Scoliosis of the cranial base: radiological and mathematical analysis using finite elements system analysis (FESA) of a case.

INTRODUCTION AND PROPOSED STUDY: Numerous deforming syndromes of the craniofacial complex involve also the symmetry of the cranial base. This study considers a particular alteration, that of 'scoliosis', in which the line Nasion-Sella-Basion-Inion is not rectilinear but curved, due to a torsion of the cranial base in the horizontal plane. MATERIALS: Plagiocephaly was studied in one patient, which was probably caused by altered timing or mechanism of closure of the cranial sutures. METHODS: This study was carried out using CT images of the patient's craniofacial complex, using standard neuroradiological points. In order to study the forces that operated in the various complexes, a mathematical analysis was applied namely, a finite element system analysis (FESA). RESULTS: From this mathematical study, which has enabled us to evaluate the forces that operate in determining the deformity, it has been possible to locate two sites of force concentration. They were located at different levels and on opposite sides. CONCLUSION: This finding could explain why the middle and lower thirds of the face were also involved.     

To blink or not to blink: fine cognitive tuning of the defensive peripersonal space

The blink reflex elicited by the electrical stimulation of the median nerve at the wrist [hand blink reflex (HBR)] is a subcortical, defensive response that is enhanced when the stimulated hand is inside the peripersonal space of the face. Such enhancement results from a tonic, top-down modulation of the excitability of the brainstem interneurons mediating the HBR. Here we aim to (1) characterize the somatotopical specificity of this top-down modulation and investigate its dependence on (2) cognitive expectations and (3) the presence of objects protecting the face, in healthy humans. Experiment 1 showed that the somatotopical specificity of the HBR enhancement is partially homosegmental, i.e., it is greater for the HBR elicited by the stimulation of the hand near the face compared with the other hand, always kept far from the face. Experiment 2 showed that the HBR is enhanced only when participants expect to receive stimuli on the hand close to the face and is thus strongly dependent on cognitive expectations. Experiment 3 showed that the HBR enhancement by hand-face proximity is suppressed when a thin wooden screen is placed between the participants' face and their hand. Thus, the screen reduces the extension of the defensive peripersonal space, so that the hand is never inside the peripersonal space of the face, even in the "near" condition. Together, these findings indicate a fine somatotopical and cognitive tuning of the excitability of brainstem circuits subserving the HBR, whose strength is adjusted depending on the context in a purposeful manner.     

Migraine and depression: bidirectional co-morbidities?

Even if the bidirectional co-morbidity between migraine and depression has been supported by epidemiological and genetic studies, many aspects of this association have not been completely understood. This may be due to the heterogeneous character of migraine and depression as well as to their multifactorial pathogenesis. In this review, we have briefly reported the more recent findings published about the co-morbidity between migraine and depression by discussing the above reported issues and the relative clinical and therapeutic implications.     

Biomarkers of periodontal inflammation in the Australian adult population

Background:  Several inflammatory biomarkers are implicated in the pathogenesis of periodontitis including interleukin-1β (IL-1β) and C-reactive protein (CRP). This study investigated the presence of these factors in gingival crevicular fluid (GCF) and their relationship to clinical and social determinants of periodontitis in the Australian population.
Methods:  Equal numbers of periodontitis cases and non-cases were sampled during oral epidemiologic examination in the National Survey of Adult Oral Health. GCF was sampled from four sites where probing pocket depth (PPD) and recession were recorded. From these, IL-1β and CRP were quantified by ELISA and the log amount of GCF IL-1β (pg) per person and the proportion of adults with detectable CRP was computed.
Results:  Periodontitis cases (n = 511) had significantly higher levels of IL-1β and CRP than non-cases (n = 562). PPD, clinical attachment loss, plaque and gingivitis indices were positively associated with elevated levels of both biomarkers. Levels of both were positively associated with age, low socio-economic position and non-Australian birth.
Conclusions:  The presence of IL-1β and CRP in GCF are associated with periodontal disease parameters within the Australian population. The levels of both biomarkers are influenced by age, education and eligibility for public dental care.     

