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ce4less.com ce4less.com ce4less.com ce4less.com ce4less.com ce4less.com 1 EPILEPSY Jassin M. Jouria, MD ABSTRACT Epilepsy is a seizure disorder of varied etiology and symptomology and its treatment depends on multiple factors, including age of onset and type of seizure. Sometimes the seizure is absent or mild enough to go untreated by medication and resolves over time. Most often, epilepsy is a life long condition that requires close medical management. Anti-epileptic drug therapy often requires serum monitoring for dose adjustment and drug interaction surveillance. Screening for comorbid medical and psychiatric conditions, especially depression, anxiety, and feelings of social stigma and isolation is needed. Educating patients and families to increase awareness of epilepsy and treatment options in their unique circumstance will assist them to overcome stereotypes and help them obtain a higher quality of life. Introduction Epilepsy is a complex brain disorder that is characterized by seizures, which are caused by disturbances in the brain’s electrical functions. The term epilepsy encompasses a variety of different neurological syndromes, each ranging in its symptoms, severity, and duration. The characteristic seizures are present in all types of epilepsy, but they differ in clinical presentation and symptom severity depending on the type of epilepsy. Epilepsy is most common in young children and the elderly, but it can affect individuals of all ages. In many cases, the cause of epilepsy is unknown. In those instances when a cause is identified, we find that the cause varies

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EPILEPSY

Jassin M. Jouria, MD

ABSTRACT

Epilepsy is a seizure disorder of varied etiology and symptomology and its

treatment depends on multiple factors, including age of onset and type of

seizure. Sometimes the seizure is absent or mild enough to go untreated by

medication and resolves over time. Most often, epilepsy is a life long

condition that requires close medical management. Anti-epileptic drug

therapy often requires serum monitoring for dose adjustment and drug

interaction surveillance. Screening for comorbid medical and psychiatric

conditions, especially depression, anxiety, and feelings of social stigma and

isolation is needed. Educating patients and families to increase awareness of

epilepsy and treatment options in their unique circumstance will assist them

to overcome stereotypes and help them obtain a higher quality of life.

Introduction

Epilepsy is a complex brain disorder that is characterized by seizures, which

are caused by disturbances in the brain’s electrical functions. The term

epilepsy encompasses a variety of different neurological syndromes, each

ranging in its symptoms, severity, and duration. The characteristic seizures

are present in all types of epilepsy, but they differ in clinical presentation

and symptom severity depending on the type of epilepsy.

Epilepsy is most common in young children and the elderly, but it can affect

individuals of all ages. In many cases, the cause of epilepsy is unknown. In

those instances when a cause is identified, we find that the cause varies

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between environmental or genetic factors, or as part of traumatic injury.

Some epileptic syndromes will only last a short time, especially those caused

by trauma; however, some other epileptic syndromes will be lifelong

conditions that cannot be cured.

While many individuals will experience a single, unprovoked seizure at some

point in their lives, epilepsy is not considered as a diagnosis until the patient

has had two or more unprovoked seizures. Once this occurs, the patient will

begin the process for assessing and diagnosing the type of epilepsy.

Overview Of Epilepsy

Epilepsy affects the central nervous system, thereby causing disruptions in

the nerve cell activity in the brain. When this activity is disrupted, seizures

occur (1). These seizures will cause the patient to experience abnormal

behavior, symptoms, and sensations. In some instances, patients will lose

consciousness. The presentation of seizures will vary. Some patients will

stare blankly for a brief period of time, typically a few seconds. Other

patients may experience twitching and jerking of their bodies (2). The type

of seizure experienced by the patient depends upon the etiology and the

severity of the condition.

Regardless of the severity of the seizures, most patients will require

treatment, as seizures can pose a significant risk to the patient. Seizures

can occur when the patient is engaging in activities such as driving,

operating machinery, or swimming, When this occurs, the patient is at an

increased risk of experiencing significant injuries (3).

Specific symptoms and features typically define epileptic syndromes. The

categories include: (4)

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Seizure types

Age when seizures begin

Electroencephalogram (EEG) findings

Brain structure (usually assessed with a brain MRI scan)

Family history of epilepsy or genetic disorder

Prognosis (future outlook)

Approximately fifty percent of epilepsy cases are caused by unknown

factors. In the remaining cases, the causes are typically genetic,

environmental, or trauma related (5).

