Full Penetrance of Morgagni-Stewart-Morel Syndrome in a 75-Year-Old Woman: Case Report and Review of the Literature

Attanasio, Francesca; Granziera, Serena; Giantin, Valter; Manzato, Enzo

doi:10.1210/jc.2012-3242

Cited by 20 publications

(30 citation statements)

References 24 publications

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“…Metabolic and endocrine dysfunctions could be interpreted, not only as isolated components of the syndrome but also as the reason behind its pathogenesis. Endocrine or nutritional disorders may lead to an altered bone metabolism . The pathophysiology of the psychiatric symptoms of MSM syndrome, which has been showed in multiple publications, remains unknown at this point.…”

Section: Discussionmentioning

confidence: 99%

Finding of Hyperostosis Frontalis Interna During the Autopsy Procedure: Forensic Issues

Bascou

Savall

Vergnault

et al. 2019

Journal of Forensic Sciences

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Hyperostosis frontalis interna is a common phenomenon, which may have been overrated in its significance in the past, and may, currently be underrated in its significance. We present three cases of hyperostosis frontalis interna found during medicolegal autopsies and discuss their forensic considerations. The patients were all middle‐aged women with metabolic and endocrine manifestations and psychiatric ailments; thickening of the inner table of the frontal bone of the skull was found during each autopsy. We describe the relationship between hyperostosis frontalis interna, metabolic manifestations, and neuropsychiatric symptoms as part of Morgagni‐Stewart‐Morel syndrome. There is still considerable disagreement in the scientific community as to whether this syndrome is a clinical entity. Nonetheless, awareness of Morgagni‐Stewart‐Morel syndrome can be of help in understanding the circumstances surrounding death. In some other cases, hyperostosis frontalis interna could be used by forensic pathologists as criteria for sexing and aging a skeleton.

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Section: Discussionmentioning

confidence: 99%

Finding of Hyperostosis Frontalis Interna During the Autopsy Procedure: Forensic Issues

Bascou

Savall

Vergnault

et al. 2019

Journal of Forensic Sciences

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“…The known symptoms of HFI are mostly based on case reports, and they tend to be non-specific and benign. 3 In a severe form of HFI, motor aphasia, rightsided parkinsonism, and moderate dementia with worsened response to levodopa were reported. 4 In a less severe case with 0.6-cm endostosis, reduced cerebral blood flow in the frontal region was detected.…”

Section: Discussionmentioning

confidence: 99%

“…1 Although meaningful association between HFI and neurological disorders has not been proven, HFI frequently accounts for neuropsychiatric symptoms including frontal executive dysfunctions, epilepsy, cognitive impairments, parkinsonism, and frontal headache. [2][3][4] Here, we present an HFI case involving a woman with symptoms of depression and parkinsonism.…”

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confidence: 99%

Hyperostosis frontalis interna presenting as depression and parkinsonism in an older woman

et al. 2015

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Dear Editor, Hyperostosis frontalis interna (HFI) is a well-described entity from ancient times that occurs in 24% of women and 5.2% of men. 1 Although meaningful association between HFI and neurological disorders has not been proven, HFI frequently accounts for neuropsychiatric symptoms including frontal executive dysfunctions, epilepsy, cognitive impairments, parkinsonism, and frontal headache. 2-4 Here, we present an HFI case involving a woman with symptoms of depression and parkinsonism. CASE PRESENTATIONA 78-year-old woman complained of excessive fatigue, decreased rate of speech, and motor behaviours over a period of five years. As a result, she was diagnosed with Parkinson's disease and was prescribed levodopa and levodopa/carbidopa/ entacapone in outpatient neurology clinics. Because of unresponsiveness to these treatments and the nonprogressive course, the patient was referred to geropsychiatric outpatient clinics. Her physical and mental examinations revealed excessively decreased rate of speech but not poverty of speech, hypophonia, and a thought rate in the normal range without delusion. Affect was limited, decrease in facial movements, and mood was dysthymic. The patient's spontaneous attention was impaired, but her memory was intact. Bradykinesia, bilateral +2 rigidity in the upper extremities, and gait with small steps but without tremor were noted. We performed cranial magnetic resonance imaging that revealed HFI (Fig. 1).The patient was assessed in a geriatrics consultation that aimed to exclude secondary causes of Figure 1 Brain computed tomography of the patient showing left frontal thickness (12.8 mm).bs_bs_banner

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“…Morgagni-Stewart-Morel syndrome (MSM) is characterized by metabolic, endocrine and neuropsychiatric disorders accompanying hyperostosis frontalis interna (HOFI) [1,2].The main finding of the disease is HOFI; defined as the benign growth of the inner surface of the frontal bone [3].The disease can manifest with a wide variety of endocrine and neuropsychiatric conditions. Except HOFI, other findings are partially monitored in most of the cases [1,4].We present MSM patient with HOFI and neuropsychiatric findings under the light of radiological and clinical findings.…”

Section: Introductionmentioning

confidence: 99%

Morgagni-Steawart-Morel syndrome: Case report

Doğan

Mouline²

2019

Turkish Journal of Clinics and Laboratory

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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 a misunderstood and less reported pathology. The clinical presentation is not well defined and its finding is usually coincidental. The patient may present variable symptomatology. The idea that we should keep in mind is that HOFI represent the main pattern of MSM. Thus, it is highly important to specify the presence and degree of HOFI in radiological reports. ÖzHiperostozis 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 internaCorresponding author * : Emrah Doğan,

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Full Penetrance of Morgagni-Stewart-Morel Syndrome in a 75-Year-Old Woman: Case Report and Review of the Literature

Cited by 20 publications

References 24 publications

Finding of Hyperostosis Frontalis Interna During the Autopsy Procedure: Forensic Issues

Finding of Hyperostosis Frontalis Interna During the Autopsy Procedure: Forensic Issues

Hyperostosis frontalis interna presenting as depression and parkinsonism in an older woman

Morgagni-Steawart-Morel syndrome: Case report

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