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[额内板增生症的磁共振成像表现——1例莫尔加尼综合征]

[MRI findings of hyperostosis frontalis interna--a case of Morgagni syndrome].

作者信息

Akashi T

机构信息

Department of Psychiatry, Komagane Hospital, Nagano, Japan.

出版信息

No To Shinkei. 1996 Jul;48(7):667-70.

PMID:8753003
Abstract

We report a patient with Morgagni syndrome. The main aim of this paper is to discuss hyperostosis frontalis interna (HFI) and coexisting clinical feature and to describe the pathomorphology in detail on the basis of MRI images of the skull. The patient, a woman, was 82 years old when she first came to our hospital. She had a 20-year history of hypertension and chronic headache, and had been excessively obese till three years before. On admission she presented with a broad spectrum of clinical symptoms and signs including insomnia, disorientation to place, loss of memory, dementia, night delirium, reduced deep tendon reflexes in the lower extremities, urinary incontinence and upward gaze palsy. Because of a fair recovery within several days, it was suspected that so-called "treatable dementia" played a considerable role in the above-mentioned clinical state. Laboratory testing data, including hormone levels, were all within normal limits. EEG examinations showed slowed, diffuse, and poorly developed alpha-waves with no paroxysms. Cranial CT in horizontal sections disclosed a deformed frontal bone with convexlens-shaped thickening bilaterally and diffuse high density on both sides. MRI images revealed more detailed structures: the outer plate, diploe and inner plate of the skull, and abnormal ossifications. Based on these findings we diagnosed her illness as Morgagni syndrome. Recent reports, though few in number, have tended to focus on the EEG findings, hormones and psychiatric states in this syndrome, and descriptions of the HFI itself seem to be rare. The true cause of this syndrome is not yet known, so this rare presentation of MRI images of HFI is thought to be important in explaining this peculiar phenomenon in the skull.

摘要

我们报告一例莫尔加尼综合征患者。本文的主要目的是讨论额骨内板增生症(HFI)及其共存的临床特征,并根据头颅MRI图像详细描述其病理形态学。该患者为女性,初诊时82岁。她有20年高血压和慢性头痛病史,直到三年前一直过度肥胖。入院时,她出现了一系列广泛的临床症状和体征,包括失眠、地点定向障碍、记忆力减退、痴呆、夜间谵妄、下肢深部腱反射减弱、尿失禁和向上凝视麻痹。由于数天内病情有明显好转,怀疑所谓的“可治性痴呆”在上述临床状态中起了相当大的作用。实验室检查数据,包括激素水平,均在正常范围内。脑电图检查显示α波减慢、弥漫且发育不良,无阵发性发作。头颅CT水平断面显示额骨变形,双侧呈凸透镜状增厚,两侧弥漫性高密度影。MRI图像显示了更详细的结构:颅骨的外板、板障和内板,以及异常骨化。基于这些发现,我们将她的疾病诊断为莫尔加尼综合征。近期报告虽数量不多,但倾向于关注该综合征的脑电图表现、激素和精神状态,而对HFI本身的描述似乎很少见。该综合征的真正病因尚不清楚,因此HFI的这种罕见的MRI图像表现被认为对解释颅骨中的这种特殊现象很重要。

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