The following table provides an explanation of the potential cause in cases

where the cause of epilepsy may be identified (6):

Genetic

Influence

Some types of epilepsy, which are categorized by the type of seizure

the individual experiences, run in families. In these cases, it's likely

that there's a genetic influence.

Researchers have linked some types of epilepsy to specific genes;

though it's estimated that up to 500 genes could be tied to the

condition. For most people, genes are only part of the cause of

epilepsy. Certain genes may make a person more sensitive to

environmental conditions that trigger seizures. Generalized epilepsy

seizure types appear to be more related to genetic influences than

partial seizure epilepsies.

Head Trauma Head trauma that occurs due to a car accident or other traumatic

injury can cause epilepsy. Head injuries can cause epilepsy in both

adults and children, with the risk highest in severe head trauma. A

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first seizure related to the injury can occur years later, but only very

rarely. People with mild head injuries that involve loss of

consciousness for fewer than 30 minutes have only a slight risk that

lasts up to 5 years after the injury.

Brain conditions - Brain conditions that result in damage to the brain,

such as brain tumors or strokes, also can cause epilepsy. Stroke is a

leading cause of epilepsy in adults older than age 35.

Infectious

Diseases

Infectious diseases, such as meningitis, AIDS and viral encephalitis,

can cause epilepsy.

Prenatal injury Before birth, babies are sensitive to brain damage that could be

caused by several factors, such as an infection in the mother, poor

nutrition or oxygen deficiencies. This brain damage can result in

epilepsy or cerebral palsy.

Developmental

Disorders

Epilepsy can sometimes be associated with developmental disorders,

such as autism and neurofibromatosis.

Brain

Chemistry

Factors

Ion Channels - sodium, potassium, and calcium - act as ions in the

brain. They produce electric charges that must fire regularly in order

for a steady current to pass from one nerve cell in the brain to

another. If the ion channels that carry them are genetically damaged,

a chemical imbalance occurs. This can cause nerve signals to misfire,

leading to seizures. Abnormalities in the ion channels are believed to

be responsible for absence and many other generalized seizures.

Neurotransmitters - Abnormalities may occur in neurotransmitters, the

chemicals that act as messengers between nerve cells. Three

neurotransmitters are of particular interest:

Gamma aminobutyric acid (GABA), which helps prevent nerve

cells from over-firing.

Serotonin's role in epilepsy is also being studied. Serotonin is a

brain chemical that is important for well-being and associated

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behaviors (such as eating, relaxation, and sleep). Imbalances

in serotonin are also associated with depression.

Acetylcholine is a neurotransmitter that is important for

learning and memory.

Risk Factors

Epilepsy and seizure disorders affect nearly 3 million Americans and more

than 45 million people worldwide. While anyone can develop epilepsy, there

are a number of factors (outlined below) that will increase an individual’s

risk of developing epilepsy and seizure disorders (7).

Age factor:

Epilepsy affects all age groups. The risk is highest in children under the age

of 2 and older adults over age 65. In infants and toddlers, prenatal factors

and birth delivery problems are associated with epilepsy risk. In children age

10 and younger, generalized seizures are more common. In older children,

partial seizures are more common.

Gender factors:

Men have a slightly higher risk than

women of developing epilepsy.

Family History:

People who have a family history of

epilepsy are at increased risk of

developing the condition.

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While there are numerous factors that may cause epilepsy, as well as a

variety of epileptic syndromes, all types share one common feature: all

forms of epilepsy are characterized by recurrent seizures (1). These seizures

are caused by uncontrolled electrical discharges in the nerve cells in the

cerebral cortex. Many individuals will experience a single seizure at some

point in their lifetime. This is not considered epilepsy (3).

Very few initial seizures will recur. In fact, only approximately twenty five

percent of initial seizures will recur (8). Once a patient experiences two or

more recurring seizures, he or she has a 70 % chance of experiencing

recurring seizures. This will result in

a diagnosis of epilepsy.

