Epilepsy

Cortical dysplasia is a malformation that occurs during fetal development when normal brain cells migrate to incorrect locations in the developing brain. It is one of the most common causes of epilepsy in children. The presence of these abnormal clusters of cells irritates the surrounding brain and triggers seizures. Surgical treatment involves resection of the focus of dysplasia, or abnormally located cells. These dysplasia can be small and focused or can involve an entire lobe of the brain.

Perinatal stroke may result in permanent injury to the brain. Studies show that four of five newborn infants who experience a stroke around the time of birth will develop neurologic disorders such as cerebral palsy, epilepsy, or language delay. Most often, when seizures result from a perinatal injury (injury shortly before, during or after birth), treatment involves disconnection or surgical removal of the affected area of brain to control the seizures. Because this area of the brain is already damaged and not working properly, this kind of surgery can be done safely with little risk of causing new problems for the child.

Mesial temporal sclerosis is scarring of the inner portions of the brain’s temporal lobe which is sometimes caused by brain injury or infection. This can lead to partial (focal) seizures that start in the temporal lobe but can spread to other areas of the brain. It is often associated with intractable epilepsy (seizures that don’t stop with medication). Surgery to remove the scarred areas of the temporal lobe may cure a patient’s seizures.

Tuberous sclerosis complex (TSC) is a genetic disorder that causes tumors to form in different organs, including the brain. While these tumors are usually benign (they do not spread or grow quickly), their presence can disrupt the surrounding brain cells and result in seizures as well as other developmental disorders such as autism.  Occasionally, tumors that do grow may also block the flow of fluid in the brain, a condition called hydrocephalus.  TSC can be treated with medications that shrink the tumors, specific seizure medications that control seizures that the tumors cause, and in some cases, with surgery to remove them.

Infantile spasms is a rare but serious form of epilepsy that begins in infants usually between the ages of 4 and 12 months.  Infantile spasms may develop in children with prior normal development or in children with other known or suspected brain disorders.  Seizures are usually brief, lasting only a second, but occur in repeated clusters usually within minutes after waking.  Often a child with spasms will have a sudden startle-like movement of both arms with or without the head suddenly falling forward or eyes rolling. Urgent diagnosis is needed. If the spasms continue unrecognized, babies will regress or lose developmental milestones. Other types of seizures may also develop. Treatment is generally with high-dose steroids, vigabatrin, ketogenic diet, and for babies whose spasms are not controlled, prompt evaluation for epilepsy surgery.

Lennox-Gastaut syndrome is a rare form of epilepsy in children that typically starts between the ages of 2 and 5 years. It can result in many different kinds of seizures (both convulsive and non-convulsive) that vary widely from child to child and often happen many times a day, resulting in decreased alertness and loss of skills. Lennox-Gastaut syndrome is difficult to treat, often requiring the use of several medicines at the same time. Some children with this diagnosis benefit from a specialized diet called the ketogenic diet. For some, palliative surgeries such as corpus callosotomy, a surgery that partially disconnects the right and left side of the brain to prevent seizures from spreading to both sides, is recommended to decrease the number of seizures or their severity. For other children, a small implantable device called a vagal nerve stimulator (VNS) may be recommended. This device sends a small electrical signal to the vagus nerve and has been shown to reduce the number of daily seizures for many patients.

Rasmussen encephalitis is a rare childhood disorder in which cells in a part of one hemisphere of the brain become inflamed and swollen, triggering progressive seizures that lead to weakness, loss of function, and cognitive deficits. The cause of the inflammation is not often known, but could be the result of a viral infection, an auto-immune disorder, or trauma. Successful seizure control requires a hemispherotomy which is a surgery to disconnect the affected side of the brain from the healthy side.

Sturge-Weber Syndrome is a rare congenital (present at birth) condition characterized by a birthmark (called a port wine stain) on one side of the face and neck, as well as neurological abnormalities. It is caused by abnormal blood vessels on the surface of the brain that can cause seizures that may become generalized and evolve into other types of seizures over time. Surgery called a hemispherotomy, which involves disconnecting the abnormal side of the brain from the normal side, can be used to treat it.