Rett syndrome in South Africa

Rett syndrome is a fairly recently recognized neurodevelopmental disorder of unknown aetiology that affects exclusively girls in whom early development is apparently normal but by the age of 6-18 months autistic behaviour and dementia, apraxia of gait, stereotypic repetitive hand movements, seizures and deceleration of head growth occur. Except for one Brazilian case, all previous reported cases have been from the northern hemisphere. We report three children (two Indian and one African) with the clinical features of Rett syndrome and believe these to be the first documented cases in Indian and African children in the southern hemisphere. More widespread knowledge of this syndrome is required, especially in developing countries, in order to prevent unnecessary and costly investigations, and to help families deal effectively with this important syndrome.