Spinal cord compression as a result of Rosai-Dorfman disease of the upper cervical spine in a child.
BACKGROUND
Rosai-Dorfman disease is characterized by massive painless cervical lymphadenopathy, but can also include nasal obstruction, tonsillar enlargement, or hearing abnormalities. The disease occurs most often in the third and fourth decades. Most authors have suggested that it represents either an autoimmune disease or a reaction to an infectious agent that has yet to be discovered. Less than 50 cases of central nervous system involvement have been reported.


METHODS
We report a child with cervical spinal cord compression due to Rosai-Dorfman disease of the cervical spine.


CONCLUSIONS
This disease has variable outcomes from relatively benign to insidious over decades. Our case is unusual in that the patient was a child and had involvement of the spine with resultant cord compression, a combination that has been reported rarely.
Tubbs
R Shane
RS
Department of Cell Biology, University of Alabama at Birmingham, USA. richard.tubbs@ccc.uab.edu
Kelly
David R
DR
Mroczek-Musulman
Elizabeth C
EC
Hammers
Yuki A
YA
Berkow
Roger L
RL
Oakes
W Jerry
WJ
Grabb
Paul A
PA
D000293:Adolescent; D018949:Antigens, CD1 / Q000032:analysis; D001706:Biopsy; D002574:Cervical Vertebrae / Q000473:pathology; D003937:Diagnosis, Differential; D006644:Histiocytes / Q000473:pathology; D015618:Histiocytosis, Sinus / Q000150:complications* / Q000175:diagnosis / Q000473:pathology; D006801:Humans; D008297:Male; D019547:Neck Pain / Q000209:etiology* / Q000473:pathology; D010294:Parietal Bone / Q000473:pathology; D013117:Spinal Cord Compression / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology
D018949:Antigens, CD1; C419393:CD1a antigen