A 2-year-old boy with psychomotor retardation, congenital unilateral ptosis, bilateral adducted thumbs, weakness of upper limbs, and Hirschsprung's disease (aganglionosis), with complete agenesis of the corpus callosum and hypoplasia of the inferior vermis and cerebellum is reported. His 24-year-old maternal uncle, with severe psychomotor retardation but none of the other physical problems, als...

The corpus callosum has been proposed as a mechanism of interhemispheric inhibition that allows language dominance to develop [1]. Callosal agenesis or dysgenesis provides a test of this hypothesis, as patients lacking a normal corpus callosum should also lack normal language lateralization [2]. We report pre- and postoperative functional magnetic resonance imaging (fMRI) and neuropsychological...

background: x-linked lissencephaly with ambiguous genitalia (xlag) is a recently described genetic disorder, in which patients present with lissencephaly, agenesis of the corpus callosum, refractory epilepsy of neonatal onset, acquired microcephaly, and male genotype with ambiguous genitalia. xlag is responsible for a severe neurological disorder of neonatal onset in boys. a gyration defect con...

Aicardi syndrome is defined by the clinical triad of infantile spasms, agenesis of the corpus callosum, and pathognomonic chorioretinal lacunae. Almost all patients are females with severe cognitive and physical disabilities. All of the cases reported in the literature have had early-onset seizures. Most cases of Aicardi syndrome exhibit very slow development, even when seizures are eventually ...

The role of the corpus callosum versus other cerebral commissures in the interhemispheric integration of visual information was studied in four individuals with complete agenesis of the corpus callosum, two individuals with partial agenesis, one total commissurotomy patient, and normal individuals. Evoked potential (EP) indices of interhemispheric transmission of visual sensory responses were o...