Multiple intracranial aneurysms associated with branchio-oto-dysplasia.
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Branchio-oto-dysplasia is characterized by abnormalities of embryonic branchial arch system and deafness inherited as autosomal dominant with variable gene expression. We present a rare case of multiple intracranial aneurysms associated with branchio-oto-dysplasia. A 40-yr-old man with severe headache presented as spontaneous subarachnoid hemorrhage on brain computed tomographic scan. The patient also manifested clinical features of branchio-oto-dysplasia and right hemifacial hypoplasia. Carotid angiogram confirmed an aneurysm in the anterior communicating artery. Intraoperative findings demonstrated multiple aneurysms in the anterior communicating artery and in the left posterior communicating artery, which were clipped successfully. Postoperative course was uneventful. This condition has not been reported previously. We also reviewed literatures to discuss whether the intracranial aneurysm was as a coincidental finding or a part of this malformation. (+info)
A comparison of concentrations of lead in human tissues.
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This postmortem study of lead concentrations in the tissues of 129 subjects is an extension to a report by Barry and Mossman (1970). Lead concentrations in bone greatly exceeded the concentrations in soft tissues and were highest in the dense bones. Bone lead concentrations increased with age in both sexes, more especially in male subjects and in dense bone, varying between mean values of 2-16 ppm in the ribs of children to over 50 ppm in the dense petrous temporal bones of elderly male adults. Male adults contained over 30% more lead in their bones than females. Mean concentrations of lead in the soft tissues varied from less than 0-1 ppm in organs such as muscle and heart to over 2 ppm in the aorta. In most tissues with lead values in excess of 0-2 ppm the male concentrations exceeded female values by about 30%. With the exception of the aorta, spleen, lung, and prostate, lead concentrations did not increase with age in the soft tissues of either sex after about the second decade of life. Children showed concentrations of lead in their soft tissues comparable to female adults, but the concentrations in bone were much lower. It is suggested that children do not possess the same capacity as adults to retain lead in bone. In male adults occupationally exposed to lead the concentrations of lead in bone exceeded the concentrations in unexposed male adults within the same age group by two-to three-fold. Soft tissue lead concentrations between the two groups were less divergent. An assessment of the total body burden of lead revealed higher levels in adult male subjects than in females at mean values of 164-8 mg compared to 103-6 mg, respectively. Over 90% of the total body burden of lead in adults was in bone, of which over 70% was in dense bone. Male adults occupationally exposed to lead had mean total body burdens of 566-4 mg Pb, of which 97% was in bone. The release of lead from bone in conjunction with calcium was not considered to be of physiological significance. Lead concentrations of hair and nails were higher than soft tissue lead concentrations and varied widely. Hair lead measurements were not considered to provide a reliable assessment of lead absorption. The concentrations of lead in tissues of a mixed group of subjects with no known occupational exposure to lead have been shown to be comparable to the findings in earlier studies. Present levels of lead in the environment are not considered to be a hazard to the health of the population in general. (+info)
Primary Ewing's sarcoma of the occipital bone--case report.
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A 17-year-old female presented with a very rare case of primary Ewing's sarcoma of the skull involving the occipitotemporal region. Systemic examination found no evidence of metastasis. The tumor was surgically removed, and the patient underwent radiotherapy and chemotherapy. Fourteen months after surgery there has been no recurrence of the tumor. Cranial primary Ewing's tumor has a good prognosis after radical surgery and adjuvant therapy. (+info)
Morphology of the temporomandibular joint in skeletal class iii symmetrical and asymmetrical cases: a study by cephalometric laminography.
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The aetiology of asymmetric growth in the mandible is not well understood. Previous studies have indicated that the functional lateral shift of the mandible in the period of prepubertal growth may translate to a true skeletal asymmetry, exclusively in skeletal Class III malocclusion. This asymmetry develops more characteristic features during the pubertal and post-pubertal growth periods. Early correction of a functional lateral shift of the mandible is recommended. The purpose of this study was to examine the relationship between the morphology of the temporomandibular joints and asymmetry in skeletal Class III malocclusion in adult female patients. Cephalometric and laminographic findings in 36 asymmetric skeletal Class III patients with a lateral shift of mandible (group 3) were compared to those of 25 symmetric skeletal Class I patients (group 1) and the same number of symmetric skeletal Class III malocclusions (group 2). All the patients had received no orthodontic treatment. The results showed that the TMJ of the side to which the mandible shifted showed a significantly narrower and shorter shape of the condyle head, smaller superior condylar space, and steeper eminence than those of the unshifted side. (+info)
Temporal bone pathology in Wegener's granulomatosis.
