J T Lambrecht1, S Stübinger, B Siewert, F Härle. 1. Klinik für Zahnärztliche Chirurgie, - Radiologie, Mund- und Kieferheilkunde, Universität Basel. J-Thomas.Lambrecht@unibas.ch
Abstract
BACKGROUND: Gorlin-Goltz syndrome is an autosomal dominant disorder with variable penetration characterized primarily by keratocysts of the jaws, multiple basal cell carcinomas, skeletal abnormalities and intracranial calcifications. METHOD: In this study, 4787 radiographs with occipitomental x-rays from the hospital archives for oral and maxillofacial surgery of the Christian Albrechts University Kiel were examined for calcifications in the area of the falx cerebri. RESULTS: Four characteristic alterations in the falx cerebri could be assigned to four groups of structures. Those in group 4 could be found only within Gorlin-Goltz syndrome patients, and differed significantly in form and extent from the remaining three groups. CONCLUSION: The plurilamellar appearance of this group could be rated as a pathognomonic symptom of the Gorlin-Goltz syndrome.
BACKGROUND: Gorlin-Goltz syndrome is an autosomal dominant disorder with variable penetration characterized primarily by keratocysts of the jaws, multiple basal cell carcinomas, skeletal abnormalities and intracranial calcifications. METHOD: In this study, 4787 radiographs with occipitomental x-rays from the hospital archives for oral and maxillofacial surgery of the Christian Albrechts University Kiel were examined for calcifications in the area of the falx cerebri. RESULTS: Four characteristic alterations in the falx cerebri could be assigned to four groups of structures. Those in group 4 could be found only within Gorlin-Goltz syndrome patients, and differed significantly in form and extent from the remaining three groups. CONCLUSION: The plurilamellar appearance of this group could be rated as a pathognomonic symptom of the Gorlin-Goltz syndrome.
Authors: L Lo Muzio; P F Nocini; A Savoia; U Consolo; M Procaccini; L Zelante; G Pannone; P Bucci; M Dolci; F Bambini; P Solda; G Favia Journal: Clin Genet Date: 1999-01 Impact factor: 4.438