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The Lissencephaly Contact Group

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What is Lissencephaly?

Lissencephaly is a set of rare brain disorders and you will probably find that very few people have heard of it. Lissencephaly is used as an 'umbrella' term to describe a range of disorders where the whole or parts of the surface of the brain appear smooth.

You may have also been told that your child may be diagnosed with either agyria or pachygyria - these terms are generally used to describe the degree of brain smoothness and are often included in some of the diagnoses above.

The word lissencephaly is derived from the Greek "lissos" meaning smooth and "encephalos" meaning brain. The human brain normally has a convoluted surface. In Lissencephaly these convolutions are completely or partially absent from the brain, or areas of it, have a smooth appearance. The convolutions are also called "gyri" and their absence is known as "agyria" (without gyri). In some cases convolutions are present, but thicker and reduced in number and you may hear the term "pachygyria" (broad gyri) being used. The diagnosis is usually made with the help of a CT Scan or MRI Scan of the brain.

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