Erdheim-Chester disease of the central nervous system: new manifestations of a rare disease. AJNR Am J Neuroradiol 2011 Dec;32(11):2126-31
Date
10/01/2011Pubmed ID
21960492Pubmed Central ID
PMC7964409DOI
10.3174/ajnr.A2707Scopus ID
2-s2.0-84455188350 (requires institutional sign-in at Scopus site) 47 CitationsAbstract
BACKGROUND AND PURPOSE: ECD is a rare non-Langerhans-cell histiocytosis, which can involve the CNS; therefore, CNS imaging findings have been described in only a small number of patients. To gain additional insight into the CNS manifestations of ECD, we reviewed the findings on imaging of the brain, head and neck, and spine in patients with ECD who presented to our institution. Here, we illustrate manifestations that have not, to our knowledge, been previously described.
MATERIALS AND METHODS: CT, MR imaging, and PET/CT studies of the brain, maxillofacial region, and spine were reviewed in 11 patients with ECD.
RESULTS: Four new manifestations of ECD were present, including the following: a stellate appearance of intracranial extra-axial lesions, ependymal enhancement along the lateral ventricle with deep linear extension to the lentiform nucleus, irregular enhancement in the pons, and diffuse involvement of the vertebral column on PET/CT.
CONCLUSIONS: ECD has a variety of imaging appearances in the CNS, including new manifestations described herein. Neuroradiologists should be aware of these manifestations to avoid mistaking them for other disease processes.
Author List
Sedrak P, Ketonen L, Hou P, Guha-Thakurta N, Williams MD, Kurzrock R, Debnam JMAuthor
Razelle Kurzrock MD Center Associate Director, Professor in the Medicine department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
AdultAged
Brain Diseases
Diagnostic Imaging
Erdheim-Chester Disease
Female
Humans
Male
Maxillofacial Abnormalities
Middle Aged
Rare Diseases
Spinal Diseases