Epilepsy is generally classified into

two main categories based on seizure type, and these are described in the

table below (9):

PARTIAL SEIZURES

These seizures are more common than generalized seizures and occur in one or

more specific locations in the brain. In some cases, partial seizures can spread

to wide regions of the brain. They are likely to develop from specific injuries, but

in most cases the exact origins are unknown (idiopathic).

Simple Partial

Seizures

A person with a simple partial seizure (sometimes known as

Jacksonian epilepsy) does not lose consciousness, but may

experience confusion, jerking movements, tingling, or odd

mental and emotional events. Such events may include déjà

vu, mild hallucinations, or extreme responses to smell and

taste. After the seizure, the patient usually has temporary

weakness in certain muscles. These seizures typically last

about 90 seconds.

Complex Partial

Seizures

Slightly over half of seizures in adults are complex partial

type. About 80% of these seizures originate in the temporal

(Photo Courtesy of:

http://myqigong.blogspot.com/2011/01/epilepsy-

seizure.html)

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lobe, the part of the brain located close to the ear.

Disturbances there can result in loss of judgment, involuntary

or uncontrolled behavior, or even loss of consciousness.

Patients may lose consciousness briefly and appear to others

as motionless with a vacant stare.

Emotions can be exaggerated; some patients even appear to

be drunk. After a few seconds, a patient may begin to perform

repetitive movements, such as chewing or smacking of lips.

Episodes usually last no more than 2 minutes. They may occur

infrequently, or as often as every day. A throbbing headache

may follow a complex partial seizure. In some cases, simple or

complex partial seizures evolve into what are known as

secondarily generalized seizures. The progression may be so

rapid that the initial partial seizure is not even noticed.

GENERALIZED SEIZURES

Generalized seizures are caused by nerve cell disturbances that occur in more

widespread areas of the brain than partial seizures. Therefore, they have a more

serious effect on the patient. They are further subcategorized as tonic-clonic (or

grand mal), absence (petit mal), myoclonic, or atonic seizures.

Tonic-Clonic

(Grand Mal)

Seizures.

The first stage of a grand mal seizure is called the tonic phase,

in which the muscles suddenly contract, causing the patient to

fall and lie stiffly for about 10 - 30 seconds. Some people

experience a premonition or aura before a grand mal seizure;

most, however, lose consciousness without warning. If the

throat or larynx is affected, there may be a high-pitched

musical sound (stridor) when the patient inhales. Spasms

occur for about 30 seconds to 1 minute. Then the seizure

enters the second phase, called the clonic phase. The muscles

begin to alternate between relaxation and rigidity. After this

phase, the patient may lose bowel or urinary control. The

seizure usually lasts a total of 2 - 3 minutes, after which the

patient remains unconscious for a while and then awakens to

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confusion and extreme fatigue. A severe throbbing headache

similar to migraine may also follow the tonic-clonic phases.

Absence (Petit

Mal) Seizures.

Absence (petit mal) seizures are brief losses of consciousness

that occur for 3 - 30 seconds. Physical activity and loss of

attention last for only a moment. Such seizures may pass

unnoticed by others. Young children may simply appear to be

staring or walking distractedly.

Petit mal may be confused with simple or complex partial

seizures, or even with attention deficit disorder.

In petit mal seizures, a person may experience attacks as

often as 50 - 100 times a day.

Myoclonic seizures are a series of brief jerky contractions of

specific muscle groups, such as the face or trunk.

Atonic

(Akinetic)

Seizures.

A person who has an atonic (akinetic) seizure loses muscle

tone. Sometimes it may affect only one part of the body so

that, for instance, the jaw slackens and the head drops. At

other times, the whole body may lose muscle tone, and the

person can suddenly fall. A brief atonic episode is known as a

drop attack.

Simply Tonic or

Clonic Seizures

Seizures can also be simply tonic or clonic. In tonic seizures,

the muscles contract and consciousness is altered for about

10 seconds, but the seizures do not progress to the clonic or

jerking phase. Clonic seizures, which are very rare, occur

primarily in young children, who experience spasms of the

muscles but not tonic rigidity.

Types of Epilepsy

While there are a number of different epilepsy syndromes, there are two

primary types of epilepsy that affect a number of individuals. Each type has

specific features that distinguish it (10).

