RESUMO
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.
