[Cerebral gigantism--Sotos' syndrome].
A review of three children who attended a school for the mentally retarded shows that they have Sotos' syndrome. Sotos syndrome, or cerebral gigantism, was first described in 1964. This syndrome is characterized by rapid growth from 1-4 years of age, advanced osseous maturation, large size at birth, macrocephally and a high forehead. The head also grows fast from infancy to two years of age. The children have mild dilatation of lateral ventricles, large hands and feet. They also have dysmorphic features, such as hypertelorism, down slanting palpebral fissure, higharched palate, non progressive neurological disorder and variable degrees of mental retardation. The etiology is unknown, but autosomal recessive cases have been reported. Some cases have subsequently shown a fragile x-chromosome. Typically, the final height of the children is normal.
Eskeland
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Spesialskole for barn og ungdom, med laerevansker, Torshov skole, Oslo.
D000293:Adolescent; D000328:Adult; D005260:Female; D005877:Gigantism / Q000150:complications / Q000175:diagnosis; D006801:Humans; D008607:Intellectual Disability / Q000150:complications / Q000175:diagnosis; D008297:Male; D012886:Skull / Q000002:abnormalities; D013577:Syndrome
Cerebral gigantisme--Sotos' syndrom.