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).
Preview |
Text
Reilly_Neurology and the histiocytoses_AAM.pdf Download (623kB) | Preview |
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.
Archive Staff Only
 |
View Item |
