Ketogenic Diet for Epilepsy

The ketogenic diet for epilepsy (KDE) is a special diet that has helped many children and some adults achieve better (or even full) control of their seizures. It’s a first-line treatment for a few specific epilepsy syndromes, such as epilepsy due to mutations in GLUT-1 or pyruvate dehydrogenase deficiency.

Background

The ketogenic diet for epilepsy was developed in the 1920s by a Michigan doctor named Hugh Conklin. However, once effective medications were developed, the diet was used less and less frequently.2

It has regained recognition and has become a standard backup plan for children whose epilepsy symptoms are difficult to control with medication.1 With more than 470,000 children living with seizure disorders in the United States (according to Centers for Disease Control and Prevention statistics), it’s an important addition to the arsenal of treatments for epilepsy.

Researchers are also beginning to see how it might help adults with epilepsy and people with a variety of neurologic disorders.

What It Entails

The ketogenic diet for epilepsy is a very high-fat diet with just enough protein for body maintenance and growth, and very low amounts of carbohydrate.

When fats are broken down for energy, the body goes into what’s called a ketogenic state, in which the body generates molecules called ketones. The goal of the KDE is for the brain to use ketones for energy rather than glucose (sugar) as much as possible.

Ketones are (largely) water-soluble, so they are easily transported to the brain. The brain cannot use fatty acids for energy, but it can use ketones for a large portion of its energy requirements.1

The KDE is usually begun in a hospital setting and often begins with a one- to two-day fasting period, though there may be a trend away from both of these requirements.3

After determining the proper amount of protein (depending on age, etc.), the diet is structured as a ratio of fat grams to protein grams, plus carb grams. It usually begins with a 4 to 1 ratio and can be fine-tuned from there. The diet is often calorie and fluid-limited.4 Additionally, no packaged low-carb foods (shakes, bars, etc.) are allowed for at least the first month.

Because a gram of fat has more than twice the calories of a gram of protein or carbohydrate, this equation means that at least 75% of the calories in the diet come from fat. This is a very strict diet, and it takes time to learn how to put together meals that fit the formula. All food must be weighed and recorded.

Weaning off the diet is often attempted after two years, though some children are kept on it for longer.

Why It Works

Researchers are beginning to understand why the ketogenic diet works to lower seizure frequency. According to a 2017 review of studies, it appears that several mechanisms may be at work, including the following.

  • The diet appears to alter ketone metabolism in the brain in a way that enhances the brain’s ability to produce the neurotransmitter GABA, which has a calming effect on the brain.1
  • The diet has significant anti-inflammatory and anti-oxidative impacts, which appear to alter the way some genes involved in epilepsy are expressed.
  • Certain fatty acids featured in the diet have anticonvulsant effects and have even been shown to boost the effects of valproic acid—a common anti-seizure medication.
  • Polyunsaturated fatty acids in the diet may prevent brain cells from becoming overexcited.
  • Decanoic acid, which is part of the diet as well, appears to have a direct inhibitory reaction on the AMPA receptors in the brain. These receptors are believed to play a role in epilepsy and are the target of some epilepsy medications.
  • Effects on a key sensor of cellular energy appear to help prevent excessive firing of brain cells.
  • The diet may impact circadian activities and the expression of a growth factor in the brain in a beneficial way.5

Effectiveness

Studies generally show that about a third of children with epilepsy who follow the ketogenic diet will have at least a 90% reduction in seizures, and another third will experience a reduction of between 50% and 90%.46

This is remarkable, considering that these patients are generally those whose seizures are not well-controlled with medications.

In Adults

A growing number of studies have been done on the KDE and modified Atkins Diet in adults with seizure disorders, and the results are similar to studies with children.

One 2014 study reported that 45% of adolescent and adult participants saw a reduction of seizure frequency of 50% or greater. Tolerability appeared better in those with symptomatic generalized epilepsy.

Interestingly, it was more difficult to keep adults on the diet, since they obviously have more control over what they eat. Research is still limited in this area and more trials are needed.7

In Pregnancy

A 2017 report on use of these diets during pregnancy suggests they may be an effective way to control seizures and could possibly allow pregnant women to use lower doses of epilepsy medication. However, the safety of this still needs to be examined.8

Work With Your Medical Team

It is vital that anyone using this diet for a seizure disorder do it under the supervision of an experienced physician and dietitian. Many individual variations can influence the exact diet recommendations for each person, and coordinating this eating plan with medications can be tricky. It’s not something you should ever attempt on your own.

