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Neurology and the histiocytoses: a case of Rosai-Dorfman-Destombes disease

Carroll, Antonia S; Doherty, Carolynne M; Blake, Julian; Hunt, Stephen J; Hoskote, Chandrashekar; McNicholl, Feargal; Phadke, Rahul; ... Reilly, Mary M; + view all (2022) Neurology and the histiocytoses: a case of Rosai-Dorfman-Destombes disease. Practical Neurology 10.1136/practneurol-2022-003398. (In press). Green open access

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Abstract

The histiocytoses are a group of rare disorders characterised by the accumulation of neoplastic or non-neoplastic activated histiocytes in various tissues. Phenotypes vary widely from cutaneous lesions or lymphadenopathy that regress spontaneously to disseminated disease with poor prognosis. Neurological symptoms can be a presenting feature or appear during the course of disease. We present a challenging diagnostic and management case of Rosai-Dorfman-Destombes disease in a 48-year-old woman with a relapsing, partially steroid-responsive syndrome comprising patchy, non-length-dependent radiculoneuropathy with diffuse pachymeningitis and widespread systemic disease, and recent dramatic response to novel mitogen-activated kinase pathway inhibition. We discuss the clinical characteristics, diagnosis, recent breakthroughs in pathogenesis and emerging treatment options for Rosai-Dorfman disease and for the histiocytoses with neurological sequelae, including Langerhans cell histiocytosis and Erdheim-Chester disease.

Type: Article
Title: Neurology and the histiocytoses: a case of Rosai-Dorfman-Destombes disease
Location: England
Open access status: An open access version is available from UCL Discovery
DOI: 10.1136/practneurol-2022-003398
Publisher version: http://dx.doi.org/10.1136/practneurol-2022-003398
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher's terms and conditions.
UCL classification: UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Department of Neuromuscular Diseases
URI: https://discovery.ucl.ac.uk/id/eprint/10154089
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