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Head Neck Pathol. 2010 Jun;4(2):126-31. doi: 10.1007/s12105-009-0155-9. Epub 2009 Dec 11.

Cherubism combined with epilepsy, mental retardation and gingival fibromatosis (Ramon syndrome): a case report.

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1
Department of Oral Medicine and Radiology, Tamil Nadu Government Dental College and Hospital, Chennai, 600 003, India.

Abstract

Cherubism is an inherited, autosomal dominant disorder that characteristically affects the jaws of children. The disease typically manifest as a bilateral swelling with associated submandibular lymph node enlargements and usually regresses as age advances. The disease is microscopically indistinguishable from other giant cell lesions and is essentially a clinical diagnosis. The association of cherubism with gingival fibromatosis, epilepsy, mental retardation, stunted growth, and hypertrichosis is referred as Ramon syndrome. We report a case of Ramon syndrome in an 8 year old girl.

PMID:
20512637
PMCID:
PMC2878617
DOI:
10.1007/s12105-009-0155-9
[Indexed for MEDLINE]
Free PMC Article
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