title: Erdheim-Chester disease: 25-year history with early CNS involvement.
abstract: We report a case of Erdheim-Chester disease (ECD) with a 25-year history following initial presentation with diabetes insipidus and brainstem involvement. The exceptionally long history is particularly notable, given that ECD is a life-threatening disorder and there is a recognised association between central nervous system involvement and poor outcome. The case is a timely reminder of the presenting features of the condition, given the emergence of potential new treatment options.
authors.affiliation: Department of Clinical Neuroscience, University of Bristol, Bristol, UK.
authors.forename: C M
authors.initials: CM
authors.lastname: Rice
authors.affiliation: North Bristol NHS Trust, Bristol, UK.
authors.forename: C A
authors.initials: CA
authors.lastname: Hall
authors.affiliation: North Bristol NHS Trust, Bristol, UK.
authors.forename: P
authors.initials: P
authors.lastname: McCoubrie
authors.affiliation: North Bristol NHS Trust, Bristol, UK.
authors.forename: S A
authors.initials: SA
authors.lastname: Renowden
authors.affiliation: North Bristol NHS Trust, Bristol, UK.
authors.forename: N
authors.initials: N
authors.lastname: Cohen
authors.affiliation: Department of Clinical Neuroscience, University of Bristol, Bristol, UK North Bristol NHS Trust, Bristol, UK.
authors.forename: N J
authors.initials: NJ
authors.lastname: Scolding
mesh_terms: D001927:Brain Diseases / Q000209:etiology*; D003919:Diabetes Insipidus / Q000150:complications; D031249:Erdheim-Chester Disease / Q000150:complications*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged