Scientists identify cause of double-cortex syndrome

Published on June 22, 2012 at 2:30 AM · 5 Comments

Researchers at McGill University have discovered the cause of an inherited form of epilepsy. The disease, known as double-cortex syndrome, primarily affects females and arises from mutations on a gene located on the X chromosome. Drs. Susanne Bechstedt and Gary Brouhard of the Department of Biology have used a highly advanced microscope to discover how these mutations cause a malformation of the human brain. The results of their study are published in the journal Developmental Cell.

When the brain develops in the uterus, new brain cells are born deep within the brain, near the center. These newborn brain cells then crawl out of the so-called "niche" where they were born and migrate outward to the edges of the brain. This outermost layer of the brain is known as the cerebral cortex and is the seat of all higher-level thinking and cognition.

In girls with a mutation on their X chromosome, the outward migration of brain cells unfortunately fails. Instead of making it all the way to the edges of the brain, some of the brain cells pile up on top of one another and form a secondary or "double-cortex." The activity of these abnormally placed brain cells gives rise to seizures and also, in some cases, mental retardation.

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  1. John Nichols John Nichols Canada says:

    Does a male with xxy have migraine problems also on epileptic medication, tegratol CR, 200 mg take 1 1/2 tabs 2x day, and clobazam 10 mg 1 tab 2x day.

  2. Linda Egdell Linda Egdell Canada says:

    The info on this subject amazing. Our dtr who has uncontrolled epilepsy, some mental challenges and a double cortex lives with us. She is now 38. It lifts the spirits to know that research continures in this area.

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