Lack of association of cranial lacunae with intracranial hypertension in children with Crouzon syndrome and Apert syndrome: a 3D morphometric quantitative analysis

Purpose Cranial lacunae (foci of attenuated calvarial bone) are CT equivalents ofBcopper beating seen on plain skull radio-graphs in children with craniosynostosis. The qualitative presence of copper beating has not been found to be useful for the diagnosis of intracranial hypertension (IH) in these patients. 3D morphometric analysis (3DMA) allows a more systematic and quantitative assessment of calvarial attenuation. We used 3DMA to examine the relationship between cranial lacunae and IH in children with Crouzon and Apert syndromic craniosynostosis

C2 prosthesis: anterior upper cervical fixation device to reconstruct the second cervical vertebra

Destruction of the second cervical vertebra leads to a highly unstable situation. Reconstruction is difficult because the axis plays a central role in rotatory movements and has a unique function in redistributing axial loads. The axis transfers the axial load of the two lateral masses of the atlas to three surfaces on the third cervical vertebra: the two articular facets and the vertebral body. As reconstruction is difficult and the instability in this region is life threatening, pathological processes are often treated less radically compared to other areas of the cervical spine. However, this more moderate approach may result in worse outcomes and prognoses. This paper presents the development of a new implant (C2 prosthesis) and two illustrative cases describing the implementation of this new implant. The C2 prosthesis provides anterior support and therefore allows a more radical surgical approach

Protein Folding Activity of the Ribosome is involved in Yeast Prion Propagation.

6AP and GA are potent inhibitors of yeast and mammalian prions and also specific inhibitors of PFAR, the protein-folding activity borne by domain V of the large rRNA of the large subunit of the ribosome. We therefore explored the link between PFAR and yeast prion [PSI(+)] using both PFAR-enriched mutants and site-directed methylation. We demonstrate that PFAR is involved in propagation and de novo formation of [PSI(+)]. PFAR and the yeast heat-shock protein Hsp104 partially compensate each other for [PSI(+)] propagation. Our data also provide insight into new functions for the ribosome in basal thermotolerance and heat-shocked protein refolding. PFAR is thus an evolutionarily conserved cell component implicated in the prion life cycle, and we propose that it could be a potential therapeutic target for human protein misfolding diseases

Metopic synostosis

Premature closure of the metopic suture results in a growth restriction of the frontal bones, which leads to a skull malformation known as trigonocephaly. Over the course of recent decades, its incidence has been rising, currently making it the second most common type of craniosynostosis. Treatment consists of a cranioplasty, usually preformed before the age of 1 year. Metopic synostosis is linked with an increased level of neurodevelopmental delays. Theories on the etiology of these delays range from a reduced volume of the anterior cranial fossa to intrinsic malformations of the brain. This paper aims to provide an overview of this entity by giving an update on the epidemiology, etiology, evolution of treatment, follow-up, and neurodevelopment of metopic synostosis