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Spinal cord compression as a result of Rosai–Dorfman disease of the upper cervical spine in a child

Spinal cord compression as a result of Rosai–Dorfman disease of the upper cervical spine in a child,10.1007/s00381-004-1121-1,Childs Nervous System,R.

Spinal cord compression as a result of Rosai–Dorfman disease of the upper cervical spine in a child   (Citations: 8)
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Background: Rosai-Dorf- man disease is characterized by massive painless cervical lymphad- enopathy, 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 cen- tral nervous system involvement have been reported. Case illustration: We report a child with cervical spinal cord compression due to Rosai-Dorfman disease of the cervical spine. Prognosis: 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 combi- nation that has been reported rarely.
Journal: Childs Nervous System - CHILD NERV SYST , vol. 21, no. 11, pp. 951-954, 2005
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    • ...Radiographically, the skeletal lesions of RDD are typically intramedullary and lytic [5, 11, 14]...
    • ...Lesional margins might be either poorly or sharply defined but are rarely sclerotic [5, 14]...
    • ...Central calcifications have not been reported [5, 14]...
    • ...MRI, when done, reveals low signal intensity on T1-W images and high signal intensity on T2-W images in the intramedullary lesion as well as in the soft-tissue extension [5, 14]...
    • ...Contrast enhancement on MRI has been described by very few investigators [14, 15]...
    • ...According to reviews by Tubbs et al. [14] and Oner et al. [15], three patients with concomitant cord compression and intracranial involvement, two with myelopathy caused by intradural extramedullary manifestations and three with symptomatic epidural compression had been described up to 2007...
    • ...A completely healed lesion might be characterized by a thin sclerotic rim, and eventually by the disappearance of any evidence of sclerosis [14]...

    Priya Guptaet al. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman diseas...

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