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Emergency Management of Refractory Seizures: An Overview

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Drug refractory epilepsy means medications have not successfully controlled seizures despite trying at least two different drugs correctly.

Medically reviewed by

Dr. Prakashkumar P Bhatt

Published At February 13, 2024
Reviewed AtMarch 11, 2024

Introduction:

Epilepsy is a condition where people can have sudden, abnormal brain activity that causes seizures. These seizures can be very short or last a few minutes. Some individuals with epilepsy may experience what is called "refractory epilepsy," which means that regular medicines do not work well or not at all in controlling their seizures.

Depending on the type of seizures a person has, their treatment approach may vary. There are two main types:

  • Generalized seizures (affecting both sides of the brain).

  • Focal seizures (starting in a more minor part of the brain and potentially spreading).

Refractory epilepsy can significantly impact a person's life, causing difficulties at work or school, constant worry about when the subsequent seizure might occur, and even injuries due to the seizures. If a healthcare provider suspects someone of refractory epilepsy, they may recommend visiting a specialized medical center for epilepsy care.

What Are the Causes and Symptoms of Refractory Seizure?

Causes:

  1. Some seizures result from known causes like head injuries, infections, fevers, or brain tumors.

  2. However, the cause of seizures in epilepsy is often unknown.

  3. It is also unclear why some people with epilepsy do not find relief from anti-epilepsy medications.

  4. Sometimes, these medications stop being effective, possibly due to extremely high brain activity during seizures.

  5. The exact reasons behind these phenomena remain a mystery.

Symptoms:

  • Shaking or convulsions.

  • Passing out.

  • Confusion.

  • Tongue biting.

  • Lip smacking.

  • Uncontrolled eye movements.

  • Odd behavior like screaming and yelling.

  • Losing control of bowels or bladder.

  • Staring blankly.

  • Falling down.

  • Muscles getting very stiff.

If a person has seizures while on epilepsy medications, they might have refractory epilepsy.

What Are the Alternative and Potential Future Treatments for Epilepsy?

  1. Alternative Therapies: Some alternative treatments, like herbal remedies or traditional practices such as ayurvedic medicine, are being studied to see if they can help manage epilepsy. Behavioral therapies are also considered, like identifying and avoiding seizure triggers or using relaxation techniques like meditation.

  2. Current Medications: Although there are more epilepsy drugs available today, many patients still do not respond well to them. Newer drugs are designed based on understanding how epilepsy works in the brain, but they do not always work as expected. Some have fewer side effects but work similarly to older medications.

  3. Research Focus: Researchers are trying to find better treatments and ways to prevent epilepsy from developing in the first place (antiepileptogenesis). They believe finding reliable markers (biomarkers) for epilepsy could be a critical step. These markers could help identify people at high risk for epilepsy, assess the progression of the disease, and guide treatment decisions.

  4. Biomarkers: Biomarkers are like signals showing if epilepsy is developing or if someone has a high risk of seizures. These markers would make clinical trials for new treatments more efficient and less expensive. They could also help diagnose epilepsy faster, potentially even after a single seizure, and tailor drug treatment to each person's needs without trial and error.

  5. Surgical Guidance: Biomarkers could also help pinpoint the exact area in the brain where seizures originate, making it easier to plan and carry out surgical procedures to remove the problematic brain tissue.

  6. Future Research: While some biomarkers currently require invasive procedures to detect, future research may make it possible to identify them through non-invasive methods like scalp EEG (electroencephalogram) or brain imaging.

What Is the Emergency Supportive Treatment That Is Done for Epilepsy Patients?

When someone arrives at the emergency room while still having a seizure, they need immediate basic care.

  1. Airway and Breathing: Seizures can sometimes make it hard for a person to breathe, and low oxygen levels can worsen the seizure. So, first, the person's head and neck should be positioned to keep their airway open. If needed, any blockages should be removed. Oxygen can be given through a mask or small tubes in the nose. If breathing remains problematic, a tube may be placed in the windpipe (trachea) to help breathing. But, the most important thing is to stop the seizure with medication, which makes managing breathing much easier afterward.

  2. Glucose Levels: Low blood sugar (hypoglycemia) is an uncommon cause of prolonged seizures in children, but checking blood sugar levels is important. A sugar solution is given through a vein if the blood sugar is too low.

