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November 14, 2006; 67 (9) Views & Reviews

Rosai-Dorfman disease presenting with widespread intracranial and spinal cord involvement

Desmond P. Kidd, Tamas Revesz, Neil R. Miller
First published November 13, 2006, DOI: https://doi.org/10.1212/01.wnl.0000242893.55416.8e
Desmond P. Kidd
MD, FRCP
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Tamas Revesz
MD, FRCPath
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Neil R. Miller
MD, FACS
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Citation
Rosai-Dorfman disease presenting with widespread intracranial and spinal cord involvement
Desmond P. Kidd, Tamas Revesz, Neil R. Miller
Neurology Nov 2006, 67 (9) 1551-1555; DOI: 10.1212/01.wnl.0000242893.55416.8e

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Abstract

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.

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  • Article
    • Abstract
    • History.
    • Clinical features of the systemic disease.
    • Diagnostic histologic features.
    • Case histories.
    • Neurologic involvement in Rosai-Dorfman disease.
    • Differential diagnosis.
    • Treatment and follow-up.
    • Treatment recommendations.
    • Conclusion.
    • Footnotes
    • References
  • Figures & Data
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