Rosai-Dorfman disease presenting with widespread intracranial and spinal cord involvement.
Rosai-Dorfman disease is associated with a histiocytic infiltration of lymphoid tissue, but may also involve the orbit, nasopharynx, respiratory pathways, gastrointestinal tract, endocrine glands (particularly the thyroid), bone, and skin. Neurologic manifestations are rare, occurring in 4% of one series, and the diagnosis is often not made until pathology is available. Using data from two cases, we present the clinical manifestations, pathology, and treatment, and review the literature regarding the ocular and neurologic manifestations. Finally, we discuss the optimum management of this disorder.
Kidd
Desmond P
DP
Department of Clinical Neurosciences, Royal Free Hospital, London, UK. d.kidd@medsch.ucl.ac.uk
Revesz
Tamas
T
Miller
Neil R
NR
D000328:Adult; D000368:Aged; D001921:Brain / Q000473:pathology / Q000503:physiopathology; D001932:Brain Neoplasms / Q000175:diagnosis* / Q000503:physiopathology / Q000532:radiotherapy; D019299:Decompression, Surgical; D005260:Female; D006261:Headache / Q000209:etiology / Q000503:physiopathology; D006644:Histiocytes / Q000473:pathology; D015618:Histiocytosis, Sinus / Q000175:diagnosis* / Q000503:physiopathology / Q000532:radiotherapy; D006801:Humans; D008297:Male; D019635:Neurosurgical Procedures; D000857:Olfaction Disorders / Q000209:etiology / Q000503:physiopathology; D009900:Optic Nerve / Q000473:pathology / Q000503:physiopathology; D011829:Radiation Dosage; D011878:Radiotherapy / Q000379:methods / Q000592:standards; D019291:Skull Base / Q000473:pathology / Q000503:physiopathology; D019292:Skull Base Neoplasms / Q000175:diagnosis* / Q000503:physiopathology / Q000532:radiotherapy; D013116:Spinal Cord / Q000473:pathology / Q000503:physiopathology; D013120:Spinal Cord Neoplasms / Q000175:diagnosis* / Q000503:physiopathology / Q000532:radiotherapy; D016896:Treatment Outcome; D015354:Vision, Low / Q000209:etiology / Q000503:physiopathology