Cherubism combined with epilepsy, mental retardation and gingival fibromatosis (Ramon syndrome): a case report.
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.
Department of Oral Medicine and Radiology, Tamil Nadu Government Dental College and Hospital, Chennai, 600 003, India.
J
J
Suhanya
Chakshu
C
Aggarwal
Khadijah
K
Mohideen
S
S
Jayachandran
I
I
Ponniah
D000015:Abnormalities, Multiple; D002636:Cherubism / Q000175:diagnosis* / Q000235:genetics; D002648:Child; D004827:Epilepsy / Q000175:diagnosis* / Q000235:genetics; D005260:Female; D005351:Fibromatosis, Gingival / Q000175:diagnosis* / Q000235:genetics; D006130:Growth Disorders / Q000175:diagnosis / Q000235:genetics; D006801:Humans; D008607:Intellectual Disability / Q000175:diagnosis* / Q000235:genetics; D013577:Syndrome