Sotos syndrome (cerebral gigantism): analysis of 8 cases.
نویسندگان
چکیده
Sotos syndrome or cerebral gigantism is characterized by macrocephaly, overgrowth, mental retardation and central nervous system abnormalities. Congenital heart defects may be present. We report 8 patients with this syndrome and relate their clinical features, neuroimaging and echocardiographic findings.
منابع مشابه
Sotos' syndrome (cerebral gigantism) with peripheral dysostosis.
Evans, P. R. (1971). Archives of Disease in Childhood, 46, 199. Sotos' syndrome (cerebral gigantism) with peripheral dysostosis. A girl had short stumpy hands and feet due to osseous dystrophy; in other respects she showed all the main and many of the minor features of Sotos' syndrome during infancy. Then she was on the 97th centile for height, but she grew to be a woman on the 10th centile. Me...
متن کاملEvidence for autosomal recessive inheritance in cerebral gigantism.
Three cases of cerebral gigantism, two sibs and their double first cousin, are describedina largeinbred family from Israel. Two of the three were observed and diagnosed at birth and two were followed for two years. They all presented the signs and symptoms considered typical ofthis syndrome, as well as some ofthe less frequent findings. Generalized oedema and flexion contractures of the feet we...
متن کاملNeuroblastoma in a patient with Sotos' syndrome.
Sotos' syndrome, or cerebral gigantism, is a disorder of growth regulation. Tumours have occasionally been reported in children with Sotos' syndrome, but it is uncertain whether this is a coincidence, or whether it is aetiologically related to the underlying disorder of growth. We report a 15 month old child with a paraspinal neuroblastoma and Sotos' syndrome and suggest that children with this...
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BACKGROUND/AIMS True gigantism is an exceptional and fascinating pediatric disease. Our aim in this study was to describe the different etiologies of a large group of children with gigantism and the natural history of their growth. METHODS In this multicenter study, we considered as giant children, adolescents and adults whose heights were ≥3 SD compared to their target stature or to our popu...
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ورودعنوان ژورنال:
- Arquivos de neuro-psiquiatria
دوره 60 2-A شماره
صفحات -
تاریخ انتشار 2002