What is Cortical Dysplasia?

Cortical dysplasia is a brain disorder resulting from the abnormal early development of neurons in the brain's cortex. The abnormal neurons result in a number of neurological symptoms that become apparent early in life. It is the leading cause of epilepsy in young children. Diagnosing and treating the seizures that result from cortical dysplasia can be difficult since the portions of the brain affected in each individual are unique.

Definition

• Cortical dysplasia occurs when developing brain cells, or neurons, in an embryo or fetus fail to reach the parts of the brain's grey matter for which they are genetically destined. As a result, those areas of the cortex lack the appropriate neural connections to function properly. The affected cortical areas can be small and defined or can include an entire hemisphere of the brain.

Causes

• While exact causes of cortical dysplasia have not been identified, a large amount of research indicates that the condition may have a strong genetic component. Many young children who develop epilepsy stemming from cortical dysplasia have a family history of epileptic problems; in addition, those with other family members suffering from epilepsy tended to experience seizures at a younger age.

Symptoms

• Recurring seizures in an infant or very young child is the single most common symptom of cortical dysplasia. The epileptic brain activity stemming from cortical dysplasia surfaces very early in an affected child's life since the cortex abnormalities occur in utero and are present at birth. Other neurological symptoms will vary with which parts of the brain are affected, though other common symptoms include mental retardation; hyperactive, often aggressive behavior; a larger than average head; and abnormal deep tendon reflexes.

Diagnosis

• Cortical dysplasia can be difficult to diagnose since the affected portions of the brain differ with each individual. Following a neurological exam, scans such as a PET scan, CT scan and an MRI are performed to try to detect abnormal parts in the cortex. An electroencephalograzm, which uses electrodes on the scalp to monitor and record brain activity, is also used to pinpoint the location of the brain from which the seizures originate.

Treatment

• Treating cortical dysplasia is centered on treating and controlling the seizures that result from the condition, and the methods used will vary with each patient. Anticonvulsant medications are successful for some individuals, while others may have such severe and unmanagable seizures, surgery to remove the abnormal portion of the brain might be the most effective option.