Morgagni-Steawart-Morel sendromu: Olgu sunumu

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.

Anahtar Kelimeler:

Morgagni-Stewart-Morel Sendromu, Hiperostozis frontalis interna

Morgagni-Steawart-Morel syndrome: Case report

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,

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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.