Epilepsy is a neurological disorder that causes recurrent seizures. A seizure is a sudden, abnormal change in the electrical activity of the brain. This causes temporary symptoms such as muscle twitching, loss of consciousness, or a blank stare.
Antiepileptic drugs (AEDs) are the first line of treatment. But for some people, AEDs are unable to control their seizures. This is called refractory epilepsy.
Other names for intractable epilepsy include:
- drug resistant epilepsy
- rebellious epilepsy
- drug-resistant epilepsy
In refractory epilepsy, seizures continue even with AEDs. Of course, it can be frustrating and stressful.
To learn more about the condition, read on. We’ll explore the potential causes of intractable epilepsy, along with treatment options and prospects.
Refractory epilepsy occurs when AEDs do not improve the severity or frequency of seizures. He is diagnosed after trying at least two AEDs (alone or together) without positive results.
As a result, the condition is often characterized by frequent medication changes.
Refractory epilepsy can appear in several ways:
- You get medicine for epilepsy, but it doesn’t work.
- Your existing medicine, which used to control your seizures, stops working.
- You have serious side effects from AEDs, which makes it difficult to continue treatment.
The exact cause of intractable epilepsy is unknown. However, there are a few theories behind the condition:
- Pharmacokinetic hypothesis. Efflux transporters are proteins that move toxic substances out of cells. This hypothesis proposes that the efflux transporters in the organs are overactive and reduce the levels of AED in the body, which decreases the effectiveness of the drugs.
- Neural network hypothesis. This theory says that seizures suppress the body’s natural antiepileptic network and prevent AEDs from reaching the right neurons.
- Intrinsic gravity assumption. In this hypothesis, we say that severe forms of epilepsy are more resistant to AEDs.
- Genetic variant hypothesis. This hypothesis claims that genes related to the transport of AEDs in the body lead to drug resistance.
- Target hypothesis. AEDs work by targeting specific pathways and receptors in the brain. This hypothesis states that changes in these targets reduce the effects of drugs.
- Carrier assumption. This hypothesis proposes that efflux transporters in the blood-brain barrier are overactive, reducing the amount of drug entering the brain.
Sometimes a person’s seizures can seem refractory even though they actually are not. This is called apparent drug resistance.
In this case, the drugs cannot control the seizures for the following reasons:
- incorrect use
- wrong dosage
- other drugs cause interactions, decreasing effectiveness
- non-epileptic condition causing seizures
- misdiagnosis of seizure type or epileptic syndrome
- lifestyle factors, such as illegal drug use or stress
Refractory epilepsy causes seizures despite taking anti-epileptic drugs. The symptoms of these seizures are the same as those for seizures in general.
Possible symptoms include:
- loss of consciousness or consciousness
- loss of bladder or bowel control
- blank stare
Your doctor will use several methods to diagnose refractory epilepsy:
- Medical background. Since intractable epilepsy involves frequent medication changes, your doctor will ask you about any medications you have taken.
- Electroencephalogram. An electroencephalogram (EEG) measures the electrical activity of your brain. This helps your doctor to identify abnormal electrical patterns.
- Imaging scans. Imaging, such as a CT scan or MRI, can show where the seizures are occurring in your brain.
The goal of treatment for intractable epilepsy, like epilepsy in general, is to manage seizures.
Treatment options include:
Changing antiepileptic drugs
Your doctor may recommend that you take another AED on its own or with another medicine. Here are some examples of AEDs:
However, if you’ve taken two AEDs in the past without positive results, another AED is unlikely to work. This may be due to the way your brain or body interacts with AEDs. In this case, you will need to try other treatments.
During brain surgery for epilepsy, your surgeon removes the part of the brain where the seizures occur.
Surgery can be divided into two categories:
- Curative. The goal is to get rid of seizures.
- Palliative. The aim is to reduce the number and severity of seizures.
The following are examples of curative procedures used for epilepsy:
- anterior temporal lobectomy
- lensionectomy (used for tumors, cortical malformations, venous malformations; the surgeon will remove a lesion believed to be causing the seizures)
The following are examples of palliative procedures used for epilepsy:
- body callosotomy
- multiple subpial section
Your doctor can determine if brain surgery is right for you. Success rates for surgery – seizure removal – depend on the type of surgery you have had, but can range from 50 to
Vagus nerve stimulation
Vagus Nerve Stimulation (VNS) uses a device to simulate your vagus nerve, which can improve seizures. The device is implanted under your skin in your chest.
VNS can cause side effects such as:
- difficulty in breathing
Other neurostimulation devices include:
- reactive neurostimulation (RNS), which involves an electrical generator implanted in the skull
- deep brain stimulation (DBS), where an electrode is implanted in the brain and a stimulator is implanted under the skin of the chest
A doctor may recommend following an anti-epileptic diet such as the Modified Atkins Diet or the Ketogenic Diet. The more restrictive keto diet is commonly prescribed for children who do not respond to AEDs.
These diets can be difficult to follow successfully because they often require accurate measurement of fats and carbohydrates.
If you’re on a low-carb diet for epilepsy, be sure to work with a registered dietitian. They can help you get the nutrients you need.
Side effects of an anti-epileptic regimen can include upset stomach and constipation.
In addition to the above treatments, it is important to minimize seizure triggers.
Common triggers include:
- lack of sleep
- mental and emotional stress
- overstimulation, like bright lights
If AEDs don’t help your seizures, see a doctor. They might suggest a different dosage or medication.
See a doctor if you have:
- worsening of seizures
- more frequent seizures
- seizures longer than usual
- loss of consciousness for a long time
- difficulty waking up after a seizure
The outlook for intractable epilepsy varies from person to person.
Generally, you can expect a better outlook when refractory epilepsy is diagnosed early. This is because it can take a long time to find effective alternative treatments when AEDs don’t work.
To improve your long-term outlook, see your doctor regularly and follow their instructions. Take note of your seizure triggers and do your best to avoid them.
This can help prevent or minimize long term effects, such as:
- a permanent disability
- cognitive problems
- dependence on family and friends
- reduced quality of life
Trying different treatments can be expensive, even if you have health coverage. If you need help, talk to your healthcare professional. They might be able to suggest resources or organizations that provide financial assistance.
Refractory epilepsy can affect your physical, emotional, and mental health. It’s normal. If you need help, go to an epilepsy center. These centers often have support services that can help you improve your outlook.
If AEDs are unable to control your seizures, it is called refractory epilepsy. It occurs when AEDs don’t work, stop working, or cause side effects that make it difficult to keep taking the drug. There are some theories as to why this happens, but the exact cause is unknown.
The goal of treatment is to find effective alternative therapies. This can include nerve stimulation, brain surgery, or lifestyle changes. Your doctor may also suggest additional medications or different doses.
If AEDs don’t improve your seizures, ask to be referred to a comprehensive epilepsy center where healthcare professionals have special training in diagnosing and treating refractory epilepsy.
The outlook is generally better when intractable epilepsy is diagnosed early.