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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.

Aged

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.

Aged

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

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

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.

Aged

Androgens, bone mineral content and hyperostosis frontalis interna in pre-menopausal women.

Greater than normal bone mineral content, bone width of the radius measured by photon absorptiometry and bone mineral content to bone width ratio found in women with hyperostosis frontalis interna pointed on a generalized alteration of the skeletal system. An increase in serum dehydroepiandosterone, its sulphate and testosterone levels and a significant correlation between serum free dehydroepiandosterone and bone mineral content in subjects without any sign of hirsutism or obesity suggested an involvement of androgens in pathogenesis of this metabolic bone disorder.

17-alpha-Hydroxyprogesterone

[A case of adult-onset spike-wave stupor associated with hypoparathyroidism and hyperostosis frontalis interna (HFI)].

A 34-year-old women without history of seizures was admitted to our hospital because of the diminished responsiveness and the repeated blinking for two days. Her past history showed neither surgery nor inflammation of the thyroid gland. General physical examination was normal. On neurological examination, the patient was blinking frequently and staring without any responsiveness. However, she occasionally became able to respond correctly to verbal orders, such as "open your eyes", "open your mouth", "stand up" and so on, which occurred abruptly just like the switch-on. She was otherwise normal neurologically. The laboratory data showed hypocalcemia, hyperphosphatemia, decreased level of parathyroid hormone and normal renal function, indicating the presence of idiopathic hypoparathyroidism. EEG showed the continuous generalized 2-4 Hz spike and wave complexes with the maximum intensity on frontal lobes. Skull roentgenograms and MRI CT of the head disclosed hyperostosis frontalis interna (HFI) and the compression of superior medial frontal lobes by the HIF. SPECT also showed the diminished circulation and hypometabolism in the superior frontal lobes. Based on clinical and EEG findings, the diagnosis of spike-wave stupor was made. She was successfully treated with valproic acid and ethosuximide. Spike and wave complexes on EEG completely disappeared after administration of alfacalcidole. It is assumed that both hypoparathyroidism and HFI were deeply involved in the development of spike-wave stupor in this adult case.

Adult