Morgagni-Stewart-Morel syndrome

What causes Morgagni-Stewart-Morel syndrome?

The cause of Morgagni-Stewart-Morel syndrome and hyperostosis frontalis interna is unknown, although some have suggested that a hormonal imbalance due to genetic and environmental factors may be the cause of many of the symptoms of the syndrome.

Last updated on 05-01-20

How might Morgagni-Stewart-Morel syndrome be diagnosed?

The diagnosis of Morgagni-Stewart-Morel syndrome is based upon a radiological finding of hyperostosis frontalis interna, as well as a combination of clinical features including obesity, virulism (a female disorder in which there is development of secondary male sexual characteristics like growth of facial and body hair), possible mental disturbance, and other findings.

Last updated on 05-01-20

Is Morgagni-Stewart-Morel syndrome associated with other conditions?

Morgagni-Stewart-Morel syndrome is commonly associated with diabetes mellitus, diabetes insipidus, or hyperparathyroidism.

Last updated on 05-01-20

Who is most commonly diagnosed with Morgagni-Stewart-Morel syndrome?

Morgagni-Stewart-Morelsyndrome is most commonly diagnosed in females. Hyperostosis frontalis interna, a major symptom of Morgagni-Stewart-Morel syndrome, is more common in elderly patients and postmenopausal women, especially when associated with obesity.

Last updated on 05-01-20

Is hyperostosis frontalis interna always associated with Morgagni-Stewart- Morel syndrome?

While people with Morgagni-Stewart-Morel syndrome nearly always have hyperostosis frontalis interna, hyperostosis frontalis interna is usually an incidental finding on x-ray films, computed tomography, or magnetic resonance imaging of the head and by itself is not indicative of any disease.

Last updated on 05-01-20

What is Morgagni-Stewart-Morel syndrome?

Morgagni-Stewart-Morel (MSM) syndrome is a disorder characterized by thickening of the frontal bone of the skull (hyperostosis frontalis interna), as well as obesity and excessive hair growth (hypertrichosis). Other signs and symptoms may include seizures, headaches, diabetes insipidus, and sex gland disturbances. The cause of Morgagni-Stewart-Morel syndrome is not fully understood. Some instances of dominant inheritance have been reported, but whether it is autosomal dominant or X-linked dominant is not known. Treatment may include medication for headaches and seizures and surgery to remove the excessive bone of the skull.

Last updated on 05-01-20

Is Morgagni-Stewart-Morel syndrome genetic?

The role genes play in the development of Morgagni-Stewart-Morel syndrome is still unclear. There have been a few case reports describing multiple members of the same family with Morgagni-Steward-Morel syndrome and in these families the syndrome appears to be inherited in a autosomal dominant or X-linked dominant fashion.

Last updated on 05-01-20

How might Morgagni-Stewart-Morel syndrome be treated?

Currently, there is no cure or specific recommendations for the treatment of this syndrome. Treatment depends on the symptoms present, and may include medication and diet and lifestyle modification for weight control. Diabetes and hypertension are treated with standard medication.

Seizures and headaches that may be associated with hyperostosis frontalis interna (HFI) are typically treated with standard medications.

In one journal article, the authors discuss a person with Morgagni-Stewart- Morel syndrome who reportedly had violent headaches. Surgery was performed to remove the hypertrophic frontal bone on the patient. He then had dura and bone reconstruction. The authors reported that the headaches stopped immediately after the operation.

Another article described a patient with HFI and intracranial hypertension (increased pressure inside the skull and brain). The authors reported good results with a craniotomy (a surgical operation in which a bone flap is temporarily removed from the skull to access the brain) performed to decrease the intracranial pressure.

Last updated on 05-01-20

Name: National Headache Foundation NHF 820 N Orleans, Suite 201
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The RareGuru disease database is regularly updated using data generously provided by GARD, the United States Genetic and Rare Disease Information Center.

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