Suppr超能文献

病例报告:一名患有威尔逊氏病的年轻男子出现创伤性脑干挫伤延迟诊断

Case report: concordant traumatic brainstem contusion delayed diagnosis in a young man with Wilson's disease.

作者信息

Marcus A, Ammermann C, Klein M, Schmidt M H

机构信息

Department of Child- and Adolescent Psychiatry, Central Institute of Mental Health, Mannheim, FRG.

出版信息

Eur Child Adolesc Psychiatry. 1995 Jan;4(1):46-54. doi: 10.1007/BF01987966.

Abstract

Wilson's disease is a hereditary autosomal recessive disorder of copper metabolism. The corresponding gene locus has been localized on the long arm of chromosome 13. Three different clinical variants of the disease can be distinguished: hepato-cerebral, abdominal/hepatic, and central nervous type. The heterogeneity of symptoms can cause problems in differential diagnosis, especially when another concordant disorder can also explain the pathogenesis of symptoms. The case report of a young man who suffered from brainstem contusion demonstrates the possibilities of misinterpretation because presenting symptoms could be attributed either to traumatic brain injury followed by adjustment disorder or Wilson's disease. Clinical signs included leftsided hemiparesis, bilateral gaze direction nystagmus, marked dysarthria with consecutive pervasive mutism, choreo-athetoid movements, spasmodic torticollis and diplopia dependent on gaze direction. Slit lamp examination showed Kayser-Fleischer's corneal ring. EEG- and computer assisted tomography investigations revealed non-specific findings. The patient was treated with D-Penicillamine. Alternative treatment with oral zinc preparations is discussed.

摘要

威尔逊病是一种遗传性常染色体隐性铜代谢紊乱疾病。相应的基因位点已定位在13号染色体长臂上。该疾病可分为三种不同的临床类型:肝脑型、腹/肝型和中枢神经型。症状的异质性会导致鉴别诊断出现问题,尤其是当另一种相关疾病也能解释症状的发病机制时。一名患有脑干挫伤的年轻男子的病例报告显示了误诊的可能性,因为出现的症状既可能归因于创伤性脑损伤继发适应障碍,也可能归因于威尔逊病。临床体征包括左侧偏瘫、双侧凝视方向眼球震颤、明显构音障碍并伴有持续性缄默症、舞蹈样手足徐动症、痉挛性斜颈以及依赖凝视方向的复视。裂隙灯检查显示有凯泽 - 弗莱舍尔角膜环。脑电图和计算机辅助断层扫描检查结果无特异性。患者接受了青霉胺治疗。文中还讨论了口服锌制剂的替代治疗方法。

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验