[Calcification of the Falx Cerebri. A Pathognomonic Symptom of Gorlin-Goltz Syndrome]

Overview

Journal HNO

Specialty Otorhinolaryngology

Date 2005 Feb 8

PMID 15696312

Citations 4

Authors

J T Lambrecht

S Stubinger

B Siewert

F Harle

Affiliations

Soon will be listed here.

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.

Citing Articles

Bulky supernumerary bone of the falx cerebri: case report and literature review.

Tetik A, Slama N, Crampon F, Prum G, Derombise B, Duparc F Surg Radiol Anat. 2023; 45(6):757-760.

PMID: 37069447 DOI: 10.1007/s00276-023-03143-6.

A Unique Case of Gorlin-Goltz Syndrome with Associated Sotos Syndrome.

Lata J, Kaur J Ann Maxillofac Surg. 2020; 10(1):232-237.

PMID: 32855948 PMC: 7433959. DOI: 10.4103/ams.ams_91_19.

Incidental finding of lamellar calcification of the falx cerebri leading to the diagnosis of gorlin-goltz syndrome.

Saulite I, Voykov B, Mehra T, Hoetzenecker W, Guenova E Case Rep Dermatol. 2014; 5(3):301-3.

PMID: 24403894 PMC: 3884206. DOI: 10.1159/000356146.

[Obstructive sleep apnea syndrome in the setting of Gorlin-Goltz syndrome].

Grundig H, Sinikovic B, Gunther J, Jungehulsing M HNO. 2013; 61(9):786-90.

PMID: 23463414 DOI: 10.1007/s00106-012-2655-2.

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