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[莫尔加尼-斯图尔特-莫雷尔综合征。病例报告及文献复习]

[Morgagni-Stewart-Morel syndrome. Case report and review of the literature].

作者信息

Gracia-Ramos Abraham Edgar

机构信息

Departamento de Medicina Interna, Subdirección de Servicios Clínicos, Hospital General "General José María Morelos y Pavón", Instituto de Seguridad y Servicios Sociales de los Trabajadores del Estado, Zumpango de Ocampo, Estado de México, México.

出版信息

Rev Med Inst Mex Seguro Soc. 2016 Sep-Oct;54(5):664-9.

Abstract

BACKGROUND

Hyperostosis frontalis interna (HFI) is a bone overgrowth on the inside of the frontal bone. This alteration can occur in isolation or together with neuropsychiatric symptoms, metabolic and endocrine manifestations which together form the Morgagni-Stewart-Morel syndrome. In this regard, the case of a patient who meets criteria for this syndrome is presented and a review of the literature is performed with focus on its pathophysiology.

CLINICAL CASE

A 74 years old female with a history of exposure to wood smoke, vitiligo, type 2 diabetes mellitus, hypertension and cognitive impairment who enters the hospital by malaise, dizziness, anxiety, confusion, disorientation and difficulty walking. In she were performed imaging of the skull where was observed the presence of extensive hyperostosis frontalis interna, cortical atrophy and a left thalamic lacunar infarction. During this hospital stay the presence of grade I obesity, hyperglycemia, hypertriglyceridemia and hyperuricemia was documented.

CONCLUSIONS

The patient met the criteria of Morgagni-Stewart-Morel syndrome to manifest the presence of hyperostosis frontalis interna with metabolic, endocrine and neuropsychiatric manifestations. The pathophysiological origin of the syndrome is unknown, although it has been postulated that an endocrine imbalance motivated by genetic and environmental factors may be the cause.

摘要

背景

额骨内板增生症(HFI)是额骨内侧的骨质过度生长。这种改变可单独出现,或与神经精神症状、代谢和内分泌表现同时出现,这些共同构成莫尔加尼 - 斯图尔特 - 莫雷尔综合征。就此,本文介绍了一名符合该综合征标准的患者病例,并对文献进行综述,重点关注其病理生理学。

临床病例

一名74岁女性,有接触木烟、白癜风、2型糖尿病、高血压和认知障碍病史,因不适、头晕、焦虑、意识模糊、定向障碍和行走困难入院。对其进行颅骨成像检查时,发现存在广泛的额骨内板增生、皮质萎缩和左侧丘脑腔隙性梗死。在此次住院期间,记录到患者存在I级肥胖、高血糖、高甘油三酯血症和高尿酸血症。

结论

该患者符合莫尔加尼 - 斯图尔特 - 莫雷尔综合征的标准,表现为额骨内板增生伴代谢、内分泌和神经精神表现。该综合征的病理生理起源尚不清楚,尽管有人推测,由遗传和环境因素导致的内分泌失衡可能是其病因。

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