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Aicardi syndrome

Background

Aicardi syndrome is a very rare neurological condition due to a congenital abnormality of brain development. The characteristic features consist of under development or absence of the development (agenesis) of a brain structure known as the corpus callosum, a form of epilepsy (infantile spasms) and an abnormality to the structure and appearance of the retina of the eyes.

It is an example of one of the syndromic causes of agenesisof the corpus callosum. In Aicardi syndrome, in addition to the structural abnormality of the corpus callosum, other structures within the brain can also be abnormal. This can result in a variety of types of brain cyst and/or abnormal structure within areas of both grey or white matter of the brain.

What are the symptoms? View What are the symptoms?

Medical text written February 2000 by Dr K R E Pohl, Consultant Paediatric Neurologist, Guy's Hospital, London, UK. Last reviewed August 2005 by Dr L Rosenbloom, Consultant Paediatric Neurologist, Alder Hey Children's Hospital, Liverpool, UK.

 

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