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[挪威小说家阿玛丽·斯克拉姆(1846 - 1905)对伴有巨头畸形、脑瘫、癫痫、智力残疾和生长发育迟缓的早期脑积水的描述]

[A description of early hydrocephalus with macrocephaly, cerebral palsy, epilepsy, intellectual disability, and growth retardation by Norwegian novelist Amalie Skram (1846-1905)].

作者信息

Hassel Bjørnar

机构信息

Avdeling for nevrohabilitering, Oslo universitetssykehus, Ullevål, Norway.

出版信息

Tidsskr Nor Laegeforen. 2012 Dec 11;132(23-24):2632-5. doi: 10.4045/tidsskr.12.0912.

DOI:10.4045/tidsskr.12.0912
PMID:23338097
Abstract

In her novel «S.G. Myre» written in 1890, Norwegian author Amalie Skram describes Hans (Hansemann) Tønnesen, a 16-year-old boy with an enormous head, convulsions, and a rudimentary command of language. He is bullied by children in the street and is held in low esteem by his mother, who refers to him as a beast, crazy, or «a cross». Presumably, the boy has hydrocephalus with macrocephaly, epilepsy, and mental retardation. His retarded growth, small teeth and cracked skin suggest growth hormone deficiency resulting from his hydrocephalus. A couple of interesting details, such as his «splaying his fingers and moving them in various directions to make himself understood» and his «becoming red with exertion and grimacing terribly» when trying to speak, suggest extrapyramidal, dyskinetic cerebral palsy. He may also be deaf. A cousin of Skram's shared several of these traits and is a likely model for Hansemann. A question that occurs to a modern reader is to what degree Hansemann is mentally retarded, or whether he is wrongly perceived as such because of his physical challenges and his communication problems.

摘要

挪威作家阿玛丽·斯克拉姆在其1890年创作的小说《S.G. 迈尔》中,描绘了汉斯(汉斯曼)·托内森,一个16岁的男孩,头部巨大,患有惊厥,语言能力发育不全。他在街上遭到孩子们的欺负,母亲也不尊重他,称他为野兽、疯子或“怪物”。据推测,这个男孩患有脑积水伴巨头畸形、癫痫和智力障碍。他生长发育迟缓、牙齿细小且皮肤干裂,表明其脑积水导致生长激素缺乏。一些有趣的细节,比如他“张开手指向不同方向移动以让别人明白他的意思”,以及试图说话时“用力时脸涨得通红,表情极其扭曲”,暗示了锥体外系运动障碍型脑瘫。他可能也失聪了。斯克拉姆的一个表亲也有这些特征,很可能是汉斯曼的原型。现代读者会想到的一个问题是,汉斯曼的智力障碍到了什么程度,或者他是否因为身体残疾和沟通问题而被错误地认为是智力障碍。

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