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1/13. 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.
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2/13. 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.
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3/13. 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.
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4/13. 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.
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5/13. 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.
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6/13. Polyostotic fibrous dysplasia with cranial hyperostosis: new entity or most severe form of polyostotic fibrous dysplasia?

    Polyostotic fibrous (McCune-Albright) dysplasia is an uncommon nonhereditable disorder characterized by localized or widespread cystic changes in the skeleton. The limb bones are predominantly affected; craniofacial involvement is rare. We have encountered a severely affected man, with the additional manifestation of massive craniofacial hyperostosis. It is questionable whether this condition is an autonomous entity or represents the end of the spectrum of severity of polyostotic fibrous dysplasia.
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7/13. Appearance of hyperostosis frontalis interna on indium-111 leukocyte scans: potential diagnostic pitfall.

    The appearance of hyperostosis frontalis interna on an [111In]leukocyte scan is reported. Recognition of the potential for normal accumulation of 111In-labeled white blood cells within this common process involving the skull is necessary to avoid misdiagnosis.
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8/13. Unilateral hyperostosis frontalis interna. Case report.

    The authors describe a case of atypical unilateral prominent hyperostosis frontalis interna in a 50-year-old Japanese woman with psychiatric symptoms. Radiological, computerized tomographic, operative, and histological findings of this rare case are presented.
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keywords = hyperostosis
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9/13. Cranial epidural fibrous tumor associated with hyperostosis: a case report.

    This report describes an unusual frontal epidural fibrous tumor in an adult woman. There had been no prior surgical procedure, radiation therapy, or significant head trauma. Microscopically, the lesion consisted of hypocellular, sparsely vascular, dense fibrous tissue. The fibrous mass was accompanied by hyperostosis of the frontal bone. The clinical and pathological characteristics of this lesion are illustrated. The differential diagnosis is discussed with reference to the literature regarding intracranial fibrous lesions. We are unaware of previous report of similar lesions in this location.
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keywords = hyperostosis
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10/13. A special form of hyperostosis frontalis interna.

    The article describes a very severe case of hyperostosis of the frontal bone and discusses its possible differential diagnosis. What makes this case special is the fact that the osseous changes include all 3 layers of the bone. Macroscopically the lesion resembles an osteoma as described by Burkhardt (1970) and v. Eiselsberg (1906). The histological examination lead to the final diagnosis of hyperostosis frontalis interna. One must note that there were certain effects caused by a metastasis of a lobular carcinoma of the breast which influenced the osseous changes from the outer surface. The rough, spicula-like structure in the centre of the frontal squama should be put down to tumour erosion. The peripheral areas of the hyperostosis, however, still exhibit the original smoother texture of the disease. The final diagnosis in this unusual and possibly unique case was an intense hyperostosis frontalis interna with secondary changes due to the metastases of a lobular carcinoma of the breast.
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keywords = hyperostosis
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