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Epilepsy has been around since ancient times.

A Babylonian tablet that may date as far back as


2000 B.C. describes many of the seizure types that we see today, and associates each with a
different god. The ancient Greeks also thought that people with epilepsy were being "seized" by
the gods, so they called epilepsy "the sacred disease." In about 400 B.C., Hippocrates criticized
this view and wrote about epilepsy as a disorder of the brain that could in theory be treated with
diet and drugs. The ancient Ayurvedic medical system of India also describes epilepsy as a
physical disorder. Epilepsy is mentioned in the Bible; the book of Mark describes Jesus treating a
young man with tonic-clonic seizures, and mentions fasting as a treatment. However, until the
19th century A.D., when the first effective anti-epileptic drug was introduced, epilepsy was still
considered a supernatural disease, and people with epilepsy were treated with fear and suspicion.

Epilepsy is common and exists all over the world. The World Health Organization estimates that
approximately 50 million people in the world have epilepsy, and that it is a major cause of
disability, especially in young people.

It is thought that about 1 in every 100 to 200 people has epilepsy. One study found that in 1990,
about 47,000 people in Ontario had epilepsy. Among children under 12, about 1 in 320 had
epilepsy, while 1 in 175 children aged 12 to 15 had epilepsy.

In Canada, around 15,000 people are diagnosed with epilepsy each year. Epilepsy most often
starts either in childhood or in old age.

What happens in the brain when someone has a seizure?


The brain is made up of billions of neurons (nerve cells) working together to process input from
the senses and to control the body’s voluntary movements (like walking and talking) and
involuntary movements (like digestion and heartbeat). Each area of the brain is very specialized:
for instance, body movements are controlled by the neurons in a strip of the brain called the
motor cortex. One group of neurons controls the right hand, another group controls the left foot,
and so on.

Normally, neurons fire singly or in small, controlled groups. For instance, when you decide to
lift your hand, the neurons that send signals to the appropriate muscles fire in a controlled way --
like turning on a light switch -- and your hand moves.

When someone has a seizure, however, many neurons fire all together in an uncontrolled way.
Neighbouring neurons may be drawn in as well — like a small electrical storm in the brain.

Evoked Potentials, or EP, can help detect abnormalities of the central and peripheral nervous
system pathways. These abnormalities may not be clear from clinical symptoms or other tests.
The procedure is like an EEG; it involves sticking electrodes to the child's head or body and then
measuring the electrical activity of her brain in response to different stimuli.
There are three types of EP tests, depending on the part of the nervous system to be evaluated
and the type of stimuli used:

 Visual EP evaluates the child's visual nervous system. Electrodes are attached to the
child's head and she is shown a flashing light or a patterned screen for stimulation.
Younger children wear goggles.
 Auditory EP evaluates the child's auditory nervous system. Electrodes are attached to the
child's head and she is asked to listen to sounds through earphones.
 Somatosensory EP evaluates the sensory motor system of the body. Electrodes are
attached to the child's head to measure brain activity while mild electrical current is
applied to parts of her arms and legs. The electrical current is the stimulation.

EP tests may be part of the pre-surgical evaluation, or they may also be done during the
operation to ensure the surgery does not damage vital functional areas of the brain or spinal cord.

A functional MRI (fMRI) is used to identify the location of important functional areas of the
brain, such as those involved in motor function and language production or comprehension. This
information is used to determine the type of surgery that is best for your child and what areas of
the brain to avoid during the surgical procedure.

Functional MRI is a special type of MRI test. Conventional MRI takes a picture of the structure
of the brain, but fMRI identifies areas of brain function. The procedure combines scanning and
neuropsychological testing techniques to identify regions of brain function.

Functional MRI is done using the same scanner as an MRI. However, instead of just lying still or
sleeping in the scanner, your child will be asked to perform some special tests and answer
questions. The scan highlights areas of increased blood flow, indicating active areas of the brain.
Like the Wada test, the fMRI is used to identify where the areas associated with language are
located in the brain. Then the surgery can be planned to leave these areas intact.

