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[进行性面部半侧萎缩]

[Progressive facial hemiatrophy].

作者信息

Naumbaev A N, Sharipov A Sh, Iakhontov B V

出版信息

Zh Nevropatol Psikhiatr Im S S Korsakova. 1991;91(5):88-9.

PMID:1654716
Abstract

The authors describe a case of progressive facial hemiatrophy in a woman aged 26 years, coming from the Isfarin region of Tadzhikistan. The patient views herself as being ill for 14 years, since the moment of an epileptic attack with tonic and clonic convulsions. Approximately at the same time she noted a small dry ulcer on the left on the vertex. The ulcer slowly increased, followed by skin atrophy. The disease progressed for 4 to 5 years. At present to the left there are folds in the form of scars on the face. The skin is thinned, united with the bones in the frontal and parietal areas, the subcutaneous fat is atrophic. The lips and nose at the left are subatrophic. Negligible enophthalmos, hemiatrophy of the tongue at the left. Alopecia. A certain deterioration of memory and reduction of the critical attitude are recorded. The patient is in a state of euphoria. Left-sided anosmia. The left auricular floor is subatrophic, hearing is almost lacking. Diffuse elevation of the tendinous reflexes of the limbs on the left side. X-ray signs of osteoporosis of the bones of the cranial vault on the left.

摘要

作者描述了一例来自塔吉克斯坦伊斯法林地区的26岁女性进行性面部半侧萎缩症。患者自出现强直阵挛性癫痫发作起,认为自己患病已14年。大约在同一时间,她注意到头顶左侧有一个小的干燥溃疡。溃疡逐渐增大,随后出现皮肤萎缩。疾病进展了4至5年。目前,左侧面部有瘢痕形式的褶皱。皮肤变薄,在额部和顶叶区域与骨骼相连,皮下脂肪萎缩。左侧嘴唇和鼻子轻度萎缩。有轻微眼球内陷,左侧舌头半侧萎缩。脱发。记录到记忆力有一定减退,批判性态度下降。患者处于欣快状态。左侧嗅觉丧失。左耳廓底部轻度萎缩,几乎无听力。左侧肢体腱反射弥漫性亢进。左侧颅顶骨骨质疏松的X线征象。

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