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1/22. P300 and executive function alterations: possible links in a case of Morgagni-Stewart-Morel syndrome.

    To evaluate possible cause-effect relationships between hyperostosis frontalis interna and cognitive dysfunction, we performed a neurophysiological (event-related potentials, ERPs) and neuropsychological study in a case of Morgagni-Stewart-Morel (MSM) syndrome associated with frontal lobe compression. Neuropsychological evaluation evidenced selective impairment of executive function. Visual and auditory oddball ERPs revealed delayed P300 latency and reduced auditory P300 amplitude with multi-peaked morphology. ERP abnormalities and cognitive dysfunction could be due to the frontal bone-cortex conflict documented by neuroradiological investigations. ( info)

2/22. Leontiasis ossea in a patient with hyperparathyroidism secondary to chronic renal failure.

    osteitis fibrosa describes the bone changes seen in renal osteodystrophy secondary to longstanding hyperparathyroidism. We report a 19-year-old man with longstanding chronic renal failure with a severe form of osteitis fibrosa affecting the jaws and other maxillofacial bones causing bizarre facial and dental deformity in a patient-uraemic leontiasis ossea. ( info)

3/22. Maxillary brown tumor and uremic leontiasis ossea in a patient with chronic renal insufficiency.

    Findings of renal osteodystrophy in cranial bones are not uncommon and include osteomalacia, osteosclerosis, erosion of the cortical bone, brown tumors and resorption of the lamina dura. However, massive thickening of the cranial vault and facial bones, called uremic leontiasis ossea, have been reported very rare. In the present article, we describe the case of an uncooperative female patient with a brown tumor, involving the left maxillary sinus and massive thickening of the cranial vault and facial bones, secondary to severe secondary hyperparathyroidism during 8 years of regular hemodialysis treatment. ( info)

4/22. hyperostosis frontalis interna: case report and review of literature.

    hyperostosis frontalis interna (HFI) has been reported in high frequency among post-menopausal elderly women. Although it was widely discussed in the past, this entity is rarely mentioned in the current pathology literature. We report a postmortem case of a 56 yr-old post-menopausal woman with irregular thickening of the internal surface of the frontal bone. histology revealed an organized trabecular pattern with overall thickening of the cancellous bone. The periosteum and cortical bone were unaffected. The finding was considered to be unrelated to her death. HFI should be recognized as a benign entity and distinguished from other disorders that involve the frontal skull bone, such as Paget's disease, acromegaly, and malignancy. The etiology of HFI is unknown, but current hypotheses implicate hormonal stimulation. ( info)

5/22. Morgagni Stewart Morel syndrome--additional features.

    A case of Morgagni Stewart Morel syndrome with progressive depression in frontal bone, headache, transient monoparesis, obesity; imbalance, neuropsychiatric symptoms and recurrent disc prolapse with absent right radial pulse is discussed. This syndrome was first mentioned 235 years back, but till now exact pathology is not known. Balance assessment using dynamic posturography was done, which revealed abnormal vestibular function. To our knowledge this is the first case examined for Dynamic Posturography. ( info)

6/22. hyperostosis frontalis interna, a genetic disease?: Two medieval cases from Southern poland.

    Two cases of thickening of the internal tables of the frontal bones (hyperostosis frontalis interna, (HFI)) have been examined. These were two female skeletons from the 16th century Dominican Church in Raciborz (Southwest poland). The similarity of their morphological and metrical traits indicates that they could be related, and suggests that HFI is likely to have a genetic base. These two skeletons are the subject of an analysis which may possibly throw some new light on the controversial and continually disputed nature of this illness. ( info)

7/22. Evidence of a genetic basis of Morgagni-Stewart-Morel syndrome. A case report of identical twins.

    We report two 71-year-old female monozygotic twins presenting with advanced hyperostosis frontalis interna, obesity, shortness and cognitive impairment. They both have suffered from generalized seizures since their early adulthood. Moreover, the patients showed some additional conditions only occurring in one individual or the other such as migraine, marked recurrent depressive disorder or polyarthrosis. The symptoms common to both twins appear to correspond to the Morgagni-Stewart-Morel syndrome and indicate a genetic basis of this disorder as these features occur in genetically identical patients. ( info)

8/22. Facial leontiasis ossea: a rare presentation of hyperparathyroidism secondary to chronic renal insufficiency.

    The term renal osteodystrophy is often used in a generic sense to include skeletal disorders of patients with chronic renal failure due to secondary hyperparathyroidism. The prevalence of this condition among patients on hemodialysis is considerably high. However, extreme forms such as facial leontiasis ossea are very rare, only 2 well-characterized cases having been reported thus far in the literature. In the present article we report the case of a female patient who developed hyperparathyroidism secondary to end-stage renal disease which was manifested as facial leontiasis ossea and culminated in dysphagia and respiratory difficulties caused by excess bone tissue growth. ( info)

9/22. Management and outcome of two pregnancies in a woman with craniodiaphyseal dysplasia.

    Hyperostotic bone dysplasias are characterized by progressive hyperostosis and sclerosis of the cranium and facial bones. As a result of progressive bony overgrowth, intracranial pressure may increase and lead to brain and nerve compression, cranial nerve palsies, and an increased incidence of seizures. The long bones often exhibit defective modeling as well as variable degrees of metaphyseal and diaphyseal hyperostosis. In addition, the axial skeleton (including the pelvis) is often hyperostotic and sclerotic. The clinical features of these disorders may have relevance to the outcome of pregnancy; however, there are no reports on the management and pregnancy outcome of patients affected with hyperostotic bone disease. In this report, we describe the course of two pregnancies in a woman with craniodiaphyseal dysplasia (a rare craniotubular dysplasia). Prenatal assessment, method of delivery, choice of anesthesia, and neonatal management are discussed. Although this disorder is rare, the pathophysiologic considerations relevant to pregnancy outcome may be applicable to the management of pregnant women with other hyperostotic bone dysplasias. ( info)

10/22. hyperostosis frontalis interna: a Nubian case.

    The aim of this article is to present evidence of hyperostosis frontalis interna in a 40-year-old female recovered from a Meroitic cemetery (ca. 300 A.D.) in Sudanese Nubia. A review of the literature concerning the Morgagni-Stewart-Morel (MSM) syndrome suggests that the changes in the skull fragment are consistent with this diagnosis. This case is the earliest example of the condition so far reported, and therefore, in archaeological time and space, this is a disease not only of modern civilization, but also of antiquity. Current endocrinological reports suggest that the hyperostosis is the hallmark of a generalized disorder of bone metabolism, with increased androgens, prolactin, and somatotropins. hyperostosis frontalis interna is the central feature of a syndrome first described over 200 years ago by the early pathologist Giovanni Batistta Morgagni, professor of anatomy at Padua (1719). He found thickening of the internal tables of the frontal bones in association with virilism and obesity. Stewart (1928) and Morel (1929) independently added several neuropsychiatric problems to this complex and questioned the possibility of an endocrine basis for the syndrome. ( info)
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