Since the child's participation is necessary in this test, she cannot be sedated or sleeping for a
fMRI. The child needs to be old enough and mature enough to lie still for the scans and follow
the instructions. This test is usually used for older children and adults.

Intracarotid Amobarbital Test (Wada Test)


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Evaluation / Intracarotid Amobarbital Test (Wada Test)
This procedure is used to evaluate brain functions and identify which hemisphere of the brain is
essential for speech and memory functions. Then the surgery can be planned to leave these areas
intact and spare these important functions.

The Wada test is a neuropsychological test that is performed while half of the brain is asleep or
"frozen." During the test, the child will be asked to answer some questions and perform some
tasks. Since the child's participation is necessary in this test, he needs to be old enough and
mature enough to lie still for the test and follow the instructions. This test is usually used for
older children and adults.

The procedure is somewhat invasive: it involves the insertion of a catheter, or small tube, into a
blood vessel. This is done under local anaesthesia, so that the child is awake to follow the
instructions for the test.

The procedure is done in two parts. The first part is called an angiogram. During the angiogram,
a dye is injected into the catheter and watched via an X-ray machine as it passes through the
blood vessels. This is to evaluate the blood flow and the structure of the blood vessels in the
brain. In the second part of the test, a drug called sodium amobarbital is injected into the
catheter. This drug puts each half of the brain to sleep in turn, while the child is asked questions
to test his language and memory. This is done to see which side of the brain is dominant for
language and memory. For example, if your child is unable to speak while the left side of his
brain is asleep, this means that the left side of his brain is critical for language function.

Magnetic Resonance Imaging (MRI)


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Evaluation / Magnetic Resonance Imaging (MRI)

Neurological MRI - Sagittal View


MRI is a neuroimaging technique that can show details of the brain's structure. It shows structural
abnormalities in the brain and helps identify starting points for seizures.

The MRI machine scans the brain in "slices" to produce images. Each image shows a different
“slice” or level of the brain. They are also called cross-sectional images. These images or scans
are interpreted by a radiologist or a neurologist.

MRI does not use radiation or X-rays. Instead, a powerful magnet, radio signals, and a computer
are used to create the pictures. An MRI scan is fairly noisy but does not hurt. A series of short
scans make up the full MRI study which lasts about one hour. Each scan takes from one to five
minutes, during which time the child must stay still.

Magnetoencephalogram (MEG)
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Evaluation / Magnetoencephalogram (MEG)

MEG
A magnetoencephalogram (MEG) is a neuroimaging device that measures magnetic fields in the brain.
These magnetic fields are generated by the electrical activity of neurons (nerve cells) in the brain. An
MEG may be used in a similar way to an electroencephalogram (EEG) to identify abnormal electrical
signals in the brain and where they are coming from. An MEG is a functional imaging technique,
meaning that it looks at brain activity, not just brain structure.

An MEG is used to identify abnormal electrical discharges that produce magnetic signals in the
brain and, by mapping the sources of these discharges and comparing them with an MRI scan,
pinpoint the source of seizures. In presurgical evaluation, MEG is used to find and map the
motor, sensory, and language areas of the brain so that these areas can be avoided during surgery

Neuropsychological Assessment
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Evaluation / Neuropsychological Assessment

A neuropsychological assessment is a comprehensive evaluation of your child's thinking,


behaviour, and problem-solving skills. It consists of a series of tests and observations. The
specific tests done will depend on your child's age, development level, seizure history,
medication history, behaviour issues, and school performance. The tests will be done by a
neuropsychologist or by an assistant and interpreted by the neuropsychologist.
The neuropsychological assessment is done to:

 Understand the impact of your child's neurological condition on his cognitive functioning.
 Identify patterns of decreased cognitive function. This can help to identify the area of the brain
where seizures begin, by revealing areas of decreased brain function.
 Predict whether surgery will put your child at risk for language and memory problems.
 Help plan the best treatment for your child.
 Provide baseline information about your child's condition before the operation; later
neuropsychological assessments can be compared with this information.