A Typical Day’s Menu

Below is a shortened description of a menu appearing in the 2015 Pediatric Annals article, “The Ketogenic Diet: A Practical Guide for Pediatricians.” It’s meant to give the idea of what children eat on the diet, not serve as an exact prescription. Remember, all of these foods are carefully weighed and measured.

  • Breakfast: Eggs made with heavy cream, cheese, and butter; small serving of strawberries, pineapple, or cantaloupe
  • Lunch: Hamburger patty topped with cheese; cooked broccoli, green beans, or carrots with melted butter; whipped heavy cream
  • Dinner: Grilled chicken breast with cheese and mayonnaise; cooked vegetables with butter; whipped heavy cream
  • Snacks: Whipped heavy cream, small servings of fruit, sugar-free gelatin

Variations substitute coconut oil or MCT oil for some of the heavy cream and butter.

Eating While at School

With a school-aged child, keeping them on the diet during the school day is difficult but essential. Thinking and planning ahead can help you be successful. You may want to try some of the following strategies:

  • Talk to your child: Make sure your child understands the diet and why sticking to it is essential. Let them know they shouldn’t trade food with other kids. As hard as it is, they also shouldn’t eat food from vending machines or treats handed out in class.
  • Talk to the school: The teacher, guidance counselor, nurse, and administration all need to be aware of your child’s special dietary needs (as well as other health-related matters). You’ll want to have regular conversations with them, and you may want to have a 504 plan or individualized education plan (IEP) in place as well.
  • Become a planner: Gather several recipes for appropriate meals that can make convenient, easy-to-pack lunches. If possible, you may want to provide appropriate treats for your child for holiday parties and other special events that you may know about ahead of time. The Charlie Foundation and Clara’s Menu are good resources for child-friendly keto recipes.
  • Educate family members: It’s important that family members and any regular caregivers know how to prepare a meal for the child with epilepsy.
  • Establish routines: The timing of meals and snacks needs to be consistent in order for your child’s glucose levels to remain as stable as possible. You may need to work with your child’s teacher(s) on this.
  • Involve a friend: Having a friend at school who understands the importance of your child’s diet may help them feel less awkward about being “different” and give them someone to lean on for support when needed. Make sure your child is OK with this and give them input on which friend to choose.

You’ll also want to make parents of your child’s friends aware of the special diet and that what some people may consider “a little harmless cheating” may not be harmless at all. It’s a good idea to provide food for your child to take to parties and playdates.How to Raise Kids Who Are on a Special Diet

Alternatives to the Super-Strict Ketogenic Diet

The Modified Atkins Diet is a popular alternative that helps many who find the ketogenic diet too difficult to adhere to. This diet is far less restrictive, as calories, fluids, and protein are not measured.9

The diet begins with 10 grams of carbohydrate per day for the first month, which slowly increases to 15 or 20 grams. It is similar to the very strict induction phase of the standard Atkins diet.

One 2014 study reported that 45% of adolescent and adult participants saw a reduction of seizure frequency of 50% or greater. Tolerability appeared better in those with symptomatic generalized epilepsy.

Nocturnal Seizures

Overview

An epileptic seizure is caused by unusual electrical activity in the brain. This usually causes to muscles of the body to tighten or weaken temporarily.

Nocturnal seizures happen when a person is sleeping. They are most common:

  • Right after falling asleep
  • Just before waking up
  • Soon after waking up

Any seizure can occur during sleep. However, there are certain seizure conditions that are more likely to experience nocturnal seizures, including:

Symptoms

Nocturnal seizures are often unnoticed because the patient is asleep when they happen. However, there are signs that may suggest the patient’s is experiencing these seizures, including:

  • Loss of bladder control
  • Biting their tongue

If the patient notices these symptoms of interrupted sleep they should talk to their doctor:

  • Difficulty concentrating
  • Awaking suddenly for an unknown reason
  • Feeling drowsy
  • Increase in daytime seizures triggered by a lack of sleep

Involuntary muscle movement linked to seizures can include:

  • Stiff arms
  • Stiff legs
  • Jerking body movements

Causes and Risk Factors

The cause of seizures is often unknown. However, there are certain seizure conditions that are more likely to experience nocturnal seizures, including:

Seizures can be linked to:

Diagnosis

The most useful way to diagnose the type of seizure is an electroencephalogram (EEG). This records electrical activity in the brain. The EEG can record unusual spikes or waves in electrical activity patterns. Different types of seizures can be identified with these patterns.

