“I have intractable epilepsy,” shared one MyEpilepsyTeam member. “Meds since age 13, and two neurosurgeries later, still having seizures at age 59.” Intractable epilepsy refers to epilepsy that remains uncontrolled despite appropriate treatment with at least two antiepileptic drugs. You may have also heard intractable epilepsy referred to as uncontrolled, drug-resistant, or refractory epilepsy.

If you or your child has intractable epilepsy, it can be hard to predict when the next seizure will happen. While intractable epilepsy can be difficult to treat, it’s not always impossible. Keep reading to learn more about intractable epilepsy and how it’s managed and treated.

What Are the Symptoms of Intractable Epilepsy?

People with intractable epilepsy can experience uncontrolled seizure symptoms, such as:

• Convulsions
• Shaking or falling
• Staring into space
• Rigid or stiff muscles
• Loss of consciousness
• Loss of bowel and bladder control

Because intractable epilepsy isn’t a specific type of epilepsy, the seizures can look different from person to person.

The main sign of intractable epilepsy is that medications can’t control how often seizures occur, or how severe they can be.

Who Gets Intractable Epilepsy?

Studies show that about 30 percent of people with epilepsy don’t have their seizures controlled by anti-seizure medications, according to a 2021 review. In the studies reviewed, about 25 percent of children and 15 percent of adults diagnosed with epilepsy had intractable epilepsy.

Intractable epilepsy isn’t a specific seizure disorder, which makes it difficult to predict who’ll have it. However, it’s more common in certain groups. Studies show that children who develop epilepsy before age 2 are at a higher risk of intractable epilepsy, especially if they have the following risk factors:

• Epilepsy caused by a structural abnormality, an infection, or problems with brain development
• A syndrome linked with intractable epilepsy, such as Lennox-Gastaut syndrome, Dravet syndrome, Doose syndrome, or tuberous sclerosis complex
• A genetic condition associated with intractable epilepsy
• A history of status epilepticus (seizures that last five minutes or more)
• Seizures that continue after at least six months of treatment
• Lack of prompt treatment for seizures
• A developmental condition such as autism or a speech delay

In contrast, studies show that people with genetic generalized epilepsy (GGE) have a lower risk of intractable epilepsy. GGE is a subtype of general epilepsy that includes conditions like childhood and juvenile absence epilepsy, juvenile myoclonic epilepsy, and epilepsy with generalized tonic-clonic seizures alone (EGTCS).

What Types of Seizures Occur With Intractable Epilepsy?

Because intractable epilepsy isn’t a specific type of epilepsy, the seizures can look different from person to person. There are two main types of seizures — generalized and focal seizures (also known as partial seizures).

• General seizures affect both brain hemispheres at the same time from the beginning.
• Focal seizures start in a specific part of the brain but can spread to a larger area, becoming generalized.

People with focal seizures may be more likely to develop intractable epilepsy than people with generalized seizures.

How Is Intractable Epilepsy Diagnosed?

Intractable epilepsy is diagnosed using tests similar to those used for diagnosing other forms of epilepsy, such as MRI scans and electroencephalograms (EEGs). You may see an epilepsy specialist called an epileptologist or a neurologist at an epilepsy center.

Your doctor may ask questions to find out why your medication isn’t working. For example, your medication might be interacting with other medications, or you might be using the wrong drug, taking the wrong dose, or not taking it regularly. Other reasons for inadequate treatment might include exposure to seizure triggers such as flashing lights, illness, lack of sleep, or increased stress.

Electrical stimulation may be an option for people who can’t or don’t want to have brain surgery.

You may need to try several medications before your doctor diagnoses you with intractable epilepsy. A MyEpilepsyTeam member described their experience: “I have tried eight or nine medications and was only able to tolerate three of them without terrible side effects, and those have not been able to control my seizures.”

Additionally, your doctor must rule out other conditions that can mimic seizures, including:

• Fainting
• Stroke
• Low blood sugar
• Migraine headaches
• Movement disorders
• Panic attacks

How Is Intractable Epilepsy Treated?

