Document Detail

Congenital retinal dystrophies: a study of early cognitive and visual development.
MedLine Citation:
PMID:  1374226     Owner:  NLM     Status:  MEDLINE    
The reported incidence of mental retardation in Leber's congenital amaurosis has varied from 10% to 87%. There has been no review of the estimate since it became possible to delineate an increasing number of diagnostic subcategories. In this study, the visual and cognitive development of 38 children with congenital retinal dystrophies has been followed up prospectively. Children with associated disorders in other systems and those with central nervous system malformations or degenerations were significantly more likely to have learning disability than those without additional medical problems. Most subgroups made little or no visual progress with the exception of the group with associated hypoplasia of the cerebellar vermis. The study highlights the importance of using the specialised techniques now available to delineate fully the visual diagnosis and paediatric perspective because of their relevance to cognitive and visual prognosis.
M M Black; P M Sonksen
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Archives of disease in childhood     Volume:  67     ISSN:  1468-2044     ISO Abbreviation:  Arch. Dis. Child.     Publication Date:  1992 Mar 
Date Detail:
Created Date:  1992-05-29     Completed Date:  1992-05-29     Revised Date:  2010-09-07    
Medline Journal Info:
Nlm Unique ID:  0372434     Medline TA:  Arch Dis Child     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  262-5     Citation Subset:  AIM; IM    
Department of Neurology and Developmental Paediatrics, Wolfson Centre, London.
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MeSH Terms
Child Development
Child, Preschool
Cognition Disorders / etiology*
Developmental Disabilities / etiology
Mental Retardation / etiology
Retinal Degeneration / congenital*,  psychology
Vision Disorders / congenital*
Vision, Ocular

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