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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.

Aged↗

[Japanese cases of hyperostosis frontalis interna].

Although hyperostosis frontalis interna is common in the western countries, it has been rarely reported in the literature in Japan. We had a chance to observe 5 cases diagnosed as hyperostosis frontalis interna. They were found among 10,902 patients who came to our hospital from August 1, 1993 to September 30, 1995. All the patients in these five cases are females aged 67 to 85 (mean = 74.2 years). Four of the 5 cases had been treated as hypertension, 2 as diabetes mellitus, and 1 as hyperlipoidemia. Two cases were accompanied by unruptured aneurysms. The pathology of one case accompanied by chronic subdural hematoma revealed no apparent development of Haversian systems of bone. It seems that the prevalence of this disease in Japan would increase from now on due to the fact that the life style and the diet among Japanese people has been getting westernized.

Age Factors↗

Gerontological aspects of hyperostosis frontalis interna.

Authors discuss hyperostosis frontalis interna observed in a large number of aged persons, on the basis of age and sex distribution as well as its clinical and roentgenomorphological analysis. In various forms of the ossification of the frontal bone no significant difference was found between the localisation of hyperostosis and the clinical symptoms. On other hand, there is a direct correlation between the extension and severity of hyperostosis and the frequency of occurence of the associated symptoms (obesity, hypertension). They found the aetiological classification more adequate than the morphological categorization of Moore. Their cases are discussed 1. as partial phenomenon of the Morgagni's syndrome; 2. as independent alteration, showing no other symptoms; 3. as transitionary forms inserted between the two groups mentioned above. They discuss also the question of senile, compensatory hyperostosis frontalis interna. On the basis of the study of a large autopsy material they support the opinion that there is a direct connection of this form with old age.

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Hyperostosis frontalis interna: an anthropological perspective.

Hyperostosis frontalis interna (HFI) is manifested by the accretion of bone on the inner table of the frontal bone. Despite the vast literature on HFI, ambiguity exists as to its etiology, osteogenesis, demography, and history. This stimulated the present broad-scale study of HFI which included the evaluation of 1,706 early 20th century skulls (1,007 males and 699 females) from the Hamann-Todd and Terry human osteological collections, as well as 2,019 pre-19th century East-Mediterranean, Amerindian, and Central European skulls. In addition, 72 cadavers were dissected for gross inspection and histology. Special attention was paid to the relationship of the brain and meninges to endocranial lesions. HFI is an independent condition, not a symptom of a more generalized syndrome as suggested in the past. It can appear in a variety of forms but each is the result of the same process and probably of the same etiology. Investigators' previous failure to recognize the mild stages of HFI (types A and B) as an early form of the general HFI process led to erroneous statistics and interpretations of observations. HFI should also be considered a phenomenon separate from HCI, hyperostosis cranialis diffusa (HCD), and other endostoses, even when it appears in association with them. To avoid ambiguity and facilitate the description of cranial hyperostoses, uniform nomenclature (HFI, HCD) has been recommended. HFI is rarely seen in historic populations, regardless of geographical origin. It is most commonly found among females and is believed to be associated with prolonged estrogen stimulation. While its magnitude of manifestation and frequency are much higher in females, HFI is not a purely female phenomenon. Males with hormonal disturbances such as atrophic testis were found to manifest HFI type D. HFI is associated with age insofar as it is much less frequent in females under 40 years of age. Although advanced cases of HFI (types C and D) have been observed in individuals as young as 40 years of age, it is more frequently found after age 60. The frequency of HFI type D will not increase from age 60. Type-predicted analysis by cohort reveals significant ethnic differences. Changes in African American (AA) females appear earlier in life and progress more rapidly than in European American (EA) females. Analysis of radiographs shows a discrepancy between the anatomic prevalence of HFI and its radiological recognition, which is very poor for mild cases. This apparently resulted in the misconceptions that HFI is entirely an old-age phenomenon, and that it is exclusively female. Histological analysis shows that the inner table along with the closely attached dural layer play a major role in the osteogenesis of HFI. Contrary to previous models, no evidence for diploe or ectocranial plate involvement was found. Cadaver study suggests that the predilection for the frontal area may be related to an altered blood supply and/or vascular stretching.

