Many people with ONH have abnormalities of the brain. These abnormalities may include how is the brain formed (brain structure) and how the brain works (brain function). While both usually occur, sometimes a child has a problem only with the structure of the brain and at other times, a child has a problem only with the function of the brain. All the problems with the brain can range from minor to very serious.

The normal brain is made up of two equal parts (hemispheres), which are connected by nerve fibers (corpus callosum) and are separated by fluid-filled spaces (ventricles). The ventricles have dividers between them (septum pellucidum).

Some people with ONH have a problem with the formation of the septum pellucidum; this is called septo-optic dysplasia or DeMorsier's syndrome. Others have an abnormal corpus callosum. Some people also have other parts of the brain which are abnormally formed. These can be seen with the CT (computerized tomography) or MRI (magnetic resonance imaging). Problems with the formation of the brain can be quite varied often involving the hemispheres and the ventricles. These can lead to small brains, excessively large ventricles, and fluid filled sacs (cysts) in the ventricles.

In some people with ONH, these brain problems are minor and do not cause abnormal brain function. In others, brain function is affected, ranging from mild to severe. The major areas which can be involved are the use of large muscle (gross motor) and small muscle (fine motor), intelligence and learning, speech, and interacting with people. Many of these problems can be helped with therapy. Some children with ONH also have seizures (fits, convulsions) and may need to take medicines to control the seizures.

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