Morgagni – Stuart – Morel syndrome (metabolic craniopathy) is a condition associated with a wide range of endocrine problems, including diabetes mellitus and diabetes insipidus and hyperparathyroidism [1] . Other signs and symptoms include headache , dizziness , excessive male-type hair growth, menstruation problems, galactorrhea , obesity , depression, and epileptic seizures [1] [2] . Thickening of the inner surface of the frontal part of the skull is, as a rule, the initial stage of the disease, known as hyperostosis frontalis interna [1] [3] .
| Morgagni - Stuart - Morel Syndrome | |
|---|---|
 Computed tomography scan of the skull of a patient with frontal bone hyperostosis | |
| ICD-10 | M 85.2 |
| ICD-9 | 733.3 |
| Omim | 144800 |
| Diseasesdb | 33260 |
Content
- 1 Discovery History
- 2 Etiology and pathogenesis
- 3 Diagnostics
- 4 Treatment
- 5 notes
Discovery History
The syndrome was first described in 1765 by the Italian pathologist Giovanni Battista Morgagni [3] and later, in 1928, by the English neurologist Douglas Stewart. [4] Swiss physician Ferdinand Morel (1888-1957) in 1930 added to the clinical picture disorders of the menstrual cycle and impotence [5] . The term “Morgagni – Stuart – Morel syndrome” was introduced in 1937 by Folke Henschen [6] .
Etiology and pathogenesis
The etiology of the disease has not been fully studied, but it is associated with hypertrophy of the adrenal cortex due to hypersecretion of adrenocorticotropic hormone , the effect of which determines the secondary symptoms. Similar symptoms were observed in women with increased prolactin against the background of frontal bone hyperostosis in 43% of cases [7] . A number of studies have shown autosomal dominant inheritance of the disease [8] [9] . The disease is characteristic mainly for women.
Diagnostics
Diagnosis is based on complaints and clinical presentation; also used histological methods of research . On the roentgenogram, a thickening of the frontal bone is visualized. Morgagni-Stewart-Morel syndrome is differentiated primarily from Itsenko-Cushing's disease and adiposogenital dystrophy .
Treatment
The treatment is symptomatic . In rare cases, the overgrown inner part of the frontal bone can be surgically removed.
Notes
- ↑ 1 2 3 Nallegowda M., Singh U., Khanna M., Yadav SL, Choudhary AR, Thakar A. Morgagni Stewart Morel syndrome - additional features (English) // Neurology India : journal. - 2005 .-- March ( vol. 53 , no. 1 ). - P. 117-119 . - DOI : 10.4103 / 0028-3886.15078 . - PMID 15805672 .
- ↑ Psychiatria. Podręcznik dla studentów medycyny / Bilikiewicz A. - 3. - Warszawa: Lekarskie PZWL, 2006. - P. 150. - ISBN 83-200-3388-8 .
- ↑ 1 2 She R., Szakacs J. Hyperostosis frontalis interna: case report and review of literature (English) // Annals of Clinical and Laboratory Science : journal. - 2004. - Vol. 34 , no. 2 . - P. 206-208 . - PMID 15228235 .
- ↑ Stewart RM. Localized cranial hyperostosis in insane (English) // Journal of Neurology and Psychopathology. - 1928. - Iss. 8 . - No. 321 .
- ↑ Morel F. L'hyperostose frontale interne. Syndrome de l'hyperostose frontale interne avec adipose et troubles cérébraux .. - Paris, 1930.
- ↑ Henschen F. Morgagni's syndrome. - Jena, 1937.
- ↑ Hyperostosis frontalis, galactorrhoea / hyperprolactinaemia, and Morgagni-Stewart-Morel syndrome // The Lancet . - Elsevier , 1983-02-26. - T. 1 , no. 8322 . - S. 474 . - PMID 6131191 .
- ↑ Koller MF, Papassotiropoulos A., Henke K., Behrends B., Noda S., Kratzer A., Hock C., Hofmann M. Evidence of a genetic basis of Morgagni-Stewart-Morel syndrome. A case report of identical twins (English) // Neuro-degenerative diseases: journal. - 2005. - Vol. 2 . - P. 56-60 . - DOI : 10.1159 / 000089284 . - PMID 16909048 .
- ↑ Knies PT, Le Fever HE Metabolic craniopathy: hyperostosis frontalis interna. (English) // Annals of Internal Medicine : journal. - 1941. - No. 14 . - P. 1858-1892.