A neuropsychological assessment is done before surgery. This is called a baseline study. Another
assessment is done after surgery to see the impact of surgery and identify areas where your child
would benefit from rehabilitation. The test may also be repeated at future times if any problems
emerge. It may also be done at important developmental transition points in a child's life, such as
going from junior high school to high school or from high school to post-secondary education, or
for job planning, if there has been a change in the child's functioning, or if up-to-date
information is required.

Elements of the neuropsychological assessment


The assessment will be tailored to your child's age, skill level, ability to cooperate, and the nature
of her seizure disorder. Not all tests are done on all children. The general skills that may be
tested are:

 Intelligence: Your child's overall knowledge, thinking, and problem-solving skills will be
tested.
 Perception: This will test how well your child's brain integrates information coming in,
such as visual perception. For example, your child may be asked to copy shapes.
 Motor function: This will test your child's muscle strength and control.
 Attention: Your child will be tested on her ability to focus on tasks that continue for some
length of time.
 Memory: Your child will be tested on how well she learns and remembers new
information. For example, the tester may tell your child a story and ask her questions
about it.
 Working memory: This is the ability to keep something in mind while doing another task.
This might involve, for example, taking directions to a location and finding it on a map.
 Language skills and vocabulary: This is the ability to understand and use language.
 Concept formation and problem solving: Your child will be asked to solve new problems
that she has not seen before.
 Planning and organization: The tester will look at how your child plans and organizes.
 Processing speed: Your child will be tested on how quickly she can think.
 Academic skills: The tester will look at how well your child can do the basics: reading,
writing, and math.
 Behaviour, emotions, and personality: The tester will consider whether your child is
depressed, anxious, or has any behaviour problems. This is based on what the tester sees
during testing as well as what you report on behaviour at home or school.
The baseline, or first, assessment usually takes a full day, with breaks for rest and lunch. The
length partly depends on your child's age and attention level and how fast she can work. It may
be necessary to have more than one appointment.

Tell your child that the testers want to find out how she thinks, learns, and remembers. You can
put your child at ease by telling her that there will be no injections or painful procedures. You
can explain the test to young children as answering questions, drawing, and playing with special
toys, like puzzles and blocks. Older children can think of it as taking a fun test at school. Parents
may also be asked for input and participation, especially with young children.

Positron Emission Tomography (PET)


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Evaluation / Positron Emission Tomography (PET)

PET is a neuroimaging scan that measures how intensely different parts of the brain use glucose,
oxygen, and other substances. Areas of decreased usage may indicate a site where a seizure
begins and therefore a possible place for surgery.

A small amount of radioactive substance will be inhaled (breathed in) or injected into your
child's bloodstream. The brain is then scanned to see how and where the substance is absorbed.
This procedure is similar to a SPECT scan.

What happens before the PET


Special preparation is required for this scan. Your child should not eat or drink anything for six
hours prior to the scan. Comfortable and warm clothing is recommended.

In Canada, the availability of PET remains quite limited because of government regulations. It is
because of these strict regulations that Canadian patients are required to sign a research consent
form before they can have a PET scan. We continue to collect data on children who have had a
PET scan to provide evidence that in selected cases of children with severe epilepsy, PET has a
role in the investigation of severe epilepsy, especially as part of epilepsy surgery.

Single Photon Emission Computed Tomography (SPECT)


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Evaluation / Single Photon Emission Computed Tomography (SPECT)

SPECT is a neuroimaging scan that measures the blood flow in the brain and thereby identifies
seizure areas. SPECT is based on the principle that blood flow to specific areas of the brain
changes when a seizure begins. By analyzing information about blood flow in the brain, seizure
sites can be identified.

The procedure is somewhat invasive in that it involves injecting a small amount of radioactive
substance into the patient's bloodstream, usually via a vein in the arm, and then scanning the
brain to see how and where the substance is absorbed. This procedure is similar to a PET scan.

Sometimes two SPECT scans are done, one during a seizure and one in the interval between
seizures, and the results are compared.

