Examining the frontal subcortical brain vulnerability hypothesis in children with neurofibromatosis type 1: Are T2-weighted hyperintensities related to executive dysfunction?

Objective: It was hypothesized that neuropsychological impairments in children with neurofibromatosis type I (NF1) are associated with brain areas of increased T2-weighted signal intensity on MRI. Systematic and extensive examination of this hypothesis remains however scarce, particularly regarding executive dysfunction whereas hyperintensities are located preferentially in frontal-sub-cortical networks. In this study, we compared the executive functioning profile with characteristics of brain hyperintensities in children with NF1.

Relationships between bone mass and micro-architecture at the mandible and iliac bone in edentulous subjects: a dual X-ray absorptiometry, computerised tomography and microcomputed tomography study.

Objectives: To compare bone volume, bone mineral density, cortical thickness and bone micro-architecture in a series of paired mandibular and iliac bone samples analysed by various imagery techniques to see whether relationships exist between the various techniques and between mandibular and iliac bone.

Materials And Methods: Bone samples from the mandible and ilium were harvested in 20 cadavers and analysed by dual energy X-ray absorptiometry (DXA), computerised tomography (CT) on a conventional hospital machine and microCT.

Results: Significant correlations were found between Hounsfield density obtained by CT, and bone mass determined by microCT but not with DXA values.

[Silent sinus syndrome associated with intrasinusal ossification].

Background: The silent sinus syndrome (SSS) is a rare cause of diplopia and facial asymmetry. It is commonly attributed to a sinus atelectasis secondary to ostiomeatal obstruction. Surgical dissection of the maxillary sinus mucosal lining is known to cause auto-obliteration by bone formation.

Extreme oral manifestations in a Marfan-type syndrome.

A 12-year-old girl with an otherwise typical Marfan syndrome (Ghent criteria fulfilled) presented with highly unusual oral manifestations consisting of supernumerary teeth and severe dental crowding. Pathological examination of the supernumerary teeth revealed an elevated number of pulpoliths. No mutation in the FBN1, TGFBR1 and TGFBR2 genes was identified despite exhaustive screening, suggesting that another gene defect could explain this association of marfanoid features with dental abnormalities.

Correlating implant stability to bone structure.

Objectives: The aim of this study was to demonstrate a possible correlation between bone microarchitecture and primary implant stability.

Material And Methods: Twenty-two implants (Ankylos((R)) and Straumann((R))) were inserted into the maxillae and mandibles of human cadavers. Bone structure was determined by computed tomography in three specimens (male, age 53; female, 67; female, 80).