Morgagni–Stewart–Morel syndrome

A rare disorder characterized by thickening of the frontal bone of the skull

Morgagni–Stewart–Morel syndrome (MSM) is a rare endocrine disorder characterized by the thickening of the inner table of the frontal bone of the skull, a condition known as hyperostosis frontalis interna. This syndrome is often associated with a variety of neuropsychiatric and endocrine symptoms.

History

The syndrome is named after three physicians: Giovanni Battista Morgagni, a prominent Italian anatomist; Roy Mackenzie Stewart, a Scottish physician; and Ferdinand Morel, a Swiss psychiatrist. Morgagni first described the condition in the 18th century, while Stewart and Morel further characterized the syndrome in the 20th century.

Clinical Features

The primary feature of Morgagni–Stewart–Morel syndrome is the thickening of the frontal bone, which can be detected through radiographic imaging. Patients may present with a variety of symptoms, including:

• Headaches
• Obesity
• Hirsutism (excessive hair growth)
• Menstrual irregularities
• Neuropsychiatric symptoms such as depression, anxiety, and cognitive impairment

Pathophysiology

The exact cause of the syndrome is not well understood, but it is believed to involve hormonal imbalances, particularly involving estrogen and other sex hormones. The thickening of the frontal bone is thought to be due to abnormal bone metabolism.

Diagnosis

Diagnosis of Morgagni–Stewart–Morel syndrome is primarily based on clinical evaluation and imaging studies. X-rays or CT scans of the skull can reveal the characteristic thickening of the frontal bone. Additional tests may be conducted to assess hormonal levels and rule out other conditions.

Genetics

X-linked recessive inheritance pattern

The genetic basis of Morgagni–Stewart–Morel syndrome is not fully understood, but there is some evidence to suggest a possible X-linked recessive inheritance pattern. However, the syndrome can also occur sporadically without a clear genetic link.

Treatment

There is no specific treatment for Morgagni–Stewart–Morel syndrome. Management focuses on alleviating symptoms and may include:

• Analgesics for headache relief
• Hormonal therapy for endocrine symptoms
• Psychotherapy and psychiatric medications for neuropsychiatric symptoms

Prognosis

The prognosis for individuals with Morgagni–Stewart–Morel syndrome varies depending on the severity of symptoms and the effectiveness of symptom management. While the condition is chronic, many patients can lead relatively normal lives with appropriate treatment.

Related pages

• Endocrine disorder
• Hyperostosis frontalis interna
• Neuropsychiatric disorder