Paranasal sinuses in children: size evaluation of maxillary, sphenoid, and frontal sinuses by magnetic resonance imaging and proposal of volume index percentile curves.

Our objective was to establish the age-related 3D size of maxillary, sphenoid, and frontal sinuses. A total of 179 magnetic resonance imaging (MRI) of children under 17 years (76 females, 103 males) were included and sinuses were measured in the three axes. Maxillary sinuses measured at birth (mean+/-standard deviation) 7.3+/-2.7 mm length (or antero-posterior)/4.0+/-0.9 mm height (or cranio-caudal)/2.7+/-0.8 mm width (or transverse). At 16 years old, maxillary sinus measured 38.8+/-3.5 mm/36.3+/-6.2 mm/27.5+/-4.2 mm. Sphenoid sinus pneumatization starts in the third year of life after conversion from red to fatty marrow with mean values of 5.8+/-1.4 mm/8.0+/-2.3 mm/5.8+/-1.0 mm. Pneumatization progresses gradually to reach at 16 years 23.0+/-4.5 mm/22.6+/-5.8 mm/12.8+/-3.1 mm. Frontal sinuses present a wide variation in size and most of the time are not valuable with routine head MRI techniques. They are not aerated before the age of 6 years. Frontal sinuses dimensions at 16 years were 12.8+/-5.0 mm/21.9+/-8.4 mm/24.5+/-13.3 mm. A sinus volume index (SVI) of maxillary and sphenoid sinus was computed using a simplified ellipsoid volume formula, and a table with SVI according to age with percentile variations is proposed for easy clinical application. Percentile curves of maxillary and sphenoid sinuses are presented to provide a basis for objective determination of sinus size and volume during development. These data are applicable to other techniques such as conventional X-ray and CT scan.

Neuronal and nonneuronal quantitative BACE immunocytochemical expression in the entorhinohippocampal and frontal regions in Alzheimer's disease.

In this study, we quantitatively investigated the expression of beta-site amyloid precursor protein cleaving enzyme (BACE) in the entorhinohippocampal and frontal cortex of Alzheimer's disease (AD) and old control subjects. The semiquantitative estimation indicated that the intensity of BACE overall immunoreactivity did not differ significantly between AD and controls, but that a significantly stronger staining was observed in the hippocampal regions CA3-4 compared to other regions in both AD patients and controls. The quantitative estimation confirmed that the number of BACE-positive neuronal profiles was not significantly decreased in AD. However, some degeneration of BACE-positive profiles was attested by the colocalization of neurons expressing BACE and exhibiting neurofibrillary tangles (NFT), as well as by a decrease in the surface area of BACE-positive profiles. In addition, BACE immunocytochemical expression was observed in and around senile plaques (SP), as well as in reactive astrocytes. BACE-immunoreactive astrocytes were localized in the vicinity or close to the plaques and their number was significantly increased in AD entorhinal cortex. The higher amount of beta-amyloid SP and NFT in AD was not correlated with an increase in BACE immunoreactivity. Taken together, these data accent that AD progression does not require an increased neuronal BACE protein level, but suggest an active role of BACE in immunoreactive astrocytes. Moreover, the strong expression in controls and regions less vulnerable to AD puts forward the probable existence of alternate BACE functions.

Musical training intensity yields opposite effects on grey matter density in cognitive versus sensorimotor networks.

Using optimized voxel-based morphometry, we performed grey matter density analyses on 59 age-, sex- and intelligence-matched young adults with three distinct, progressive levels of musical training intensity or expertise. Structural brain adaptations in musicians have been repeatedly demonstrated in areas involved in auditory perception and motor skills. However, musical activities are not confined to auditory perception and motor performance, but are entangled with higher-order cognitive processes. In consequence, neuronal systems involved in such higher-order processing may also be shaped by experience-driven plasticity. We modelled expertise as a three-level regressor to study possible linear relationships of expertise with grey matter density. The key finding of this study resides in a functional dissimilarity between areas exhibiting increase versus decrease of grey matter as a function of musical expertise. Grey matter density increased with expertise in areas known for their involvement in higher-order cognitive processing: right fusiform gyrus (visual pattern recognition), right mid orbital gyrus (tonal sensitivity), left inferior frontal gyrus (syntactic processing, executive function, working memory), left intraparietal sulcus (visuo-motor coordination) and bilateral posterior cerebellar Crus II (executive function, working memory) and in auditory processing: left Heschl's gyrus. Conversely, grey matter density decreased with expertise in bilateral perirolandic and striatal areas that are related to sensorimotor function, possibly reflecting high automation of motor skills. Moreover, a multiple regression analysis evidenced that grey matter density in the right mid orbital area and the inferior frontal gyrus predicted accuracy in detecting fine-grained incongruities in tonal music.

Differential damage in the frontal cortex with aging, sporadic and familial Alzheimer's disease.

