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

HNO. 2005 Aug;53(8):701-4, 706. doi: 10.1007/s00106-004-1206-x.
[Article in German]

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.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Basal Cell Nevus Syndrome / diagnostic imaging*
  • Basal Cell Nevus Syndrome / epidemiology*
  • Brain Diseases / diagnostic imaging*
  • Brain Diseases / epidemiology*
  • Calcinosis / diagnostic imaging*
  • Calcinosis / epidemiology*
  • Child
  • Comorbidity
  • Dura Mater / diagnostic imaging*
  • Female
  • Germany / epidemiology
  • Humans
  • Male
  • Middle Aged
  • Radiography
  • Risk Assessment / methods
  • Risk Factors