Finding the right treatment may be difficult, but there are several options available for people with intractable epilepsy. One MyEpilepsyTeam member shared, “Given my 32 years of experience living with intractable epilepsy, I have seen that it’s trial and error for effective treatment and management.”

Antiepileptic Drugs

Before trying more invasive treatments, your doctor may suggest antiepileptic drugs. These drugs can be used alone or in combination. Antiepileptic drugs work by controlling the electrical activity in your brain. Different types of antiepileptics do this in different ways, such as affecting the movement of ions or chemical messengers in your brain.

If you’ve already tried two anti-seizure drugs and they didn’t work, there’s a lower chance that a third drug will be effective in treating intractable seizures. In that case, your doctor may recommend other, more invasive intractable epilepsy treatment options.

Surgery

Your doctor may recommend a type of surgery called resective epilepsy surgery to remove the part of your brain causing seizures. This procedure can be helpful if doctors know the exact area of the brain that’s causing the seizures.

A corpus callosotomy can also be effective in reducing seizures. During this procedure, a surgeon cuts the nerve fibers that connect the two halves of the brain, called the corpus callosum. This type of surgery may be offered to those with generalized seizures that affect both sides of the brain and don’t respond to medications.

Laser interstitial thermal therapy (LITT) is a minimally invasive surgery that uses heat to destroy brain tissue or cells that are causing seizures. LITT is becoming more common for treating people with drug-resistant epilepsy.

Electrical Stimulation

For people who can’t or don’t want to have brain surgery, electrical stimulation may be an option. This type of therapy involves implanting a device in your body that uses electrical activity to reduce seizures.

Options for electrical stimulation include vagus nerve stimulation (VNS), in which a device placed in the chest sends regular pulses of electrical energy to the brain through the vagus nerve in the neck. Other options, such as deep brain stimulation and responsive neurostimulation (RNS), use electrodes implanted in the brain to try to reduce the number of seizures you have.

Lifestyle Changes

Some people with epilepsy caused by problems related to metabolism (the way the body turns food into energy) may benefit from special diets. For example, people who have trouble getting glucose to their brain (known as GLUT1 deficiency) may do better on a high-fat, low-carbohydrate diet like the ketogenic diet, also known as the modified Atkins diet. A low glycemic index treatment (LGIT) diet, which limits foods that cause rapid spikes in blood sugar, may also help manage drug-resistant epilepsy.

If you have a type of vitamin-dependent epilepsy, your doctor may recommend supplements such as pyridoxine to manage the condition.

What’s the Outlook for People With Intractable Epilepsy?

Children and adults who aren’t seizure-free may need extra support. Intractable epilepsy may change or get worse over time. “I have intractable epilepsy. For many years, I just had the odd tonic/clonic seizure. For the last 20 years, they’ve changed in seizure frequency and type,” one MyEpilepsyTeam member said.

Unfortunately, the life expectancy of people with intractable epilepsy may be lower than that of people without intractable epilepsy. One population-based study in Canada found that 10 percent of people died within 2 years of developing intractable epilepsy.

Even with treatment, intractable epilepsy can have negative consequences, including:

• Difficulty with daily activities
• Developmental delays in children
• Risk of epilepsy-related injuries
• Increased risk of behavioral or emotional problems
• Difficulty concentrating
• Inability to drive
• Reproductive issues
• Increased risk of sudden unexpected death in epilepsy (SUDEP)
• Depression and anxiety
• Increased risk of side effects from taking multiple anti-seizure drugs

Talk to your healthcare team about the goals of your epilepsy treatment options. Together, you can find an epilepsy treatment that improves your quality of life with as few side effects as possible. You might also consider joining a clinical trial studying a new treatment for intractable epilepsy.

Talk With Others Who Understand

MyEpilepsyTeam is the social network for people with epilepsy and their loved ones. On MyEpilepsyTeam, more than 124,000 members come together to ask questions, give advice, and share their stories with others who understand life with epilepsy.

Do you have intractable epilepsy? Have you found a treatment that works for you? Share your experience in the comments below, or start a conversation by posting on your Activities page.