What is Intractable Epilepsy in Infants & Children?
A seizure is a burst of uncontrolled electrical activity in the brain. It temporarily affects how a person acts, feels or appears. When a person has had two or more seizures, it is called epilepsy. Medicine often helps control epilepsy. If a child’s seizures still happen after taking two seizure medications, the epilepsy is called “intractable.” Doctors sometimes call this condition as “uncontrolled epilepsy” or “drug-resistant epilepsy.”
About 30 percent of children who have epilepsy are diagnosed with intractable epilepsy. Any type of epilepsy can become intractable. Intractable epilepsy is treatable. Some children may be seizure-free with expert care.
Some children outgrow epilepsy. It often depends on what kind of epilepsy the child has. After making an accurate diagnosis, the care team will talk to families about the possibility of their child outgrowing the condition.
Causes of Intractable Epilepsy
There is no single cause of intractable epilepsy. It is difficult to predict who will develop it. A child’s risk of developing this condition may be higher if:
- The child’s epilepsy is due to a structural abnormality in the brain. An abnormality can be caused by a history of brain infection. Or a problem with brain development. It can also be caused by focal cortical dysplasia or injury from oxygen deprivation or strokes.
- The child has an epilepsy syndrome that is associated with intractable epilepsy. Examples of these epilepsy syndromes include Lennox-Gastaut, Dravet, Doose and others.
- A genetic condition associated with intractable epilepsy is found.
- The child develops epilepsy in the first two years of life.
- The child has frequent seizures lasting five minutes or more.
- The child’s seizures continue after six or more months of taking seizure medication.
- The child has multiple seizures but does not receive prompt treatment.
- The child has a developmental condition, such as autism or a speech delay. Such conditions do not cause intractable epilepsy. But they may be signs that the child’s brain did not develop properly and is more prone to seizures.
If you think your child may be having seizures, seek care quickly. Children tend to do worse the longer their seizures are uncontrolled. Early treatment can lead to a much better outcome and quality of life.
Treatment for Intractable Epilepsy
If your child is still having seizures after taking two carefully chosen seizure medications, the chances of success with a third medication are very low (about 5-10 percent). It may be time to consider other treatment options. These can include a special diet and different types of surgery.
The first step is for the epilepsy doctor (epileptologist) to order further tests and imaging to figure out what is causing the seizures. The goal is to pinpoint where the seizures are starting in the brain. This region is called the “seizure focus.” The care team will look to see how close it is to brain regions that control:
- Other critical functions
There has been a recent explosion of new therapies and treatment strategies for children with intractable epilepsy. An expert team can help you learn about the options that may work best for your child.
Special Diet for Intractable Epilepsy
Some children do better when they follow a ketogenic or modified Atkins diet. These diets are high in fat, low or no carbohydrates, and moderate in protein. A child who follows this kind of diet gets more energy from fats and proteins than carbohydrates. Experts aren’t sure why this works. But they think such diets reduce inflammation in the brain to prevent seizures from happening.
These diets may help children with intractable epilepsy who aren’t surgical candidates. Surgery might not be recommended due to where their seizure focus is located or other factors. They also can help children with specific conditions, including:
- Lennox-Gastaut syndrome
- Infantile spasms (West syndrome)
- Rett syndrome
- Tuberous sclerosis complex
- Dravet syndrome
- Doose syndrome
- GLUT-1 deficiency
Studies show that these diets help up to two-thirds of children who follow them. Some children eventually may be able to decrease the dose of their seizure medications or stop their medications.
A registered dietitian can teach families about these diets. They can also provide support along the way. It can take about three months of following one of the diets to know whether it will help your child.
Surgery for Intractable Epilepsy
Surgery can be an option for children with intractable epilepsy, even if imaging tests do not reveal a brain abnormality that is causing the seizures. About half of children with intractable epilepsy may be candidates for surgery.
The goal of epilepsy surgery is to dramatically lower how severe and how often seizures happen. When possible, the goal is to eliminate them.
Surgery for Children Whose Seizures Arise from a Single Location in the Brain
If a child’s seizures come from a single location in the brain, the care team may recommend resection or laser ablation surgery. Neurosurgeons perform these surgeries.
