Hyperostosis frontalis interna is a disorder marked by benign overgrowth of the inner table of the frontal bone. The pathological process may be bilateral and symmetrical with sessile or nodular thickened areas. The external lamina and the diploe of the bone are usually not affected, but the abnormality may extend to the parietal bones.
Hyperostosis frontalis interna usually affects postmenopausal women , but up to 12% of the whole female population may have this condition, including those of child-bearing age since the disease has also been associated with pregnancy. Men are seldom affected. The real prevalence of the disease may be underestimated because it is often asymptomatic and the diagnosis can only be established following a skull imaging study.
Rarely, the disease is associated with some of the numerous possible manifestations. The clinical examination may reveal certain physical traits: virilization, obesity, epilepsy, facial palsy, or headaches . Patients may be diabetic, therefore also exhibiting the characteristics of this condition: weight loss, polyuria, polydipsia, and polyphagia. Additional features for this condition include fatigability, vertigo, hypertrichosis, and acromegaly . Mental disturbances such as depression, paranoia, or aggressiveness are believed to be caused by intracranial volume decrease . Brain tissue compression, explaining epileptic seizures and dural irritation, may also be encountered . The disease may cause hormonal impairment, such as hyperprolactinemia or ovarian dysfunction.
In the past, hyperostosis frontalis interna was regarded as part of several syndromes, namely Morgagni (hypertrichosis, obesity, virilization, and headaches), Stewart-Morel (neuropsychiatric abnormalities and obesity), and Troell-Junet (diabetes mellitus, acromegaly, and toxic goiter) syndromes .
Entire Body System
Also called Morel's syndrome. hyperostosis frontalis interna Morgagni-Stewart-Morell syndrome A form of osteopetrosis more common in middle-aged , associated with obesity, hirsutism, fatigue, hemiplegia and hemiparesis; HFI affects cranial bones, structurally [medical-dictionary.thefreedictionary.com]
There may be associated menstrual disorders, virilism, hirsutism, mental disorders, fatigue, somnolence, visual disorders, vertigo, tinnitus, obesity, polyphagia, polydipsia, polyuria, loss of sense of smell, decrease in glucose tolerance, convulsions [whonamedit.com]
Weakness, obesity, and fatigue are also common, as are vertigo and facial palsy. Due to these symptoms being somewhat generalized, a differential diagnosis must be made. [wisegeekhealth.com]
- Weight Gain
This may lead to symptoms such as chronic headaches, weight gain, and thyroid irregularities, and is suspected to have multiple causes, including lifestyle, fertility habits, nutrition, and environment. To track the growing prevalence of HFI, Prof. [sciencedaily.com]
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). [ncbi.nlm.nih.gov]
Douglas Hunt Stewart in 1928 again emphasised the association with obesity; Ferdinand Morel in 1930 emphasised the accompanying menstrual disturbance, amenorrhea, and impotence; and S. [whonamedit.com]
[…] compromising the hypophysis–causing dysmenorrhea, virilism, hirsutism, diabetes insipidus, and glucose intolerance, and cranial nerve foramina–causing vertigo, tinnitus, anosmia, and visual defects [medical-dictionary.thefreedictionary.com]
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 [ncbi.nlm.nih.gov]
Moore The main features of this hereditary syndrome is a trias consisting of 1) hyperostosis frontalis interna, 2) adipositas, and 3) virilism and hirsutism. [whonamedit.com]
Since hyperprolactinemia was found in many of these cases, the authors suggested that this and other features of the syndrome such as hirsutism, diabetes, and menstrual troubles may be related to hyperprolactinemia. REFERENCES Gegick, C. [omim.org]
[…] frontalis interna Morgagni-Stewart-Morell syndrome A form of osteopetrosis more common in middle-aged , associated with obesity, hirsutism, fatigue, hemiplegia and hemiparesis; HFI affects cranial bones, structurally compromising the hypophysis–causing dysmenorrhea [medical-dictionary.thefreedictionary.com]
We present a novel case of migraine headache in a gentleman with Klinefelter's syndrome and HFI, along with a discussion of possible pathophysiologic mechanisms underlying both the headache and the HFI. [ncbi.nlm.nih.gov]
These factors are obesity, diabetes insipidus, and migraine headaches. [epainassist.com]
We report a case of non-syndromic HFI in an elderly female who presented with headache. DOI: Refbacks There are currently no refbacks. [sciedupress.com]
Grunt and assistants in RDG clinic in Hospital Ružinov for made radiographs. This project was supported with grant VEGA 1/0133/10 Application of geometric morphometrics to assess the exogenous traits in Early Medieval populations from Slovakia. [antropoweb.cz]
A conventional radiological evaluation may offer some information about the extent and localization of the abnormality. The bone deposition process is often confined to the frontal bone but sometimes extends to parietal bones. However, frontal bone areas related to venous channels and superior sagittal sinus are spared . Suture lines represent clear boundaries for the development of this pathological process. Lesions may be flat or nodular, isolated or confluent . The external contour of the skull remains normal, therefore the clinical examination could exclude other entities, such as Paget's disease or leontiasis ossea.
Magnetic resonance imaging (MRI) and computed tomography (CT) offer more detailed clues regarding both the bone proliferation and the status of the brain tissue and dura mater. The lesion border characteristics, along with its shape and location, are elements on which four disease types (A to D) have been defined .
If doubt persists, a histological evaluation is able to shed light on the case by describing thick lamellar bone deposition, predominantly on the inner table of the frontal bone . The pattern of bone deposition differs markedly when compared with patterns seen in Paget's disease, endosteal osteoma, or osteosarcoma. The bone changes are normally less than 1 cm thick in hyperostosis frontalis interna.
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