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1/10. 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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2/10. 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.
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3/10. 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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4/10. 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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5/10. 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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6/10. 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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7/10. 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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8/10. 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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ranking = 6
keywords = interna
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9/10. Leukocyte-marrow scintigraphy in hyperostosis frontalis interna.

    hyperostosis frontalis interna is the term used to describe the thickening of the frontal bones of the skull. This thickening of the frontal bones is accompanied by an increase in the diploic space which results in an increased quantity of hematopoietically active marrow. Increased frontal bone uptake of labeled leukocytes has been reported in this condition, and the symmetric appearance of this activity may suggest its benign etiology. We have encountered a case of hyperostosis frontalis interna in which the uptake of labeled leukocytes was asymmetric and marrow scintigraphy confirmed that the activity seen was due to marrow not infection.
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10/10. Deficits on self ordered tasks associated with hyperostosis frontalis interna.

    A 74 year old patient, EW, with dorsolateral frontal cortical compression due to hyperostosis frontalis interna, in the absence of the Morgagni or Stewart-Morel syndromes, is described. In addition to conventional neuropsychological measures EW was administered one nonspatial and two spatial self ordered working memory tasks, as well as a standard measure of fluid intelligence or g. She showed impaired performance on all three self ordered working memory tasks compared with a normal control group of 10 subjects matched for age, education, sex, and IQ. By contrast, her performance on the fluid intelligence test was comparable with that of the controls. It is concluded that the compression of dorsolateral frontal cortex accompanying hyperostosis frontalis interna may produce selective cognitive impairment.
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keywords = interna
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