In order to understand relationships between executive and structural deficits in the frontal cortex of patients within normal aging or Alzheimer's disease, we studied frontal pathological changes in young and old controls compared to cases with sporadic (AD) or familial Alzheimer's disease (FAD). We performed a semi-automatic computer assisted analysis of the distribution of beta-amyloid (Abeta) deposits revealed by Abeta immunostaining as well as of neurofibrillary tangles (NFT) revealed by Gallyas silver staining in Brodman areas 10 (frontal polar), 12 (ventro-infero-median) and 24 (anterior cingular), using tissue samples from 5 FAD, 6 sporadic AD and 10 control brains. We also performed densitometric measurements of glial fibrillary acidic protein, principal compound of intermediate filaments of astrocytes, and of phosphorylated neurofilament H and M epitopes in areas 10 and 24. All regions studied seem almost completely spared in normal old controls, with only the oldest ones exhibiting a weak percentage of beta-amyloid deposit and hardly any NFT. On the contrary, all AD and FAD cases were severely damaged as shown by statistically significant increased percentages of beta-amyloid deposit, as well as by a high number of NFT. FAD cases (all from the same family) had statistically more beta-amyloid and GFAP than sporadic AD cases in both areas 10 and 24 and statistically more NFT only in area 24. The correlation between the percentage of beta-amyloid and the number of NFT was significant only for area 24. Altogether, these data suggest that the frontal cortex can be spared by AD type lesions in normal aging, but is severely damaged in sporadic and still more in familial Alzheimer's disease. The frontal regions appear to be differentially vulnerable, with area 12 having the less amyloid burden, area 24 the less NFT and area 10 having both more amyloid and more NFT. This pattern of damage in frontal regions may represent a strong neuroanatomical support for the deterioration of attention and cognitive capacities as well as for the presence of emotional and behavioral troubles in AD patients.

Les métastases orbitaires [Orbital metastasis]

PURPOSE: The purpose of this paper is to present our data and to provide some conclusions about the attitude that has to be chosen when metastasis of the orbit is suspected. PATIENTS AND METHODS: Between 1965 and 1994, 571 patients with non-traumatic orbital diseases were controlled in the department of ophthalmology of Lausanne. Thirty-four cases of metastasis of the orbit were selected, that is 24 females and 10 males, aged from 1 to 81 years. Tumors of the breast are the most frequent origin of this metastasis, followed by cutaneous melanomas and pulmonary tumors. Orbital metastasis was the first sign of a malignant process in 7 patients. The histologic diagnosis was confirmed in 15 patients. The type of treatment is presented herein and the follow-up of more than half of the cases is given. RESULTS AND CONCLUSION: Orbital metastasis can develop after a long time in patients who were previously treated for malignant tumors. In several cases, orbital metastasis was the first sign of a malignant process which starts to become general. This diagnosis has to be taken into account when a patient was treated earlier for a malignant tumor, and it is reasonable to propose a biopsy or an excision biopsy in every orbital pathology which was not confirmed by clinical or paraclinical investigations.

Simultaneous Doppelgänger and limb amputation impression in right frontal opercular stroke.

A case is described of a patient who presented almost simultaneously the impression that his left arm was amputated and the feeling of the presence of his invisible Doppelgänger. While these body scheme disorders have both been described after (right) parietal lesions, a right frontal opercular ischaemic stroke was found in the neurological work up. Diffusion tensor imaging showed that the stroke involved the ventral bundle of the superior longitudinal fasciculus that connects the parietal to the frontal lobe. The unusual clinical presentation of this frontal lesion may have been due to a 'diaschisis'-like phenomenon via the superior longitudinal fasciculus.

Psychogenic seizures and frontal disconnection: EEG synchronisation study.

Objective Psychogenic non-epileptic seizures (PNES) are paroxysmal events that, in contrast to epileptic seizures, are related to psychological causes without the presence of epileptiform EEG changes. Recent models suggest a multifactorial basis for PNES. A potentially paramount, but currently poorly understood factor is the interplay between psychiatric features and a specific vulnerability of the brain leading to a clinical picture that resembles epilepsy. Hypothesising that functional cerebral network abnormalities may predispose to the clinical phenotype, the authors undertook a characterisation of the functional connectivity in PNES patients. Methods The authors analysed the whole-head surface topography of multivariate phase synchronisation (MPS) in interictal high-density EEG of 13 PNES patients as compared with 13 age- and sex-matched controls. MPS mapping reduces the wealth of dynamic data obtained from high-density EEG to easily readable synchronisation maps, which provide an unbiased overview of any changes in functional connectivity associated with distributed cortical abnormalities. The authors computed MPS maps for both Laplacian and common-average-reference EEGs. Results In a between-group comparison, only patchy, non-uniform changes in MPS survived conservative statistical testing. However, against the background of these unimpressive group results, the authors found widespread inverse correlations between individual PNES frequency and MPS within the prefrontal and parietal cortices. Interpretation PNES appears to be associated with decreased prefrontal and parietal synchronisation, possibly reflecting dysfunction of networks within these regions.

Complete loss of vision caused by a giant mucocele of the frontal sinus.

