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11/13. Uremic leontiasis ossea: "bighead" disease in humans? Radiologic, clinical, and pathologic features.

    PURPOSE: To describe the clinical, radiologic, and pathologic findings in patients with uremic leontiasis ossea (ULO). MATERIALS AND methods: Five patients with renal osteodystrophy developed marked hyperostosis of the facial and cranial bones. Radiologic studies included plain radiography of the skull (n = 5), computed tomography with three-dimensional reconstruction (n = 4), magnetic resonance imaging (n = 3), and fluorine-18 sodium fluoride positron emission tomography (PET) (n = 1). Specimens from bone biopsies (three patients) were examined. RESULTS: skull and facial alterations were remarkably similar. Numerous nodules of varying attenuation and signal intensity in the widened diploic space suggested brown tumors in different stages of evolution. Biochemical data and PET findings enabled confirmation of markedly increased bone turnover. Bone specimens demonstrated severe osteitis fibrosa. After parathyroidectomy, facial changes in all patients stabilized or improved mildly. CONCLUSION: A similar entity in animals, "bighead" disease, which results from nutritional and uremic secondary hyperparathyroidism, may provide a useful animal model for ULO in humans. Mild forms of this entity may be more common than the scarcity of previous reports suggests.
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12/13. 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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keywords = hyperostosis
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13/13. 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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