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[一例伴有神经系统改变的表皮痣(雅达松氏错构瘤病)]

[A case of epidermal nevus (Jadassohn's phacomatosis) with changes in the nervous system].

作者信息

Jankowicz E, Sobaniec W, Czerwińska-Ciechan K, Sobaniec-Lotowska M, Drozdowski W

机构信息

Kliniki Neurologicznej AM, Białymstoku.

出版信息

Neurol Neurochir Pol. 1991 Jul-Aug;25(4):491-6.

PMID:1803260
Abstract

A 22-year old patient with Jadassohn's naevus phacomatosis affecting the right side of the head, face and brain is reported. Besides naevus linearis on the forehead, lipomata of the right palpebra, skin and palate, 2 odontomata, hypodermal and submucosal hyperplasia of the right half of the oral cavity, a small aneurysm of the internal carotid artery in the cavernous sinus and linear calcification in the cortex of the medial surface of the occipital lobe like those in Sturge-Weber disease were found. Clinically, she was found to be mentally retarded (moderately) and having epilepsy. Epileptic attacks occurred up to the age of 13 years, while changes in EEG are still present.

摘要

报告了一名22岁患有 Jadassohn 痣样错构瘤病的患者,病变累及头部右侧、面部和脑部。除了前额的线状痣、右眼睑、皮肤和腭部的脂肪瘤、2个牙瘤、口腔右半侧的皮下和粘膜下增生外,还发现海绵窦内颈内动脉的一个小动脉瘤以及枕叶内侧面皮质的线状钙化,类似于斯特奇-韦伯综合征的表现。临床上,发现她有中度智力发育迟缓并患有癫痫。癫痫发作一直持续到13岁,脑电图仍有变化。

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Neurol Neurochir Pol. 1991 Jul-Aug;25(4):491-6.
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