Morgagni–Stewart–Morel syndrome: A new case

Morgagni–Stewart–Morel syndrome is an association of clinical features with radiological findings (HFI). The common clinical features are headache (migrainous), psychotic symptoms, metabolic and endocrine disorders (obesity, diabetes mellitus, and thyroid disease), hirsutism, osteoporosis, and menstrual disorders, but these associations are mostly based on case reports, and no clear consensus exists on the definition of the syndrome.

A 38-year-old woman, married without children, admitted in February 2019 to the Endocrinology Department for an inaugural diabetic ketosis. During the interrogation the patient complained that she had an eight-year history of severe chronic headaches never explored and she was being followed up by a psychiatrist for depression. She also reported a medical history of obesity, infertility for several years. On examination she was obese, depressed, and her body mass index (BMI) was 36 kg/m². Axial cranial CT scan showed bilateral thickening of the inner table of the frontal bone (Figure 1A) and (Figure 1B). Lab values are given in Table 1.

These endocrine dysfunctions and neuropsychiatric symptoms are interpreted as components of MSM syndrome. For the severe headache, the surgical indication was discussed with the neurosurgeons but the patient used immediately surgery and she was treated by analgesics with improvement under tramadol.

Morgagni–Stewart–Morel syndrome was first described in 1719 by Giovanni Battista Morgagni in an obese female patient during autopsy who had hirsutism and HFI defined as thickening of inner table of a skull [1].

The modern concept of its clinical significance was initiated in 1928 after Stewart reported three cases of the syndrome diagnosed at autopsy. The first living case was reported by Morel in 1930 [2],[3].

Hyperostosis frontalis interna is a morphological pattern of the frontal bone that is usually an incidental finding in X-ray, cranial computed tomography (CCT), or magnetic resonance imaging (MRI) studies [4].

Morgagni–Stewart–Morel syndrome is defined as the association of HFI with metabolic, endocrine, and neuropsychiatric disorders. But these associations are mostly based on case reports, and no clear consensus exists on the definition of the syndrome [4].

The etiology and pathogenesis behind skull thickening and MSM syndrome are till now not clear; the most interesting theories relate to extended exposure to estrogen, obesity and elevated leptin level, and genetics [5],[6].

The common clinical features are headache, hirsutism, menstrual disorders, galactorrhoea, obesity, diabetes mellitus, depression, irritability, fatigability, transitory hemiplegias, hearing impairment, cranial nerve palsies, muscle weakness, and seizures [7],[8],[9].

The treatment is generally symptomatic but there is a case of MSM syndrome described in the literature with violent headaches predominant in the clinical course treated surgically and the pain ceased immediately after the operation. The authors discuss surgical treatment especially in cases with high intensity headaches [10].

Morgagni–Stewart–Morel syndrome is also called as metabolic craniopathy due to its wide range of symptoms and endocrinal dysfunctions [11].

The known symptoms of MSM syndrome are mostly based on case reports, and they tend to be nonspecific. Patient with MSM syndrome may present varied symptoms associated to thickening of the inner table of the frontal bone, thus lead to difficulty in diagnosis.