis / diagnosis*,  pathology
Magnetic Resonance Imaging
Male
Medulla Oblongata / pathology
Pons / pathology*

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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