Morgagni-Steawart-Morel syndrome: Case report

Year 2019, , 434 - 436, 30.09.2019

Emrah Doğan Marwa Mouline

https://doi.org/10.18663/tjcl.453912

Abstract

Hyperostosis frontalis interna (HOFI) is characterized
by the benign growth of the inner plate of the frontal bone. It is most common
in older women. Etiology is unknown. Morgagni-Stewart-Morel syndrome(MSM) is
characterized by HOFI, obesity, hirsutism and mental illnesses but these
associations are mostly based only on case reports. MSM syndrome is misunderstood
and less reported pathology. The clinical presentation is not well defined andits
finding is usually coincidental. The patient may present variable
symptomatology. The idea that we should keep in mind is that HOFI representthe
main pattern of MSM. Thus, it is highly importantto specify the presence and
degree of HOFI in radiological reports.

Keywords

Morgagni-Stewart-Morel Syndrome, Hiperostosis frontalis interna

Morgagni-Steawart-Morel sendromu: Olgu sunumu

Abstract

Hiperostozis frontalis interna(HOFI) frontal
kemiğin iç tabulasının iyi huylu büyümesi ile karakterizedir. En sık yaşlı
kadınlarda görülür. Etiyoloji bilinmemektedir. Morgagni-Stewart-Morel sendromu (MSM);
HOFI, obezite, hirsutizm ve zihinsel rahatsızlıklar ile karakterizedir ancak bu
ilişkiler çoğunlukla vaka raporlarına dayanır. MSM sendromu daha az anlaşılan ve
bildirilen sendromdur. Hasta çeşitli semptomlar gösterebilir. HOFI’nin MSM’nin bir
paterni olduğu akılda tutulmalıdır. Radyolojik raporlamada bu nedenle HOFI
varlığını ve derecesini belirtmek önem teşkil eder. 

Keywords

Morgagni-Stewart-Morel Sendromu, Hiperostozis frontalis interna

References

• 1.Attanasio F,Granziera S, Giantin V, Manzato E. Full Penetrance of Morgagni-Stewart-Morel Syndrome in a 75-Year-Old Woman: Case Report and Review of the Literature. J Clin Endocrinol Metab 2013; 98: 453-57.
• 2.Nallegowda M, Singh U, Khanna M, Yadav SL, Choudhary AR, ThakarA .Morgagni Stewart Morel syndrome-additional features. Neurol India 2005; 53: 117-19.
• 3. Smith S, Hemphill RE. Hyperostosis frontalis interna. J. Neurol. Neurosurg Psychiatr 1956; 19: 42-45. 4.Klein D, Rosatti P, Mach RS, Ferrier PE, Rauch S: Morgagni-Morel syndrome: an independent clinical and genetic entity, demonstrated in a family study covering 4 generations. Schweiz Arch Neurol Neurochir Psychiatr 1973;112: 239-50.
• 5. Ruhli FJ, Henneberg M. Are hyperostosis frontalis interna and leptin linked? A hypothetical approach about hormonal influence on human microevolution. Med Hypotheses. 2002; 58: 378-81.
• 6.Rosatti. Family affected by hyperostosis frontalis interna (Morgagni-Morel Syndrome) through 4 successive generations. J Genet Hum 1972; 20: 207-52.
• 7.Koller MF, Papassotiropoulos A, Henke K et al. Evidence of a genetic basis of Morgagni-Stewart-Morel syndrome. A case report of identical twins. Neurodegener Dis. 2005; 2: 56-60.
• 8. Hershkovitz I, Greenwald C, Rothschild BM, et al. Hyperostosis frontalis interna: an anthropological perspective. Am J Phys Anthropol 1999; 109: 303-25.