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Kohlschütter-Tönz syndrome (KTS) also called Amelo-cerebro-hypohidrotic syndrome is a rare inherited syndrome characterized by epilepsy dementia intellectual disability and yellow teeth caused by amelogenesis imperfecta (abnormal formation of tooth enamel). It is a type A ectodermal dysplasia.It is autosomal recessive and symptoms appear in early childhood. The syndrome was first described in 1974 by Alfried Kohlschütter and colleagues. Only 24 affected individuals are known as of 2012.

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