Video/EEG (Epilepsy Monitoring Unit)


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Evaluation / Video/EEG (Epilepsy Monitoring Unit)

This is a combination of two techniques: an electroencephalograph (EEG) measures the electrical


activity in the brain while a video camera captures what the seizures look like. The child is
admitted to the hospital and then continuously hooked up to the EEG machine and taped on
video. The admission can last for several days. This helps the doctors get a full picture of your
child's seizures — both the electrical activity of the brain and how your child looks and behaves
before, during, and after the seizures. The doctors can play the video back at half speed, freeze
the frame, and so on. This can help them detect subtle signs that may be missed at the time of the
seizure, and compare these signs with what is happening on the EEG at that moment.

Information from both sources is used to identify the type of seizures, including whether they are
epileptic or non-epileptic. If the seizures are epileptic, information from this test can help
pinpoint the epileptogenic region — the area in the brain where seizures begin.
The procedure requires your child to be hospitalized and monitored continuously for several
days. Before coming in for the test, she may need to stop taking her anti-epileptic medication so
that her seizures will return. The treatment team will explain how and when to taper off the
medication.

Your child will have electrodes attached to her head that will enable the EEG machine to record
her brain waves. She will stay in a special room equipped with cameras and microphones to
capture all her behaviours and sounds. She may have an IV inserted in case she quickly needs
medication during a seizure. Other parameters, such as heart rate, blood pressure, and oxygen
saturation, may also be tracked. A family member is usually required to be with the child at all
times.

What happens during the video/EEG


You should wash your child's hair before coming to the hospital. Please do not use any
conditioner or gel on your child's hair.

Your child's head will be measured and marked with a wax pencil so that the EEG technologist
knows where to put the small gold circles called electrodes. The marked areas on your child's
head will be cleaned with a special gel, which is a thick soap. Then electrodes will be put on
your child's head with cream and gauze. The electrodes are hooked up to the computer with long
wires. Sometimes the electrodes are in a rubber cap like a bathing cap.

Once the electrodes are attached, your child will stay in a special room with video cameras and
microphones where she will stay for the next several days. She can behave as usual: play, eat,
and sleep. She will be continuously monitored, awake and asleep, over the four days, making a
continuous record of your child's brain activity, or brainwaves, on a long strip of recording paper
or on a computer screen. This graphic record is called an electroencephalogram (EEG).
Simultaneously, the video cameras will be continuously recording her actions and sounds.

Intracranial EEG
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Evaluation / Intracranial EEG

When scalp EEG and neuroimaging scans do not provide enough information to perform
epilepsy surgery, it may be necessary to do a complicated and invasive procedure to record
intracranial EEG (using electrodes placed on the brain surface and within the brain) to get an
accurate reading of the brain's activity. Recording from intracranial electrodes is also called
electrocorticography or ECoG.

There are two types of intracranial EEG:

 One is called intrapoerative electrocorticography. In this type of ECoG, the electrodes are placed
on the brain during a surgical procedure to remove a lesion causing epilepsy and are removed at
the end of the surgery. This procedure is only done in the operating room.
 The other is called extraoperative monitoring. In this type of ECoG, the electrodes are placed in
or over the brain where the epileptic focus is thought to reside and are left in and on the brain
at the end of the operation. Recordings are then made from the intracranial electrodes while
the child is awake and alert, and during seizures. The electrodes are then removed at a later
date, usually in about four to five days.

Both types of intracranial EEG recordings require surgery to place the EEG electrodes. The
surgeon will open the skull and place electrodes on the surface of the brain in the subdural area,
over the first layer of membrane protecting the brain. Depth electrodes may also be placed into
deep areas of the brain using very thin wires. Intracranial EEG gives a more accurate reading
than scalp EEG, but this is also a complicated and invasive procedure compared to scalp EEG.
Results from intracranial EEG can finely pinpoint the epileptogenic region (the area in the brain
where seizures begin) so that it can be surgically removed or disconnected while also identifying
functional areas of the brain that should be left intact.

When is intraoperative ECoG used?


Intraoperative ECoG is used to guide the surgeon in removing that part of the brain that is
causing the epilepsy, particularly when the neurosurgeon is removing a lesion or brain
abnormality that is causing the seizures. In this case, the surgeon does the intraoperative ECoG
before removing the lesion, to define the extent of the brain causing the seizures, and then again
after the removal of the lesion to determine if any residual epileptic activity remains.

