Document Detail

Central nervous system involvement in Leber congenital amaurosis.
MedLine Citation:
PMID:  1512663     Owner:  NLM     Status:  MEDLINE    
Sixteen patients with ocular criteria of Leber congenital amaurosis (LCA) were studied. Neurological examination was normal in 11 of 16 patients. The electroencephalogram was normal in six of eight patients, while two had nonspecific theta slowing. Computerized tomography of the brain was normal in two of five; hypoplasia of the cerebellar vermis was seen in one, and mild to moderate ventriculomegaly was seen in two of five. Six patients underwent the verbal subtests of the Wechsler Intelligence Scale and a detailed battery of neuropsychological tests. Subtests dependent on early environmental influences were most severely affected, while culture-free subtests showed average or well above average performance. These results strongly support the hypothesis that children with LCA may be capable of normal cognitive functioning, although they perform poorly on standard IQ tests. This is related to their early environmental and sensory deprivation, rather than to a primary central nervous system dysfunction.
A Steinberg; S Ronen; Z Zlotogorski; B Z Silverston; I Hirsch; I Nawratzki
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of pediatric ophthalmology and strabismus     Volume:  29     ISSN:  0191-3913     ISO Abbreviation:  J Pediatr Ophthalmol Strabismus     Publication Date:    1992 Jul-Aug
Date Detail:
Created Date:  1992-10-01     Completed Date:  1992-10-01     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  7901143     Medline TA:  J Pediatr Ophthalmol Strabismus     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  224-7     Citation Subset:  IM    
Department of Pediatrics, Shaare Zedek Medical Center, Jerusalem, Israel.
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MeSH Terms
Blindness / congenital,  physiopathology*
Child, Preschool
Nervous System Diseases / physiopathology*,  radiography
Neuropsychological Tests
Optic Atrophies, Hereditary
Sensory Deprivation
Tomography, X-Ray Computed
Wechsler Scales

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