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First report of prevalence of non‐syndromic hereditary prosopagnosia (HPA)
Author(s) -
Kennerknecht Ingo,
Grueter Thomas,
Welling Brigitte,
Wentzek Sebastian,
Horst Jürgen,
Edwards Steve,
Grueter Martina
Publication year - 2006
Publication title -
american journal of medical genetics part a
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.064
H-Index - 112
eISSN - 1552-4833
pISSN - 1552-4825
DOI - 10.1002/ajmg.a.31343
Subject(s) - epidemiology , first degree relatives , medicine , pediatrics , psychiatry , psychology , clinical psychology , family history , surgery
Acquired prosopagnosia (PA) is a rare condition after, for example, a stroke or brain injury. The congenital form of PA is generally considered to be even less common. Beside a few single case reports and anecdotal mentioning of familial cases no data on the epidemiology exists. Following a questionnaire‐based screening in local secondary schools and at our medical faculty, candidates suspicious for PA underwent a semi‐structured interview followed by examinations of first degree relatives. Among 689 local pupils and medical students of our university we found 17 with congenital PA. This corresponds to a prevalence rate of 2.47% (95% CI 1.31–3.63). The frequency is among the highest known for a monogenic disorder. All those index subjects (n = 14) of the target group who agreed to further examinations of their family members had other first degree relatives with the same cognitive disorder. This study provides epidemiological evidence that congenital PA is a very common cognitive disorder which almost always runs in families. The segregation pattern of this hereditary prosopagnosia (HPA) is fully compatible with autosomal dominant inheritance. © 2006 Wiley‐Liss, Inc.