When a patient is experiencing nocturnal seizures, a sleep study is often needed to diagnose the condition. The sleep study will require the patient to stay overnight at the hospital where their brain activity can be monitored during their sleep.

Magnetic resonance imaging (MRI) and computed tomography (CT) scans may be used to look at where the seizures are happening in the brain. The scans can show scar tissue, tumors or structural problems in the brain.

Treatment

For many patients, correct treatment can lower or prevent seizures. In some cases, patients may not have any more seizures for the rest of their life.

Treatment is based on:

  • The type of seizure
  • Any underlying conditions
  • How often seizures happen
  • How severe the seizures are
  • The patient’s age
  • The patient’s overall health
  • The patient’s medical history

Anti-seizure (or anti-epileptic) medications can be very helpful. It may take a few tries to get the right medicine and dose. The doctor will watch for side effects to find the best treatment.

Surgery may be an option if medication can’t control the seizures. A vagus nerve stimulator (VNS) is sometimes implanted and used with anti-epileptic medication to lower seizures. The VNS is a tool placed under the skin of the chest. It sends electrical energy through the vagus nerve into the neck and up to the brain.

Certain lifestyle changes may also be used:

  • Special high-fat, low-carbohydrate diet (ketogenic diet)
  • Getting plenty of sleep
  • Avoiding certain triggers, such as lack of sleep

The staff at the Cedars-Sinai Epilepsy Program will work with each patient to determine the best treatment option.

My Best Friend

A friend of mine on Facebook came across this poem on the Epilepsy Society group page.

I have a best friend She lives in my brain

She takes me away again and again

She makes my head foggy And makes me confused

We always argue and I always loose

She turns out the lights And makes it all dark

She takes all my memories and my spark

She hurts me a lot She can make me bleed

When she’s with me I don’t know where life will lead

She takes away my breath And tries to stop my heart

From my life she tries to make me part

It makes me sad That she lives in my head

She won’t even stop when I’m asleep in bed

Sometimes she’s loud Others she’s quiet

But not for to long as she rules my empire

She has broken bones She has broken skin

Our life long fight and I never win

She makes me nervous When we go out in public

I wish she wouldn’t I wish she would stop it

She is badly behaved And out out of control

Sometimes my own personal Facebook troll

She makes me sick Makes me poorly

She pushes away those who adore me

She likes us alone Just her and I

We’ve been together since I was Nine

I’ve tried to contain her With medication and such

But she brakes out of her cage and makes me feel rough

She hates flashing lights And hates exercise

From a lot of things she makes me hide

She hates my emotions And she hates a pub drink

But that last one isn’t such a bad thing She won’t let me drive

She won’t let me dance

And if I disobey her she will put me in a trance

She makes my world cloudy

There is no middle ground

There is never an up side there is only a down

Like a never ending spiral That goes around and round

I feel like I’m lost never to be found

To talk to someone about epilepsy, our helpline team offer information and emotional support to anyone affected by the condition. You can call them on 01494 601 400, Monday – Friday 9-4pm and Wednesday 9-7.30pm. or contact https://www.epilepsyscotland.org.uk/ or call https://www.epilepsy.org.uk/

Epilepsy update

Hi Folks today I’m here to give you an update on my seizures.

First of all let me say this is the first seizure of any kind in one week short of two months.

From my point of view given that my last seizure was on twenty eight of July when I had the petite mal seizure at 11.30am approximately, it came unexpectedly.

At first everything seemed ok after the seizure, until about 10 minutes later I got a what I call (embarrassing side effect) in the form of a bowel movement. Embarrassing in the sense that if you are in a public place and don’t know where the toilets are.

I usually have an idea what triggers my seizures, mostly it’s stress related, but this time I haven’t a clue what triggered it.

My seizures have been known to be more frequent, in fact I noticed during the first lockdown from Covid – 19 I was less stressed and if I remember right no seizures and that is still the case today.

So I reckon what was causing the increase in seizures was something I had to stop doing during Covid -19.

Seizures & Nightmares

I don’t normally suffer from either of these during the night. last night I suffered 2-3 of them.

Normally when I have a nightmare I can usually remember it in the morning but I couldn’t remember what happened (if it was a nightmare) except for a brief moment of being awake in each case then falling asleep again.

If it were a siezure I only suffer from Focal Partial and Simple Partial which would normally occur during the day.

Finally I have to admit I have been under a bit of stress forr various and personal reasons

So were they Seizures or nightmares I took last night?