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This study aimed to demonstrate the temporal bone histopathology of two cases of Wegener's granulomatosis in which the initial symptoms were profound hearing loss and facial nerve palsy respectively. The first case, a woman of 44, suffered profound hearing loss which was remarkably improved by steroid and cyclophosphamide treatment for a time, and which seemed to be caused by invasion from granulation tissue filled in the tympanic cavity. The second case was a 61-year-old woman presenting with the facial nerve palsy. The bony canal of the horizontal portion of the facial nerve was destroyed due to granulation tissue which filled in the tympanic cavity, and granulomatous involvement was observed in the facial nerve. Wegener's granulomatosis can involve the middle ear and/or inner ear, causing hearing loss of conductive, mixed or sensorineural type. Pathogenesis of facial nerve palsy seems to be related to Wegener's granulomatous involvement of facial nerve, because the facial nerve palsy also resolved by using steroid and cyclophosphamide. (+info)
Tissue-specific expression of SV40 in tumors associated with the Li-Fraumeni syndrome.
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Inactivation of wild-type p53 tumor suppressor function is the primary mechanism of tumor initiation in Li-Fraumeni syndrome (LFS) individuals with germline p53 mutations. Tumors derived from LFS patients frequently retain the normal p53 allele, suggesting that alternative mechanisms in addition to gene deletion must be involved in inactivating wild-type p53 protein. DNA tumor viruses, such as SV40, target p53 for inactivation through the action of viral oncoproteins. We studied the probands from two unrelated LFS families, each of whom presented with multiple malignant neoplasms. Patient 1 developed an embryonal rhabdomyosarcoma (RMS) and a choroid plexus carcinoma (CPC), while patient 2 developed a CPC and subsequently presented with both an osteosarcoma (OS) and renal cell carcinoma (RCC). We utilized DNA sequence analysis and immunohistochemistry to determine p53 gene status in the germline and tumors, as well as evidence for SV40 T-antigen oncoprotein expression. Each patient harbored a heterozygous germline p53 mutation at codons 175 and 273, respectively. In patient 1, the normal p53 gene was lost while the mutant p53 allele was reduced to homozygosity in the RMS. Both normal and mutant genes were maintained in the CPC. In patient 2, normal and mutant p53 alleles were retained in both the CPC and RCC. Both specific PCR and immunostaining detected SV40 T-antigen in both CPCs and the RCC. In addition to chromosomal alterations, epigenetic mechanisms may disrupt p53 function during tumorigenesis. In two LFS patients, we found SV40 DNA sequences and viral T-antigen expression that could account for inactivation of the normal p53 protein. Inactivation of p53 or other tumor suppressors by viral proteins may contribute to tumor formation in specific tissues of genetically susceptible individuals. (+info)
Single flap fronto-temporo-orbito-zygomatic craniotomy for skull base lesions.
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Surgery was performed, through single flap fronto-temporo-orbito-zygomatic approach in 22 patients with skull base lesions. In two of these patients, this approach was combined with a transpetrosal approach. The pathological spectrum consisted of trigeminal neurofibromas (5), spheno-orbital meningiomas (4), carotico-ophthalmic aneurysms (4), basilar top aneurysms (2), cavernous sinus haemangiomas (2), invasive pituitary tumours (2) and one patient each of metastatic adenocarcinoma of the cavernous sinus, transcranial fungal granuloma and tubercular granuloma of the cavernous sinus. Of the 14 tumours, 10 were excised totally/near totally while a subtotal excision was achieved in four. Removal of the anterior clinoid process facilitated the clipping of all the carotico-ophthalmic aneurysms. One basilar top aneurysm was wrapped and the other clipped. One patient of fungal granuloma died of fungal meningitis and one patient of basilar top aneurysm expired as a result of thalamic infarct. The advantages of this approach included excellent exposure of the skull base lesions, making the dissection distance shorter and wider, minimal brain retraction and easy replacement of the single bone flap. (+info)
Correlation between eminence steepness and condyle disc movements in temporomandibular joints with internal derangements on magnetic resonance imaging.
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A steep articular eminence is reported to be a predisposing factor for the development of disc displacement. The purpose of this study was to evaluate the biomechanics of the temporomandibular joint (TMJ) in internal derangements and, additionally, to investigate whether a relationship exists between the steepness of the articular eminence and disc displacement, with (DDR) and without reduction (DDWR). The material consisted of the sagittal TMJ magnetic resonance images (MRIs) of 39 joints (26 DDR and 13 DDWR). Sagittal and coronal TMJ MRIs were obtained at maximum inter-cuspation and in the maximum mouth-opening position. The steepness of the articular eminence, disc, and condyle rotation, and condyle translation were investigated. A Student's t-test was performed and correlation coefficients calculated. The results revealed an increase in disc rotation in the DDR group and in condyle translation in the DDWR group (P < 0.01). Condyle rotation and the steepness of the articular eminence were similar in both groups. Disc rotation was positively correlated with condyle rotation and negatively correlated with condyle translation in the DDR group (P < 0.05, r = 0.44). Condyle translation was positively correlated with steepness of the articular eminence in the DDWR group (P < 0.01, r = 0.74). There was no correlation between movements of the disc-condyle assembly and the steepness of the articular eminence in the DDR group. Nevertheless, a relationship between condyle translation and the steepness of the articular eminence was found in the DDWR subjects. (+info)