  3. Blood Pressure: Low blood pressure can make the situation worse. So, it is essential to keep the blood pressure at normal levels. If there is no sign of severe shock, a small amount of fluids is given through a vein to help maintain blood pressure.

Are There Any Medications That Can Be Used Successfully in Emergency Rooms to Stop Seizures?

Treatment Before Reaching the Hospital:

  1. Diazepam for Seizures: Diazepam is commonly used to stop seizures. It works well but can have breathing problems as a side effect, so it is usually given after arriving at the hospital, except for known cases.

  2. Prehospital Use of Diazepam: Some studies suggest that using Diazepam before reaching the hospital can be effective and safe. A study found that giving Diazepam before arriving at the hospital reduced seizure duration and the chances of having more seizures.

  3. Safety of Rectal Diazepam: Administering Diazepam through the rectum (bottom) is generally safe and rarely causes breathing problems in children.

First Hospital Treatment:

  1. Seizures in Hospital: If a child is still having a seizure when they arrive at the hospital, they will need immediate treatment because their seizure has likely lasted at least 10 minutes.

  2. Effectiveness of Diazepam: One dose of rectal Diazepam is usually effective in controlling seizures, and there is often no need for more unless the first dose does not work or if the child has another seizure.

  3. Alternative Medications: Other medications like Phenobarbitone, Phenytoin, and Lorazepam can also be used instead of Diazepam. Lorazepam is effective and has a longer-lasting effect than Diazepam, making it a good choice for some cases.

Why Refractory Seizures Are Challenging to Treat?

Difficult-to-Treat Seizures (Refractory Seizures):

  1. Defining Refractory Seizures: Refractory seizures do not respond to standard treatment with one or more anticonvulsant drugs.

  2. Traditional Approaches: In severe cases, doctors may use general anesthesia with a drug like Thiopentone, but it requires intensive care and has drawbacks. High doses of Phenobarbitone are another option.

  3. Midazolam as an Alternative: Midazolam is a newer option for difficult-to-treat seizures. It works quickly, can be given continuously, and has fewer drawbacks than other drugs. It is especially useful when other medications have failed.

  4. Midazolam Effectiveness: Midazolam has shown promise in controlling seizures even when other drugs have not worked. It might be considered earlier in treatment to avoid prolonged seizures.

  5. Use of Midazolam in the ER: In emergency rooms, intravenous and intramuscular Midazolam have been used effectively and safely to stop seizures.

How Surgical Techniques Improved the Accuracy and Effectiveness of Epilepsy Surgery?

  1. Who Benefits: Brain surgery is an option for people with refractory epilepsy, especially if their seizures originate from one specific area in the brain that is not critical for essential functions.

  2. Advancements: Advances in imaging and surgical techniques allow for more precise identification and removal of the seizure's source (seizure focus).

  3. Outcomes: Epilepsy surgery can lead to seizure freedom or significantly reduced seizure frequency for carefully selected patients.

Types of Epilepsy Surgery:

  1. Resective Surgery: This joint surgery involves removing the part of the brain where seizures originate, often seen in cases of temporal lobe sclerosis. It can result in seizure freedom for 48 % to 84 % of patients.

  2. Palliative Procedure: This surgery prevents seizure progression by interrupting nerve pathways. Corpus callosotomy, which disconnects the brain's two hemispheres, can reduce generalized tonic-clonic seizures by 80 % and other seizures by 50 %.

Other Treatment Options:

  1. Vagus Nerve Stimulation (VNS): VNS is a non-medication therapy for refractory partial seizures. It involves implanting a device connected to the vagus nerve under the skin. It helps reduce seizures in about 37 % of patients, with rare cases of complete seizure freedom.

  2. Ketogenic Diet: This diet, high in fat and low in carbs, has effectively treated refractory epilepsy. It may work by reducing neural excitability. However, it has potential complications like metabolic issues and should be carefully considered.

Conclusion:

Follow the doctor's instructions precisely when taking the anti-epilepsy medications. Properly using these medications can improve their effectiveness in managing the condition. There is also a special diet known as a ketogenic diet that might help in controlling the seizures. This diet is heavy on fats and low on carbohydrates. If someone decides to go on this diet, they must collaborate closely with their doctor and take necessary nutrient supplements.

Dr. Prakashkumar P Bhatt
Dr. Prakashkumar P Bhatt

Neurology

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