Prevalence of Hereditary Prosopagnosia (HPA) in Hong Kong Chinese Population
Prevalence of Hereditary Prosopagnosia (HPA) in Hong Kong Chinese Population
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DOI：
10.1002/ajmg.a.32552
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发表时间：
2008-11-15
影响因子：
2
通讯作者：
Wong, Virginia C. N.
中科院分区：
文献类型：
--
作者：
Kennerknecht, Ingo;Ho, Nga Yee;Wong, Virginia C. N.
面孔失认症（PA），或无法识别一个熟悉的人的脸，被认为是一种罕见的功能障碍，主要是由脑外伤获得。最近我们发现，先天性PA在白种人中很常见，患病率为2.5%。由于这些病例是家族性的，我们创造了遗传性人面失认症（HPA）一词。本研究是第一个系统的筛选HPA在一个确定的人口中的少数民族。在2004-2005年度，香港大学约750名医科学生中，有533名参加了一项以药物为基础的筛查。133名学生的回答表明，他们很可能是PA的候选人。120人同意接受诊断性面谈。最后对10例患者进行了PA的临床诊断。确定的患病率为1.88%（95% CI，1.05-2.71），与白人的患病率范围相同。我们采取了详细的家族史的四个指数prosopagnosic人，并能够进一步调查的家庭四个先证者。每个人都有其他一级亲属患有相同的视觉认知功能障碍。因此，在高加索人中，规则的常染色体显性遗传可能是最好的解释分离模式。(C)2009威利-利斯公司
Prosopagnosia (PA), or the inability to recognize a familiar person by the face alone, had been considered to be a rare dysfunction mainly acquired by trauma to the brain. Recently we have shown that the congenital form of PA, which was considered to be even rarer, is common in Caucasians, with a prevalence of 2.5%. As these cases were familial we coined the term Hereditary Prosopagnosia (HPA). The present study is the first systematic screening for HPA in a defined population of ethnic Chinese. In 2004-2005, 533 out of around 750 medical students of The University of Hong Kong took part in a questionnaire-based screening. The responses of 133 students indicated that they were likely to be candidates for PA. One hundred twenty agreed for diagnostic interview. Finally we made the clinical diagnosis of PA in 10 subjects. A prevalence of 1.88% (95% CI, 1.05-2.71) is established which is in the same range as in Caucasians. We took a detailed family history of four index prosopagnosic persons and were able to further investigate the families of four probands. Each had other first-degree relatives with the same visual cognitive dysfunction. Thus, as in the Caucasians, regular autosomal dominant inheritance might best explain the segregation pattern. (C) 2009 Wiley-Liss, Inc.