Bilateral (opercular and paracentral lobular) polymicrogyria and neurofibromatosis type 1

Anecdotal cases of polymicrogyria (PMG; a malformation of cortical development consisting of an excessive number of small gyri with abnormal lamination) in patients with neurofibromatosis type 1 (NF1) have been described; however, the cases were unilateral and had negative NF1 genetic testing. We describe an 11-year-old girl with NF1 manifesting as a complex epileptic syndrome, including partial seizures secondarily generalized and status epilepticus, who had in association, bilateral, asymmetrical (opercular and paracentral lobular) PMG. She had a 1-bp deletion (c.1862delC) in exon 12b of the NF1 gene. It is notable that, given the key role played by the NF1 gene product, neurofibromin, in normal brain development, and the relatively high frequency of other brain findings in NF1, there are not more NF1 cases with brain malformations manifesting as PMG.     

NeuPSIG guidelines on neuropathic pain assessment

This is a revision of guidelines, originally published in 2004, for the assessment of patients with neuropathic pain. Neuropathic pain is defined as pain arising as a direct consequence of a lesion or disease affecting the somatosensory system either at peripheral or central level.Screening questionnaires are suitable for identifying potential patients with neuropathic pain, but further validation of them is needed for epidemiological purposes. Clinical examination, including accurate sensory examination, is the basis of neuropathic pain diagnosis. For more accurate sensory profiling, quantitative sensory testing is recommended for selected cases in clinic, including the diagnosis of small fiber neuropathies and for research purposes.Measurement of trigeminal reflexes mediated by A-beta fibers can be used to differentiate symptomatic trigeminal neuralgia from classical trigeminal neuralgia. Measurement of laser-evoked potentials is useful for assessing function of the A-delta fiber pathways in patients with neuropathic pain. Functional brain imaging is not currently useful for individual patients in clinical practice, but is an interesting research tool. Skin biopsy to measure the intraepidermal nerve fiber density should be performed in patients with clinical signs of small fiber dysfunction.The intensity of pain and treatment effect (both in clinic and trials) should be assessed with numerical rating scale or visual analog scale. For future neuropathic pain trials, pain relief scales, patient and clinician global impression of change, the proportion of responders (50% and 30% pain relief), validated neuropathic pain quality measures and assessment of sleep, mood, functional capacity and quality of life are recommended.     

Fiber tractography assessment in double cortex syndrome

Introduction  

Subcortical band heterotopia (SBH) or double cortex syndrome is a malformation of cortical development that may be related to intractable epilepsy and severe mental retardation or to mild epilepsy and slight mental delay or normal cognitive functions. Several studies have been performed using neuroradiological or neurophysiological techniques, like SPECT, PET, MRS, fMRI, and MEG, in attempt to better characterize this neuronal migration disorder. Recently, also diffusion tensor imaging (DTI) and fiber tracking (FT) have been used to investigate on white matter anomalies in SBH, adding more information about such gray matter anomaly.     

Elsahy-Waters syndrome: Evidence for autosomal recessive inheritance

Elsahy-Waters or branchioskeletogenital syndrome is a rare MCA/MR syndrome characterized by moderate mental retardation, hypospadias and characteristic craniofacial morphology, which includes brachycephaly, facial asymmetry, exotropia, hypertelorism/telechantus, broad nose, concave nasal ridge, underdeveloped midface, prognathism, and radicular dentin dysplasia. Here we report on a 44-year-old woman and her 45-year-old brother, born to consanguineous parents, who show a striking resemblance to the earlier described patients. The hitherto reported patients were male and in one pedigree parents were consanguineous. The present report of an affected woman and her brother, born to consanguineous parents, supports autosomal recessive inheritance of this condition. We provide a short review of all previously reported patients with Elsahy-Waters syndrome and related entities.