TY - JOUR T1 - Neurology and the histiocytoses: a case of Rosai-Dorfman-Destombes disease JF - Practical Neurology JO - Pract Neurol SP - 396 LP - 406 DO - 10.1136/practneurol-2022-003398 VL - 22 IS - 5 AU - Antonia S Carroll AU - Carolynne M Doherty AU - Julian Blake AU - Stephen J Hunt AU - Chandrashekar Hoskote AU - Feargal McNicholl AU - Rahul Phadke AU - Oonagh Sheehy AU - Fion D Bremner AU - Shirley D'Sa AU - Christopher McNamara AU - Mary M Reilly Y1 - 2022/10/01 UR - http://pn.bmj.com/content/22/5/396.abstract N2 - 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. ER -