title: Epidural spinal involvement of Erdheim-Chester disease causing myelopathy.
abstract: We present a 25-year-old woman with Erdheim-Chester disease (ECD) presenting with progressive myelopathy from multiple compressive spinal epidural lesions who required cervicothoracic decompression and fusion, and summarize the literature on epidural spinal involvement of ECD. ECD is a rare non-Langerhans histiocytosis affecting multiple organ systems through infiltration and characteristically causing multifocal osteosclerosis. Central nervous system involvement, particularly of the spine, is rare.
authors.affiliation: Department of Neurosurgery, Johns Hopkins Hospital, Baltimore, MD, USA.
authors.forename: Brian Y
authors.initials: BY
authors.lastname: Hwang
authors.affiliation: Department of Neurosurgery, Johns Hopkins Hospital, Baltimore, MD, USA.
authors.forename: Ann
authors.initials: A
authors.lastname: Liu
authors.affiliation: Department of Pathology, Johns Hopkins Hospital, Baltimore, MD, USA.
authors.forename: Jason
authors.initials: J
authors.lastname: Kern
authors.affiliation: Department of Neurosurgery, Johns Hopkins Hospital, Baltimore, MD, USA.
authors.forename: C Rory
authors.initials: CR
authors.lastname: Goodwin
authors.affiliation: Department of Neurosurgery, Johns Hopkins Hospital, Baltimore, MD, USA.
authors.forename: Jean Paul
authors.initials: JP
authors.lastname: Wolinsky
authors.affiliation: Department of Neurosurgery, Stanford University Medical Center, 300 Pasteur Drive Room 201, Edwards Building MC 5327, Stanford, CA 94305, USA. Electronic address: atman@stanford.edu.
authors.forename: Atman
authors.initials: A
authors.lastname: Desai
mesh_terms: D000328:Adult; D004824:Epidural Space / Q000473:pathology*; D031249:Erdheim-Chester Disease / Q000150:complications* / Q000473:pathology*; D005260:Female; D006801:Humans; D013118:Spinal Cord Diseases / Q000209:etiology* / Q000473:pathology*
keywords: Epidural; Erdheim–Chester disease; Non-Langerhans histiocytosis; Spine