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 Volume 67, Number 9, November 14, 2006
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NEUROLOGY 2006;67:1551-1555
© 2006 American Academy of Neurology
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Rosai-Dorfman disease presenting with widespread intracranial and spinal cord involvement

Desmond P. Kidd, MD, FRCP, Tamas Revesz, MD, FRCPath and Neil R. Miller, MD, FACS

From the Department of Clinical Neurosciences (D.K.), Royal Free Hospital, London; Department of Neuropathology (T.R.), Institute of Neurology, London, UK; and the Neuro-Ophthalmology Unit (N.R.M.), Johns Hopkins Medical Institutions, Baltimore, MD.

Address correspondence and reprint requests to Dr. Desmond Kidd, Department of Clinical Neurosciences, Royal Free Hospital, Pond Street, London NW3 2QG, UK; e-mail: d.kidd{at}medsch.ucl.ac.uk

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.

Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the November 14 issue to find the title link for this article.

Disclosure: The authors report no conflicts of interest.

Received February 13, 2006. Accepted in final form July 5, 2006.


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