Friday, May 23, 2008

Rosai-Dorfman disease presenting with widespread intracranial and spinal involvement

Kidd DP, Revesa T, Miller NR. Neurology 2006; 67:1551-1555.
Disease is also known as sinus histiocytosis with masssive lymphadenopathy. It has a much more benign prognosis than histiocytosis X. A registry published in 1990 of 238 patients showed that of 238 patients, 49 had a lasting remission, 165 had stable disease not requiring treatment, and 21 had died 4 of the disease. It presents in the third to fifth decade with cervical lymphadenopathy often preceded by a viral infection, with elevated ESR and polyclonal gammopathy. Steroids and/or XRT is used for treatment. Authors present two neurologic cases. One mimicked a sellar meningioma with loss of vision, hearing and smell. A gallium scan and chest CT showed chest lesions. The other presented with four months of progressive visual loss. Only 4 % have neurologic involvement (8 patients) five with spinal dural based lesions, three with intracranial epidural lesions.

My question: cases did not describe cervical adenopathy so why was diagnosis made?

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