Adult↗

Hyperostosis frontalis interna, acromegaly and hyperprolactinaemia.

The association between hyperostosis frontalis interna (HFI), acromegaly and hyperprolactinaemia was investigated. Thirty six acromegalic patients, of whom 19 had hyperprolactinaemia, were compared with 36 randomly-selected, age-sex matched controls. There was a higher prevalence of HFI in the skull X-rays of the acromegalic cohort (P = 0.0002) when compared to the control group. This difference was apparent in both men (P = 0.01) and women (P = 0.01). Acromegalic patients with hyperprolactinaemia also expressed HFI in a higher proportion of individuals than the control group (P = 0.0001). Intra- and interobserver variability was assessed and concordance with 100% and 97% in the moderate and severe HFI sub-groups. The following sub-group analysis was undertaken: acromegalics and those acromegalics with hyperprolactinaemia were compared with the controls and a highly significant distinction was confirmed (P = 0.0007 and P = 0.00001 respectively). A relationship between HFI severity and the patient's age was noted in both male and female acromegalics. Also, the severity of HFI appeared related to disease duration in female acromegalics. The cause of HFI remains unknown but appears to be strongly associated with acromegaly, particularly in the presence of co-existent hyperprolactinaemia. The association may have symptomatic significance and the presence of HFI should be confirmed or refuted in all patients with acromegaly.

Acromegaly↗

[Paget's disease, ankylosing vertebral hyperostosis and hyperostosis frontalis interna].

Ankylosing vertebral hyperostosis and internal frontal hyperostosis have a frequency of 5% and 8% respectively. In Paget's disease, these two disorders are found with a frequency of 40% and 19.1% respectively. This frequency is not due to chance. There exists a link between the 3 diseases. Ankylosing vertebral hyperostosis is thusa new pathological association in Paget's disease. It is even the most frequent of these associations. It seems Paget's disease induces hyperostosis and not the contrary. The authors suggest the theory that cortical and periostal hypervascularisation in pagetoid bone favour the development of these hyperostoses.

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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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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.

Adult↗

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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Prevalence of hyperostosis frontalis interna in relation to body weight.

In a prospective study, hyperostosis frontalis interna (HFI) was found to be present in 49% of the subjects. The group included 259 nuns between the ages of 60 and 80. A prevalence of 84% of HFI was found in the obese subjects (greater than 40% over ideal body weight) whereas in the thin subjects HFI was found to be present in only 16%. The possible relationship between adipose tissue mass and HFI is discussed.

Adipose Tissue↗

[Unilateral hyperostosis frontalis interna coexistent with cerebral angioma].

The authors describe a rare case of coexistence of cerebral angioma with hyperostosis frontalis interna in a female patient aged 28 years, suffering from mental disturbances. The coexistence of these two diseases in one person caused considerable diagnostic and therapeutic difficulties. The described case points out that both these conditions may occur concomitantly. Another point of interest is the presence of hyperostosis frontalis interna in a young woman.

Adult↗

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.

Female↗

Hyperostosis frontalis interna and mental morbidity.

Forty-six mentally ill women with radiologically verified hyperostosis frontalis interna (HFI) were compared with 46 matched mentally ill women without HFI in respect of mental morbidity among the full siblings of the two groups. It was found that mental morbidity was lower among the siblings on mentally ill women with HFI than among those of similar mentally ill women without HFI. A reasonable interpretation of these findings is that the combination mental illness-HFI is more often exogenously determined than other forms of mental illness.

Adult↗

[Vertebral hyperostosis and hyperostosis frontalis interna].

Routine roentgenographic examination of the spine was performed in 690 patients with rheumatologic disorders. Ankylosing hyperostosis was discovered in 124 patients (18 percent). Roentgenograms of the skull revealed hyperostosis frontalis interna in 63 percent of cases, especially in female patients. No common pathogenic mechanism, however, has been discovered in these two disorders.

Adult↗

Scintigraphic findings in hyperostosis frontalis interna.

Transient diminution of activity was seen on early radionuclide images of the brain in 5 patients with hyperostosis frontalis interna and filled in on subsequent studies. Radiographs confirmed the diagnosis. In 3 patients the changes corresponded extremely well with both modalities. This entity should be considered when diminished activity is seen in the frontal area.

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