Document Detail


First report of prevalence of non-syndromic hereditary prosopagnosia (HPA).
MedLine Citation:
PMID:  16817175     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Ingo Kennerknecht; Thomas Grueter; Brigitte Welling; Sebastian Wentzek; Jürgen Horst; Steve Edwards; Martina Grueter
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  American journal of medical genetics. Part A     Volume:  140     ISSN:  1552-4825     ISO Abbreviation:  Am. J. Med. Genet. A     Publication Date:  2006 Aug 
Date Detail:
Created Date:  2006-08-25     Completed Date:  2007-01-16     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101235741     Medline TA:  Am J Med Genet A     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1617-22     Citation Subset:  IM    
Copyright Information:
Copyright 2006 Wiley-Liss, Inc.
Affiliation:
Institute of Human Genetics, Westfälische Wilhelms-Universität, Münster, Germany. kennerk@uni-muenster.de
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MeSH Terms
Descriptor/Qualifier:
Family Health
Female
Humans
Male
Pedigree
Prevalence
Prosopagnosia / epidemiology*,  genetics*
Questionnaires
Risk Factors

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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