Aicardi syndrome

This is a rare X-linked dominant condition which is associated with dysgenesis of the corpus callosum and severe mental retardation. So far, over 200 cases (all females) of this syndrome have been reported/68 However, there are other syndromes in which corpus callosal dysgenesis has been shown to be associated with mental retardation/15 Common physical features of Aicardi syndrome include microcephaly, scoliosis, muscular hypotonia, facial asymmetry, low set ears, and eye lesions such as chorioretinal lacunae. A high proportion of children develop epileptic seizures, particularly in the form of infantile spasms. Neuroimaging studies show evidence of cerebral dysgenesis in the form of neuronal migration disorder in the brains of the affected children along with corpus callosal dysgenesis. (69 The prognosis for this condition remains poor and death in infancy is common. Over a quarter of the children manifest behavioural problems in the form of aggression towards people and objects, uncommunicativeness, lethargy, and frequent night walking. Self-injurious behaviour has been reported in over half of the children with Aicardi syndrome/25

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