[Calcification of the falx cerebri. A pathognomonic symptom of Gorlin-Goltz syndrome]

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.