Please drop a comment in the box below

Ohtahara syndrome

This is a very rare epilepsy syndrome. Seizures start before 3 months of age. Many babies have an underlying structural brain abnormality or a metabolic (biochemical) disorder. This may be genetic in origin (passed on through the genes), or happen because of brain damage before or around the time of birth. A number of gene abnormalities, called mutations, may also cause Ohtahara syndrome. These include STXBP1, ARX, CDKL5 and PNKP. Other mutations will almost certainly be found in the future. However, no cause will be found in some infants. 

It is one of the group of severe epilepsy syndromes that start in the first year of life and are called the ‘Developmental and epileptic encephalopathies’.

This syndrome is quite similar to another developmental and epileptic encephalopathy called ‘Early myoclonic encephalopathy’.

Other names for Ohtahara syndrome

• Early infantile epileptic encephalopathy (EIEE) with burst-suppression

Symptoms

Seizures usually start in the first 7 to 10 days of life. Sometimes the mother may realise her baby was having seizures during the last couple of months of her pregnancy, when she sees her baby having seizures after birth.

Different types of seizures happen in this syndrome. The most common type is a tonic spasm where the baby suddenly has stiffening of the limbs, which last between 2 and 6 or more seconds. These tonic spasms may be quite subtle or quite severe. They are not the same as the epileptic spasms (infantile spasms) that occur in West syndrome. Children with Ohtahara syndrome also have focal (partial) motor seizures and myoclonic seizures with jerking of one limb, or one side of the body. These may last a few seconds to several minutes. Generalised seizures may happen later. After between 2 and 7 months, the seizure type may change to infantile spasms and the child may then develop West syndrome.

Diagnosis

A full account and description of what happens during the seizures is important for the doctors to make the diagnosis. It is always helpful if you can record a video of some of the seizures and show these to the hospital doctor.

The EEG test, which records the electrical activity in the brain, is always abnormal, showing evidence of abnormal discharges, or spikes and waves (the ‘burst’) alternating with periods of looking flat and featureless (‘suppression’). This type of record is referred to as ‘burst-suppression’ pattern. 

Epilepsy Action has more information on diagnosis.

Treatment

The seizures are often resistant to epilepsy medicines. The choice of medicine is difficult, but often some of the older medicines, such as phenobarbital, are tried first. Other medicines that may be effective include levetiracetam (Keppra), clobazam (Frisium), clonazepam (Rivotril), vigabatrin (Sabril) and zonisamide (zonegran). Other medicines such as pyridoxine and pyridoxal phosphate and a course of steroids called prednisolone may also be effective in a small number of children. Some babies with a focal (in one area only) structural brain abnormality seen on a magnetic resonance imaging (MRI) scan may be able to have epilepsy surgery to remove the abnormal area of brain. It may be possible to reduce the numbers of seizures if the baby is found to have a treatable metabolic (biochemical) disorder.

If your child has this syndrome it is very likely they will have prolonged or repeated seizures that continue over many minutes or even hours. Your child’s doctor will discuss a ‘rescue’ or emergency care plan with you to treat any prolonged or repeated seizures.

There may be research studies open that are relevant to this epilepsy syndrome. Your child’s doctor will be able to discuss this with you.

Information about treatments for children can be found on the Medicines for Children website.

Outlook

Affected babies do not behave normally. They are very floppy and excessively sleepy (described as an encephalopathy) and often have difficulty with feeding. With time they may develop stiffness (spasticity) in the limbs. They also make very little developmental progress and remain totally dependent. They often feed poorly. Sadly, they often die within the first two years of life, because of complications, including repeated chest infections. Those who do survive are usually severely disabled and will continue to have seizures despite treatment. Many babies go on to develop West syndrome, usually between 3 and 9 months of age.

Support

Contact
Charity for families of disabled children.
Freephone helpline: 0808 808 3555
Website: contact.org.uk
Email: helpline@contact.org.uk

Together for Short Lives
Helpline: 0808 8088 100
Website: togetherforshortlives.org.uk

What is a syndrome?

A syndrome is a group of signs and symptoms that, added together, suggest a particular medical condition. In epilepsy, examples of these signs and symptoms would be things like the age at which seizures begin, the type of seizures, whether the child is male or female and whether they have physical or learning disabilities, or both. The results of an electroencephalogram (EEG) are also used to help identify epilepsy syndromes.

If you would like to know more about an epilepsy syndrome, please speak to your doctor. If you would like to know more about epilepsy in general, please contact the Epilepsy Action Helpline.