- Epilepsy Resection Surgery. Removes the brain tissue causing seizures.
- Laser Ablation Surgery for Epilepsy. Uses a medical device (such as a laser) to destroy the brain tissue that is causing the seizures.
At Cincinnati Children’s, more than 50 percent of patients who have resection or ablation surgery are free from seizures. Typically, patients who do not achieve complete seizure freedom with surgery have significantly fewer seizures, sometimes 90 percent fewer.
Other Surgeries for Children with Intractable Epilepsy
Resection and ablation surgeries may be too risky if the seizure focus is near brain regions that control:
- Other critical functions
Also, these surgeries are not used to treat seizures that may be coming from multiple locations in the brain.
The care team may recommend implanting a device that will help control, target or prevent the seizures. These types of surgeries include:
- Vagus nerve stimulation (VNS)—A device (stimulator) is implanted. It sends mild pulses of electrical energy to the brain through the vagus nerve. The pulses can shorten or prevent seizures.
- Responsive neuro stimulation (RNS)—A neurostimulator device is implanted in the skull. It gives small electrical pulses when it recognizes that a seizure is about to start. The pulses control brain activity to prevent seizure symptoms.
- Deep brain stimulation (DBS)—In select cases, RNS electrodes are placed deep into the brain in an egg-like structure called the thalamus. The electrodes deliver signals to the brain to reduce seizure frequency. This therapy is like traditional DBS that is used by doctors to treat movement disorders.
The care team may recommend other types of surgery for children whose seizures come from one or more brain regions. These surgeries include:
- Lobectomy—The brain has four different lobes (sections), including the temporal, frontal, occipital and parietal. A lobectomy involves surgically removing the lobe where the seizures are starting.
- Cortical resection surgery—This involves removing the part of the brain that is causing seizures. It does not remove the entire lobe.
- Corpus callosotomy—This involves cutting a band of nerve fibers that connects the brain’s two halves (hemispheres). This interrupts the spread of seizures from one side of the brain to the other.
- Hemispherectomy—This involves removing the connection between the right and left sides of the brain. This prevents seizures from spreading from the diseased part of the brain to the part that is not causing seizures.
- Hemispherotomy—This involves removing the right or left side of the brain.
Children whose seizures come from multiple brain regions may also be candidates for VNS or RNS devices.
What Does Intractable Epilepsy Mean for Your Child?
Even with treatment, intractable epilepsy may be a lifelong condition. It can lead to many issues, including:
- Difficulty with daily living skills
- Delays in speech and motor skills (such as walking)
- Depression or anxiety due to worrying about when a seizure may occur
- Difficulty thinking and paying attention
- Not being able to drive a car due to seizure risk
- Reproductive issues
- Trouble doing schoolwork
- Trouble sleeping
Children who are not seizure-free need to be careful since accidents can occur during a seizure. They should not take baths alone or swim without adult supervision. Furniture in the home should have pads on any hard edges just in case the child falls nearby during a seizure. The care team will talk to you about steps you can take to keep your child safe.
SUDEP: Know the Risks
A very small number of children with intractable epilepsy lose their lives to a condition called SUDEP. SUDEP stands for “sudden, unexpected or unexplained death in epilepsy.” When someone dies of SUDEP, there are no other obvious reasons for the death. The autopsy shows no other cause of death. This condition is the leading cause of death in people with uncontrolled seizures.
The risk of SUDEP is approximately 1 in 4,500 children with epilepsy. Most children with epilepsy will not develop SUDEP. Children who experience motor convulsive seizures, especially at night, are at highest risk for SUDEP. Other risk factors include:
- Living or being alone a lot
- Substance and drug abuse
To reduce the risk of SUDEP, children must take their seizure medication as directed. If your child’s seizure medication causes difficult side effects, let the neurologist know. Work with the neurologist to create strategies for reducing the risk of SUDEP. This can include having your child wear a seizure-alerting device.
Children who continue to have seizures despite treatment need a lot of support. A caring, experienced medical team can help families who are dealing with this challenge. Most children with epilepsy who receive care at Cincinnati Children’s lead happy lives and still go to school despite these challenges. We have a close network of epileptologists, psychologists, nurses, medical assistants, neuropsychologists and neurosurgeons who will help support you and your family.