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].

• Epilepsy

  She also denied any traumatic brain injury or family history of epilepsy. Physical examination revealed an obese woman, in no apparent distress with hypo-phonetic heart sounds and lungs clear to auscultation bilaterally. [casereports.bmj.com]

  HFI may be accompanied by headache and some neuropsychiatric diseases such as epilepsy and dementia. [ncbi.nlm.nih.gov]

  Compression by calvarial thickening may lead to cerebral atrophy and may present with cognitive impairment, neuropsychiatric symptoms, headaches and epilepsy 1,3. [radiopaedia.org]

  (For more information on these disorders, choose “Epilepsy” and/or “Diabetes” as your search terms in the Rare Disease Database). < Previous section Next section > < Previous section Next section > Causes Hyperostosis Frontalis Interna has been found [rarediseases.org]

• Asymptomatic

  It is found predominantly in women after menopause and is usually asymptomatic. Mostly frequently it is found as an incidental finding discovered during an X-ray or CT scan of the skull. [en.wikipedia.org]

  It is an incidental, asymptomatic and benign lesion seen mostly in postmenopausal females. Aims: To determine the epidemiology of hyperostosis frontalis interna in Uyo using computed tomograms of the skull. [ajol.info]

  It's most typically found in older females and is largely asymptomatic, usually being discovered by accident when for some other reason the skull is radiographed. [boneclones.com]

  As hyperostosis frontalis interna is usually an incidental finding of no clinical significance patients are generally asymptomatic. [radiopaedia.org]

  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. [symptoma.com]

• Fatigue

  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]

• Weakness

  The group of symptoms and signs found, namely, headache, visual disturbance, dizziness, defects of memory, convulsions, weakness and easy fatigability, with various types of mental changes, are extremely general and may occur in numerous neurologic states [jamanetwork.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]

  She had weakness in all four limbs, more on the right side and she was constantly tired. In 2002 May she had two episodes of hematuria, and later ultrasound examination revealed renal stones. [neurologyindia.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]

• Hypotension

  Definition / general Also called thickened calvarium May be due to intracranial hypotension associated with chronic shunting, as skull lacks the outward pressure needed to expand ( Hum Pathol 1994;25:545 ) Gross description Circumferentially thickened [pathologyoutlines.com]

• Hirsutism

  […] 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 [thieme-connect.com]

  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]

• Hypertrichosis

  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. [symptoma.com]

  HYPEROSTOSIS FRONTALIS INTERNA Alternative titles; symbols MORGAGNI-STEWART-MOREL SYNDROME ▼ TEXT In addition to thickening of the inner table of the frontal bone, obesity and hypertrichosis may be present. This condition affects mainly females. [omim.org]

  Thickening of the inner table of the frontal bone, which may be associated with hypertrichosis and obesity. It most commonly affects women near menopause. [profiles.umassmed.edu]

  Description Thickening of the inner table of the frontal bone, which may be associated with hypertrichosis and obesity. It most commonly affects women near menopause. [cdek.liu.edu]

• Bone Disorder

  […] of androgens in pathogenesis of this metabolic bone disorder. [thieme-connect.com]

  Paget’s disease of the bone: A chronic bone disorder characterized by excessive bone remodeling with replacement of normal bone architecture with a coarsened trabecular pattern. [radiologykey.com]

• Amenorrhea

  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]

• Headache

  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]

  It has also been described in association with chronic headaches and rarely with seizures.2 This incidental image finding should not alarm neurologists yet should raise awareness about a possible susceptibility for headaches and seizures as part of the [casereports.bmj.com]

  Res Clin Stud Headache 3:191–197, 1:STN:280:DyaE3s7ltVKhug%3D%3D, 5527204 CAS PubMed Google Scholar Headache Classification Subcommittee of the International Headache Society. (2004) The International Classification of Headache Disorders: 2nd edition. [thejournalofheadacheandpain.biomedcentral.com]

  These factors are obesity, diabetes insipidus, and migraine headaches. [epainassist.com]

  Results HFI presents in predominantly postmenopausal women, normally ranging in thickness from 0.5–1cm, and has been associated with diabetes, obesity, and chronic headaches. [pulsus.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]

  Moore reported it as a benign process that causes soft tissue compression with dural irritation and pressure atrophy of brain. [4] Hasegawa et al and Latka et al described one case each with sclerotic skull bone causing compression on left frontal lobe [neurologyindia.com]

  […] restlessness, low mood decreased appetite and lack of interest in daily routine activities, in addition to these symptoms patient also complained of intermittent forgetfulness, recurrent history of Intractable headaches, sleep disturbance, worrying thoughts, irritability [medicalandresearch.com]

• Asthenia

  Frontal headache, asthenia, forgetfulness, vertigo, nervousness, obesity and poor vision were rather characteristic of this group. There was a high degree of morbidity with frequent remission and exacerbation of symptoms. [amjmed.com]

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.

and 60 who received different treatments or none at all. [ncbi.nlm.nih.gov]

Diagnosis considers a combination of clinical features including obesity,[4] virilism, and mental disturbances.[5] Treatment[edit] Treatment is based upon the symptoms, and generally includes medication, diet and lifestyle modification for weight control [en.wikipedia.org]

Other than this, treatment is purely supportive for Hyperostosis Frontalis Interna. This article does not provide medical advice. See disclaimer [epainassist.com]

With input from doctors, researchers, and the US Food & Drug Administration, NORD has created IAMRARE to facilitate patient-powered natural history studies to shape rare disease research and treatments. [rarediseases.org]

Some authors have observed that prognosis did not seem to be affected by HFI, and the clinical outcome was in accordance with the psychiatric diagnosis. [hindawi.com]

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]

Little is known about the etiology and clinical presentation of HFI. [scholars.northwestern.edu]

The etiology is unknown. The condition is generally of no clinical significance and an incidental finding. It is typically bilateral and symmetrical and may extend to involve the parietal bones. [radiopaedia.org]

Aims: To determine the epidemiology of hyperostosis frontalis interna in Uyo using computed tomograms of the skull. [ajol.info]

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]

[…] light on the past through the analysis of human and non-human skeletal materials, mummified remains and preserved tissue Integrates scientific advances in multiple fields that contribute to the understanding of ancient and historic diseases, such as epidemiology [books.google.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]

Arch Neurol 61:1366–1368, 10.1001/archneur.61.9.1366, 15364681 Article PubMed Google Scholar Ahkenazi A, Silberstein SD (2006) Hormone-related headache: pathophysiology and treatment. [thejournalofheadacheandpain.biomedcentral.com]

With more than1,500 images, the authors have assembled an accessible resource and review that presents over 170 core topics using the imaging findings as a platform to discuss pathophysiology, clinical presentation, and disease management. [books.google.com]

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]

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: https://doi.org/10.5430/crim.v4n1p57 Refbacks There are currently no refbacks. [sciedupress.com]

Migraine headaches can be treated with preventive and abortive medications. The frequent bouts of seizures can be controlled with standard anticonvulsant medications. [epainassist.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. [neurology.org]

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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.