The authors report the case of a 75-year-old man presenting with an exceptionally large giant posttraumatic mucocele of the frontal sinus years after a gunshot blast to the head. The lesion had grown so extensively that the right eye had shrunk and calcified, resulting in total monocular blindness, a complication that has been reported only once. To the best of our knowledge, it is the first time that a giant mucocele of such a large size is reported. We describe how the patient underwent surgical removal of this massive lesion, cranial base reconstruction, and a cosmetic oculoplastic procedure. The etiology, clinical presentation, and possible complications are reviewed, as well as the importance of a regular clinical follow-up and early surgical cure. Although the diagnosis and management of mucoceles are nowadays considered quite standard, the exceptional size of the lesion illustrated here emphasizes the destructive potential of such seemingly indolent lesions. Despite the benign histology of mucoceles, one should never underestimate their morbid potential or be lulled in delaying surgical cure. Large mucoceles should be removed as quickly as possible to prevent such unacceptable complications as permanent visual loss.

RAX and anophthalmia in humans: Evidence of brain anomalies.

PURPOSE: To report the clinical and genetic study of two families of Egyptian origin with clinical anophthalmia. To further determine the role of the retina and anterior neural fold homeobox gene (RAX) in anophthalmia and associated cerebral malformations. METHODS: Three patients with clinical anophthalmia and first-degree relatives from two consanguineous families of Egyptian origin underwent full ophthalmologic, general and neurologic examination, and blood tests. Cerebral magnetic resonance imaging (MRI) was performed in the index cases of both families. Genomic DNA was prepared from venous leukocytes, and direct sequencing of all the exons and intron-exon junctions of RAX was performed after PCR amplification. RESULTS: Clinical bilateral anophthalmia was observed in all three patients. General and neurologic examinations were normal; obesity and delay in psychomotor development were observed in the isolated case. Orbital MRI showed a hypoplastic orbit with present but rudimentary extraocular muscles and normal lacrimal glands. Cerebral MRI showed agenesis of the optic nerves, optic tracts, and optic chiasma. In the index case of family A, the absence of the frontal and sphenoidal sinuses was also noted. In the index case of family B, only the sphenoidal sinus was absent, and there was significant cortical atrophy. The three patients carried a novel homozygous c.543+3A>G mutation (IVS2+3A>G) in RAX. Parents were healthy heterozygous carriers. No mutations were detected in orthodenticle homeobox 2 (OTX2), ventral anterior homeobox 1 (VAX1), or sex determining region Y-box 2 (SOX2). CONCLUSIONS: This is the first report of a homozygous splicing RAX mutation associated with autosomal recessive bilateral anophthalmia. To our knowledge, only two isolated cases of anophthalmia, three null and one missense case affecting nuclear localization or the DNA-binding homeodomain, have been found to be caused by compound heterozygote RAX mutations. A novel missense RAX mutation was identified in three patients with bilateral anophthalmia and a distinct systemic and neurologic phenotype. The mutation potentially affects splicing of the last exon and is thought to result in a protein that has an aberrant homeodomain and no paired-tail domain. Functional consequences of this change still need to be characterized.

Management of diplopia secondary to neurosurgical injury of the orbital roof

BACKGROUND: Diplopia related to neurosurgical procedures is often consecutive to oculomotor nerve lesions. We hereby report an oculomotor dysfunction secondary to an orbital roof effraction and its treatment. HISTORY AND SIGNS: Following surgery for a left anterior communicating artery aneurysm, a 45-year-old woman reported vertical diplopia associated with a left orbital hematoma. The diagnosis of third cranial nerve palsy was excluded by orbital imaging which revealed an orbital roof defect with incarceration of the levator palpebrae and superior rectus. THERAPY AND OUTCOME: As neurosurgeons advised against muscle adhesiolysis, diplopia was corrected by a two-step procedure on the oculomotor muscles. We first corrected horizontal and torsional deviations by operating on the healthy eye, before correcting the vertical deviation on the fellow eye. This two-step extraocular muscle surgery allowed restoration of binocular single vision in a useful field of gaze. CONCLUSIONS: Diplopia can occur as a rare orbital complication during neurosurgical procedures. Surgery of extraocular muscles can provide good functional results

Maxillary advancement and transpalatal expansion using internal distraction in a patient with cleft lip and palate.

Correction of sagittal and transverse maxillary discrepancies in patients with cleft lip or palate remains a challenge for craniofacial surgeons. Distraction osteogenesis has revolutionized the conceptualization and approach to the craniofacial malformations and has become a reliable and irreplaceable part of the surgical armamentarium. We are reporting a case of sequential maxillary advancement and transpalatal expansion using internal distraction in a patient with unilateral cleft lip and palate presenting with severe maxillary sagittal and transverse deficiencies.

Reconstruction fronto-orbitaire complexe avec prothèse en PEEK et expansion cutanée : à propos d'un cas [Complex fronto-orbital reconstruction with a PEEK prosthesis and skin expansion: about a case].

INTRODUCTION: Reconstructions of the fronto-orbital area remain a challenge to the reconstructive surgeon, due to the functional and esthetic impact. OBSERVATION: The authors present a case of a complex fronto-orbital reconstruction with a PEEK (PolyEtherEtherKetone) implant, associated with a skin expansion. DISCUSSION: With a follow-up of over three years, the cosmetic result is excellent. The authors believe that this technique is reliable, fast with long-term good results.