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Hyperostosis Frontalis Interna

Morgagni-Stewart-Morel Syndrome

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.


Presentation

Hyperostosis frontalis interna usually affects postmenopausal women [1], 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 [2]. 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 [3]. Mental disturbances such as depression, paranoia, or aggressiveness are believed to be caused by intracranial volume decrease [4]. Brain tissue compression, explaining epileptic seizures and dural irritation, may also be encountered [5]. 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 [6].

Goiter
  • […] 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[symptoma.com]
  • Formerly HFI was associated with Morgagni’s syndrome (HFI, obesity, virilism), Stewart-Morel syndrome (HFI, obesity, neuropsychiatric disorders) and Troell-Junet syndrome (HFI, acromegaly, goiter, diabetes) but is now considered to be an entity entirely[pulsus.com]
Weight Loss
  • 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.[symptoma.com]
  • Strict diet for weight loss and controlling obesity is advised. Migraine headaches can be treated with preventive and abortive medications. The frequent bouts of seizures can be controlled with standard anticonvulsant medications.[epainassist.com]
Overeating
  • 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.[ncbi.nlm.nih.gov]
  • 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]
  • In cadavers, 87.5% of severe hyperostosis frontalis interna cases were found in females over 65 years-old. Interestingly, in two cadavers we found hyperostotic lesions spreading onto adjacent tissues such as the dura and falx cerebri.[ncbi.nlm.nih.gov]
  • DISCUSSION: When recorded consistently, HFI was positively correlated with age and longevity but had also increased among old age females over time.[ncbi.nlm.nih.gov]
  • Although hyperostosis frontalis interna (HFI) has been documented in the medical literature for over 300 years, its etiology remains undetermined. It is generally assumed to be associated with hormonal disturbances of the gonads.[ncbi.nlm.nih.gov]
Polydipsia
  • 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.[symptoma.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]
Frontal Headache
  • They can include a frontal headache, mental impairment, and depression. 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.[wisegeek.com]
Irritability
  • 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.[symptoma.com]
Polyuria
  • 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.[symptoma.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]

Workup

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 [2]. Suture lines represent clear boundaries for the development of this pathological process. Lesions may be flat or nodular, isolated or confluent [2]. 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 [2].

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 [7]. 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.

Treatment

  • , and 60 who received different treatments or none at all.[ncbi.nlm.nih.gov]
  • Men affected by this pathology suffer from diseases, syndromes or treatments causing androgen deficiency. In the case of Farinelli, castration was probably responsible for the onset and development of this lesion. 2011 The Authors.[ncbi.nlm.nih.gov]
  • Other than this, treatment is purely supportive for Hyperostosis Frontalis Interna. This article does not provide medical advice. See disclaimer[epainassist.com]
  • Other treatment is symptomatic and supportive. Investigational Therapies Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov.[rarediseases.org]
  • As the thickening of the bone is benign and not harmful to the patient, there is no treatment for hyperostosis frontalis interna. Rather, the symptoms are considered to be a separate issue and treated accordingly.[wisegeekhealth.com]

Etiology

  • The etiology of HFI is uncertain: it may be an unknown genetic predisposition, a common environmental exposure, or special metabolic diseases.[ncbi.nlm.nih.gov]
  • Although the disease is fairly common in modern populations, its etiology is poorly understood. Hyperostosis frontalis interna has been identified in antiquity, primarily in the Old World, but with a much lower frequency than in modern groups.[ncbi.nlm.nih.gov]
  • 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.[ncbi.nlm.nih.gov]
  • Giant cell arteritis (GCA), also called temporal arteritis, is a systemic inflammatory vasculitis of unknown etiology that affects medium- and large-sized arteries.[ncbi.nlm.nih.gov]
  • Although there are numerous publications on the subject, at the present time its etiology remains obscure. Several associations of symptoms, whose incidence varies according to the population studied, have been described.[ncbi.nlm.nih.gov]

Epidemiology

  • The epidemiology of HFI shows that it is relatively common in postmenopausal women but very rare in men. Men affected by this pathology suffer from diseases, syndromes or treatments causing androgen deficiency.[ncbi.nlm.nih.gov]
Sex distribution
Age distribution

Pathophysiology

  • 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]
  • The aim of this study was to reveal a possible pathophysiology of brain degenerative diseases by studying intracranial bone lesions that act to reduce intracranial volume (ICV), such as hyperostosis frontalis interna (HFI).[ncbi.nlm.nih.gov]
  • Since HFI identification, 300 years ago, studies have endeavored to reveal its prevalence, pathophysiology, and etiology (Moore, 1955 ; Perou, 1964 ; Antón, 1997 ; Hershkovitz et al., 1999 ).[onlinelibrary.wiley.com]

Prevention

  • Timely identification of HFI can prevent unnecessary diagnostic tests. 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]
  • The headaches proved to be refractory to multiple preventative medications and nerve blocks. She also had progressive difficulty with concentration, visual and verbal memory as well as organizational skills.[n.neurology.org]
  • Migraine headaches can be treated with preventive and abortive medications. The frequent bouts of seizures can be controlled with standard anticonvulsant medications.[epainassist.com]
  • Published on behalf of the Royal College of Psychiatrists, the journal’s overriding concern is to improve the prevention, investigation, diagnosis, treatment, and care of mental illness, as well as the promotion of mental health globally.[cambridge.org]

References

Article

  1. Raikos A, Paraskevas GK, Yusuf F. Etiopathogenesis of hyperostosis frontalis interna: a mystery still. Ann Anat. 2011;193(5):453–458.
  2. Hershkovitz I, Greenwald C, Rothschild BM, et al., Hyperostosis frontalis interna: an anthropological perspective. Am J Phys Anthropol. 1999;109:303-325.
  3. Crispin JC, Alcocer-Varela J. Rheumatologic manifestations of diabetes mellitus. Am J Med. 2003;114:753–757.
  4. Devriendt W, Piercecchi-Marti MD, Adalian P, et al . Hyperostosis frontalis interna: forensic issues. J Forensic Sci. 2005;50(1):143–146.
  5. Chaljub G, Johnson RF III, Johnson RF Jr, et al. Unusually exuberant hyperostosis frontalis interna: MRI. Neuroradiol 1999;41:44–45.
  6. Nikolić S, Djonić D, Zivković V, et al. Rate of occurrence, gross appearance, and age relation of hyperostosis frontalis interna in females: a prospective autopsy study. Am J Forensic Med Pathol. 2010;31(3):205–207.
  7. Chen OI, Murthy AS. Benign hyperostotic mass of the frontal bone. J Craniofac Surg. 2012;23(1):e27–e30.

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Last updated: 2018-06-21 18:59