Erdheim Chester disease – 25 year history with early CNS involvement

Claire Rice, C A Hall, Paul McCoubrie, S Renowden, N Cohen, Neil Scolding

Research output: Contribution to journalArticle (Academic Journal)peer-review

4 Citations (Scopus)
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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 CNS 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.
Original languageEnglish
Article number216747
Number of pages4
JournalBMJ Case Reports
Publication statusPublished - 4 Oct 2016


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