Erdheim Chester disease – 25 year history with early CNS involvement

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

doi:10.1136/bcr-2016-216747

Erdheim Chester disease – 25 year history with early CNS involvement

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

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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 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 language	English
Article number	216747
Number of pages	4
Journal	BMJ Case Reports
Volume	2016
DOIs	https://doi.org/10.1136/bcr-2016-216747
Publication status	Published - 4 Oct 2016

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10.1136/bcr-2016-216747

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This is the accepted author manuscript (AAM). The final published version (version of record) is available online via BMJ Publishing Group at http://doi.org/10.1136/bcr-2016-216747. Please refer to any applicable terms of use of the publisher.
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Supplementary information PDF - Figure 4
This is the accepted author manuscript (AAM). The final published version (version of record) is available online via BMJ Publishing Group at http://doi.org/10.1136/bcr-2016-216747. Please refer to any applicable terms of use of the publisher.
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Supplementary information PDF - Figure 3
This is the accepted author manuscript (AAM). The final published version (version of record) is available online via BMJ Publishing Group at http://doi.org/10.1136/bcr-2016-216747. Please refer to any applicable terms of use of the publisher.
Other version, 100 KB
Supplementary information PDF - Figure 2
This is the accepted author manuscript (AAM). The final published version (version of record) is available online via BMJ Publishing Group at http://doi.org/10.1136/bcr-2016-216747. Please refer to any applicable terms of use of the publisher.
Other version, 183 KB
Supplementary information PDF - Figure 1
This is the accepted author manuscript (AAM). The final published version (version of record) is available online via BMJ Publishing Group at http://doi.org/10.1136/bcr-2016-216747. Please refer to any applicable terms of use of the publisher.
Other version, 237 KB

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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 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.",

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AU - McCoubrie, Paul

AU - Renowden, S

AU - Cohen, N

AU - Scolding, Neil

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AB - 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.

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VL - 2016

JO - BMJ Case Reports

JF - BMJ Case Reports

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ER -

Erdheim Chester disease – 25 year history with early CNS involvement

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