[Ophthalmia neonatorum gonorrheae].
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Biomedical subjects
Publications and source records attributed to S Ronen.
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The importance of early management of sight in a baby with Hallermann-Streiff-Francois syndrome is described. Surgery for bilateral cataract was performed. Special postcataract contact lenses were fitted.
A case presenting with three episodes of recurrent benign abducens palsy is reported. The patient experienced her first episode at age of 3 years, her second when she was 13 years old, and the last at the age of 25 during pregnancy. Full recovery was attained within approximately three months after each episode.
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.
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