Epilepsy and Seizures: How to Treat? Dravet Syndrome

What are seizures and epilepsy?

Epilepsy is a condition which causes seizures.

A seizure is a sudden, uncontrolled electrical disturbance in the brain. It can cause jerking movements, changes in behavior, changes in feelings and may affect levels of consciousness. A seizure is usually a single episode. When there are two or more seizures or recurrent seizures, the condition is called epilepsy. Most seizures may not have a clear cause, while others can occur due to high fever, stroke, head injury, infection or inflammation of the brain, and major illnesses. Childhood seizure disorders like Dravet syndrome or Lennox-Gastaut are often a result of harmful genetic mutations, but not always.

Epilepsy is a neurological disorder where brain activities are abnormal, causing more than one or recurrent episodes of seizures.

What is the treatment for seizures and epilepsy?

Most cases of seizures can be managed conservatively with medication and supportive treatments. Surgery may be required to treat some cases of epilepsy. The treatment plan depends on the type and severity of seizure, age of the patient and the frequency of episodes. Many require lifelong treatment while others experience complete resolution. Children with epilepsy may outgrow the condition as they get older. Treatment of underlying medical conditions causing seizures is required.

• Medication: Antiseizure medications like valproic acid, carbamazepine, ethosuximide, etc. may be prescribed by the physician. Dosage would depend on the severity.
• Vagus nerve stimulation: A device is implanted beneath the skin of the chest that stimulates the vagus nerve in the neck. Stimulating the vagus nerve sends signals to the brain to inhibit seizures.
• Responsive neurostimulation: A device is implanted on the surface of the brain or within the brain tissue to detect abnormal brain activity and electrically stimulate the detected area to stop the seizure.
• Deep brain stimulation: Electrodes are implanted within certain areas of the brain and they produce electrical impulses that regulate abnormal brain activity.
• Diet: A diet rich in fat and low in carbohydrates, also called a ketogenic/keto diet, can improve seizure disorders.
• Surgery: The goal of surgery is to stop seizures from happening. Surgeons locate and remove the area of your brain where seizures begin. Surgery works best for people who have seizures that always originate from the same place in their brains.

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Pregnancy and seizures

Women with seizures/epilepsy can have healthy pregnancies. There is a risk of birth defects associated with certain antiseizure medications, especially valproic acid. The physician would prescribe appropriate medication and adjust the dosage during pregnancy.

Contraception and antiseizure medications

Certain antiseizure medications can decrease the efficacy of birth control pills. Hence, the doctor may recommend alternate methods of birth control.

What Causes Seizures in Young Children?

Childhood seizure disorders mostly have an underlying genetic cause, but some are not easily classifiable. Some childhood epilepsy syndromes are

• Doose syndrome (myoclonic-atonic epilepsy),
• Lennox-Gastaut syndrome,
• Dravet syndrome (severe myoclonic epilepsy of infancy)
• Benign neonatal myoclonic epilepsy
• Familial myoclonic epilepsy
• Autosomal dominant cortical myoclonus and epilepsies
• Unverricht-Lundborg disease
• Lafora body disease
• Myoclonic epilepsy with ragged red fibers (MERRF)
• The neuronal ceroid lipofuscinosis, sialidosis, and dentate-rubral-pallidoluysian atrophy (DRPLA).

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Lifestyle modifications

It is important to take medications as prescribed by the doctor alongside lifestyle modifications.

• Get adequate sleep: Inadequate rest/sleep can trigger seizures.
• Wear a medical alert bracelet.
• Get regular physical activity to help reduce stress and improve overall physical and mental health.
• Maintain adequate hydration.
• Manage physical and mental stress.
• Limit alcohol consumption.
• Avoid cigarette smoking.
• Avoid recreational drugs.
• Avoid places with bright and blinking lights, like nightclubs.

Personal safety measures

The following measures can help reduce the risk of injury during a seizure.

• Avoid swimming, being in a boat, etc. alone.
• Take showers instead of using a bathtub.
• Wear a helmet while riding or playing high-risk sports.
• Modify furniture by padding sharp edges; avoid low furniture like footstools to prevent tripping and carpet the floor to reduce the impact during falls.
• Carry or display seizure first-aid tips at home or work.
• Avoid wearing clothes that are tight around the neck.

Seizure first aid

The following steps can help a person during a seizure.

• Shout for help or contact the doctor immediately
• The person should be rolled onto their side.
• Something soft should be placed under their head.
• Any tight neckwear, scarves, etc. should be loosened.
• No objects or fingers should be placed in the mouth of a person having a seizure.
• Avoid holding or restraining a person having a seizure because it can cause injuries.
• Remove dangerous objects from around the person.
• Someone should closely observe the person during the seizure and stay with them until medical help arrives.
• The duration of the seizure should be recorded.

What are the symptoms of a seizure?

Most episodes of seizures last around 30 seconds to a couple of minutes. If they last longer than 5 minutes, it’s a medical emergency. The signs and symptoms are involuntary and the severity varies depending on the type of seizure.

Seizures are broadly classified into focal and generalized.

• Focal seizures: This is when there is abnormal brain activity in one particular part of the brain.
  • Focal seizures with loss of awareness/consciousness: This presents with blank staring and repetitive movements like chewing with loss of awareness/consciousness.
  • Focal seizures without loss of consciousness: This presents with a change in taste, smell, or emotions, jerking of the hands or legs, giddiness, and flashes of light.
• Generalized seizures: These seizures involve all areas of the brain. The following are different types of generalized seizures and their symptoms
  • Absence seizures (petit mal seizures): Staring blankly into space or body movements like smacking lips and loss of awareness.
  • Tonic-clonic seizures (grand mal seizures): This is the most dramatic type of seizures, with body stiffness, jerking, tongue biting, loss of bladder control, vomiting, and even loss of consciousness.
  • Myoclonic seizures: Sudden jerking or twitching of the arms and legs.
    • Dravet syndrome (formerly called Severe Myoclonic Epilepsy of Infancy (SMEI): is a rare, genetic form of epilepsy that begins in infancy characterized by prolonged and frequent seizures. Infants may also have behavioral and developmental delays, problems with movement and balance, speech problems and delayed language development, and growth issues. Mutations in SCN1A gene are seen in about 80% of infants with Dravet.
  • Atonic seizures (drop seizures): Loss of muscle tone, causing a fall.