Leber's Congenital Amaurosis

1984; American Medical Association; Volume: 41; Issue: 2 Linguagem: Inglês

10.1001/archneur.1984.04050140102036

ISSN

1538-3687

Autores

Joel M. Weinstein,

Tópico(s)

Fetal and Pediatric Neurological Disorders

Resumo

Three patients (two of them siblings) had Leber's congenital amaurosis and cerebellar disease. Despite blindness and severe motor deficits, all three patients have achieved relatively normal intellectual and psychosocial milestones. Computed tomographic scans, performed in two patients, demonstrated hypoplasia of the cerebellar vermis in both. The presence of delayed speech and motor development as well as structural CNS abnormalities in children with Leber's congenital amaurosis does not necessarily imply that severe intellectual impairment will be present.

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