Congenital prosopagnosia (CP) is a selective difficulty in recognizing familiar faces that is present from birth. There is mounting evidence for a familial factor in CP, possibly due to a simple autosomal inheritance pattern. However, potential candidate genes remain to be established, and the question whether genetically based CP is a single trait, or a cluster of related subtypes differing in the pattern of impairments to specific components of the face-processing system, remains unanswered. In addition, since the great majority of so far described cases with CP were adult at the time of investigation, it remains unknown which specific aspects of face processing are impaired in small children with CP. Here we present the first study that specifically addresses these questions by elucidating the specific mechanisms underlying face-recognition impairments in seven individuals with CP (aged 4-87 years) belonging to four generations of the same family. Our results indicate that genetically based CP is not a single trait but a cluster of related subtypes, since the pattern of impairments to specific components of the face-processing system varies in individuals belonging to the same family. In addition, we show that the heterogeneity of the cognitive profile in CP with respect to specific aspects of face processing is apparent from early childhood.

中文翻译：

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基于遗传的前列腺癌的认知异质性：家庭研究。

先天性prosopagnosia（CP）是识别出生时出现的熟悉面孔的选择性困难。有越来越多的证据表明CP中有家族因素，这可能是由于简单的常染色体遗传模式所致。然而，潜在的候选基因仍有待建立，基于遗传的CP是单一性状，还是在面部处理系统特定组成部分的损伤方式上不同的相关亚型簇的问题仍未得到解答。此外，由于到目前为止，描述为CP的大多数病例在调查时都是成年的，因此尚不清楚在患有CP的小儿童中面部处理的哪些具体方面受到损害。在这里，我们通过阐明属于同一家族四代的7名CP（4-87岁）个体的面部识别障碍的具体机制，提出了专门针对这些问题的第一项研究。我们的结果表明，基于遗传的CP不是单个特征而是一组相关的亚型，因为面部处理系统特定组件的损伤模式在同一家族的个体中有所不同。此外，我们表明，从儿童早期开始，就面部处理的特定方面而言，CP中认知特征的异质性就很明显。我们的结果表明，基于遗传的CP不是单个特征而是一组相关的亚型，因为面部处理系统特定组件的损伤模式在同一家族的个体中有所不同。此外，我们表明，从儿童早期开始，就面部处理的特定方面而言，CP中认知特征的异质性就很明显。我们的结果表明，基于遗传的CP不是单个特征而是一组相关的亚型，因为面部处理系统特定组件的损伤模式在同一家族的个体中有所不同。此外，我们表明，从儿童早期开始，就面部处理的特定方面而言，CP中认知特征的异质性就很明显。