Morgagni-Stewart-Morel syndrome

=Morgagni-Stewart-Morel Syndrome = Morgagni-Stewart-Morel Syndrome (MSM) is a rare disorder characterized by a triad of symptoms: hyperostosis frontalis interna, obesity, and hirsutism. This condition primarily affects women, particularly in the postmenopausal age group.

History

The syndrome was first described by Giovanni Battista Morgagni in the 18th century, and later expanded upon by Roy Mackenzie Stewart and Ferdinand Morel in the 20th century. The condition is named after these three physicians who contributed to its identification and understanding.

Clinical Features

Hyperostosis Frontalis Interna

Hyperostosis frontalis interna is the thickening of the inner table of the frontal bone of the skull. This feature is often detected incidentally on radiological examinations, as it usually does not cause symptoms. However, in some cases, it may be associated with headaches or other neurological symptoms.

Obesity

Patients with Morgagni-Stewart-Morel Syndrome often present with obesity. The exact mechanism linking the syndrome to obesity is not fully understood, but it is a consistent finding in affected individuals.

Hirsutism

Hirsutism, or excessive hair growth in women in areas where hair is normally minimal or absent, is another common feature of MSM. This symptom is thought to be related to hormonal imbalances that may be present in the syndrome.

Diagnosis

The diagnosis of Morgagni-Stewart-Morel Syndrome is primarily clinical, based on the presence of the characteristic triad of symptoms. Imaging studies, such as X-rays or CT scans, can confirm the presence of hyperostosis frontalis interna.

Pathophysiology

The exact pathophysiology of Morgagni-Stewart-Morel Syndrome is not well understood. It is believed to involve hormonal and metabolic factors, given the association with obesity and hirsutism. Some studies suggest a possible genetic component, but no specific genetic mutations have been definitively linked to the syndrome.

Treatment

There is no specific treatment for Morgagni-Stewart-Morel Syndrome. Management is symptomatic and supportive, focusing on addressing the individual symptoms such as obesity and hirsutism. Weight management through diet and exercise, as well as hormonal therapies, may be beneficial.

Prognosis

The prognosis for individuals with Morgagni-Stewart-Morel Syndrome is generally good, as the condition is not life-threatening. However, the associated symptoms can impact quality of life, and management of these symptoms is important.

See Also

• Hyperostosis frontalis interna
• Hirsutism
• Obesity
• Morgagni, G.B. (1761). "De Sedibus et Causis Morborum per Anatomen Indagatis."
• Stewart, R.M. (1928). "Hyperostosis Frontalis Interna."
• Morel, F. (1930). "Syndrome de Morgagni-Stewart-Morel."

NIH genetic and rare disease info

• NIH info on Morgagni-Stewart-Morel syndrome

Morgagni-Stewart-Morel syndrome is a rare disease.