When is extraoperative monitoring used?


Extraoperative monitoring is done as preparation for epilepsy surgery in situations where non-
invasive techniques have failed to produce enough information to perform the surgery. For
example, this procedure might be used if:

 A surface EEG does not pinpoint the seizure site.


 Various scans and exams show conflicting information or seizures in more than one region of
the brain.
 Seizures arise from functionally vital areas of the brain.

Types of intracranial electrodes


There are three main types of intracranial EEG, using strip, grid, and depth electrodes.
 Strip electrodes are multiple electrodes attached to a strip and implanted in the subdural
cortical layer of the brain to record electrical activity. They are used when the region to be
studied is small.
 Grid electrodes are multiple electrodes attached to a rectangular grid and implanted in the
subdural cortical layer of the brain to record electrical activity. Grid electrodes are used to
evaluate larger surface areas.
 Depth electrodes, which look like a single thin wire, may be used to access structures deep
within the brain, such as the amygdala and the hippocampus.

Intracranial Electrodes

Preparing for extraoperative monitoring


Since it is a complex and invasive procedure, extraoperative monitoring is only done at medical
centers with significant expertise and experience, particularly when dealing with children.

Your child's neurologist and neurosurgeon will explain to you the entire procedure, the operation
itself, the following days of continuous EEG recordings, care issues, and the risks involved. You
should feel free to ask any questions you have.

The anaesthetist will discuss the procedure with you beforehand and inform you of the
preparations required. As with all cases of general anaesthesia, your child will be required to stop
eating eight hours before the surgery.

To reduce your child's anxiety, you can explain the procedure to your child in an age-appropriate
way, using the information given below. You can also ask your child's doctor or nurse to help
explain the procedure to your child.

Before your child comes in for the operation, she may need to stop taking her anti-epileptic
medication so that her seizures will return. The treatment team will explain how and when to
taper off the medication.

What happens during extraoperative monitoring


Extraoperative monitoring involves two stages. The first stage is an operation to implant the
electrodes within the brain. The second stage involves continuous EEG recordings from the brain
surface, usually over four to five days, to identify the origin of the seizures.

Stage 1: Implanting the electrodes

The first stage, the operation, begins with general anaesthesia to put the child to sleep. The next
step depends on the type of electrode being implanted:

 Strip electrodes are smaller and may be inserted through burr holes made in the skull.
 Grid electrodes are larger and require a craniotomy for placement. A craniotomy involves
cutting open a portion of the scalp and skull and peeling back the dura membrane to expose the
brain so the electrodes can be placed on the brain surface.
 For depth electrodes, the surgeon uses a thin wire to slide the electrodes through the brain to
reach the desired location.

Stage 2: Monitoring

The child will be monitored in the ICU or in a special video/EEG monitoring room with extra
nursing care. The child will stay in this special hospital room while her brain activity is
continuously monitored on the EEG and her activities are recorded on video. Her brain may also
be stimulated with mild electrical impulses via the electrodes to localize areas controlling speech,
movement, and sensation. All this should give an accurate assessment of seizures, epileptogenic
regions of the brain, and other vital information.

As with any surgery, there is a risk of infection and so antibiotics are usually given while the
electrodes are present in the brain. Another possible complication is cerebral edema or swelling
of the brain, related to the presence of the electrodes. If this occurs, the swelling may be treated
with steroids. If it is severe, the electrodes may be removed.

What happens after extraoperative monitoring


When the doctors have enough information about the origin of the seizures, they will be able to
accurately plan their treatment approach. If surgery is decided on, a second operation is done to
remove or disconnect the epileptogenic area of the brain and at the same time remove the
intracranial electrodes. If not, an operation is done only to remove the electrodes.

By combining the information from the seizure monitoring and the functional mapping, the team
can create a brain map that shows where the seizures are coming from and where important
function is located in the brain. In this way the team can tell which parts of the brain contributing
to the seizures can be safely removed. All of this information will be presented to you and your
child as well as the team's recommendation for a surgical strategy.

Elizabeth J. Donner, MD, FRCPC

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