Long-term outcome of basilar stenosis in Erdheim–Chester disease: A case report

Erdheim-Chester disease (ECD) is a rare form of non-Langerhans cell histiocytosis. This inflammatory myeloid neoplasm is frequently complicated by neurological symptoms, but stroke is an exceptional manifestation of this disease. We report the case of a 59-year-old woman who presented a vertebrobasi...

Full description

Saved in:
Bibliographic Details
Published in:Medicine (Baltimore) 2016-09, Vol.95 (36), p.e4813-e4813
Main Authors: Mathis, Stéphane, Godenèche, Gaëlle, Haroche, Julien, Milin, Serge, Julian, Adrien, Berthomet, Aline, Baron, Clément, Palazzo, Paola, Neau, Jean-Philippe
Format: Article
Language:English
Subjects:
Clinical Case Report
Erdheim-Chester Disease - complications
Erdheim-Chester Disease - drug therapy
Female
Humans
Interferon-alpha - therapeutic use
Middle Aged
Stroke - etiology
Time Factors
Vertebrobasilar Insufficiency - etiology
Citations: Items that this one cites
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Erdheim-Chester disease (ECD) is a rare form of non-Langerhans cell histiocytosis. This inflammatory myeloid neoplasm is frequently complicated by neurological symptoms, but stroke is an exceptional manifestation of this disease. We report the case of a 59-year-old woman who presented a vertebrobasilar stroke secondary to infiltration and severe stenosis of the basilar artery, improved after interferon-alpha therapy. We performed a review of the relevant literature and reported the few other cases described. With our patient, we have found only 7 observations of cerebrovascular disorder in ECD. Most of them had supravascular arteries involvement. Stroke is a rare treatable and potentially reversible complication of ECD. The pathophysiological processes explaining stroke in this disease are uncertain, but periarterial stenosis of cerebral arteries may be a mechanism.
ISSN:0025-7974
1536-5964
DOI:10.1097/MD.0000000000004813