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Idiopathic

Epilepsy

In idiopathic generalized epilepsy, there is often, but not always, a

family history of epilepsy. Idiopathic generalized epilepsy tends to

appear during childhood or adolescence, although it may not be

diagnosed until adulthood. In this type of epilepsy, no nervous

system (brain or spinal cord) abnormalities, other than the seizures,

can be identified on either an EEG or magnetic resonance imaging

(MRI) studies. The brain is structurally normal on a brain (MRI)

scan, although special studies may show a scar or subtle change in

the brain that may have been present since birth.

People with idiopathic generalized epilepsy have normal intelligence

and the results of the neurological exam and MRI are usually

normal. The results of the EEG may show epileptic discharges

affecting a single area or multiple areas in the brain (so called

generalized discharges).

The types of seizures affecting patients with idiopathic generalized

epilepsy may include:

Myoclonic seizures (sudden and very short duration jerking of

the extremities)

Absence seizures (staring spells)

Generalized tonic-clonic seizures (grand mal seizures)

Idiopathic generalized epilepsy is usually treated with medications.

Some people outgrow this condition and stop having seizures, as is

the case with childhood absence epilepsy and a large number of

patients with juvenile myoclonic epilepsy.

Idiopathic partial epilepsy begins in childhood (between ages 5 and

8) and may be part of a family history. Also known as benign focal

epilepsy of childhood (BFEC), this is considered one of the mildest

types of epilepsy. It is almost always outgrown by puberty and is

never diagnosed in adults. Seizures tend to occur during sleep and

are most often simple partial motor seizures that involve the face

and secondarily generalized (grand mal) seizures. This type of

epilepsy is usually diagnosed with an EEG.

Symptomatic Symptomatic generalized epilepsy (SGE) encompasses a group of

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Generalized

Epilepsy

challenging epilepsy syndromes. As a group, SGE has 3 main

features: (1) multiple seizure types, especially generalized tonic and

atonic seizures; (2) brain dysfunction other than the seizures, in the

intellectual domain (mental retardation or developmental delay) and

in the motor domain (cerebral palsy); and (3) EEG evidence of

diffuse brain abnormality. The following are examples of epilepsy

syndromes that are included in the category of SGE:

Early myoclonic encephalopathy

Early infantine epileptic encephalopathy with suppression

bursts or Ohtahara syndrome

West syndrome

Epilepsy with myoclonic atonic seizures

Epilepsy with myoclonic absence

Lennox-Gastaut syndrome

Progressive myoclonic epilepsies

Epilepsy Syndromes

There are a number of different syndromes that fall under the umbrella of

epilepsy. These syndromes are defined based upon the type and severity of

seizures, as well as the area of the brain that is affected.

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(Photo Courtesy of: http://healthsciencedegree.info/seizure-brain-activity/)

To further distinguish these syndromes, factors such as age, cause, and

outcome are also included in the defining characteristics. The following

section provides a thorough overview of the various epilepsy syndromes

(9,11–16).

Temporal Lobe Epilepsy

Temporal Lobe Epilepsy (TLE) means that the seizures arise in the temporal

lobe of the brain. Experiences during temporal lobe seizures vary in intensity

and quality. Sometimes the seizures are so mild that the person barely

notices. In other cases, the person may be consumed with feelings of fear,

pleasure, or unreality. A patient may also report an odd smell, an abdominal

sensation that rises up through the chest into the throat, an old memory or

familiar feeling, or a feeling that is impossible to describe.

Types of seizures in TLE

The most common seizure type in TLE is a complex partial seizure. During

complex partial seizures, people with TLE tend to perform repetitive,

automatic movements (called automatisms), such as lip smacking and

rubbing their hands together. Three-quarters of people with TLE also have

simple partial seizures, and about half have tonic-clonic seizures at some

time. Some people with TLE experience only simple partial seizures.

Temporal lobe seizures usually begin in the deeper portions of the temporal

lobe. This area is part of the limbic system, which controls emotions and

memory. This is why the seizures can include a feeling of déjà vu, fear, or

anxiety, and why some people with TLE may have problems with memory

and depression.

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In most cases, the seizures associated with TLE can be fully controlled with

medications used for partial seizures. If drugs are ineffective, brain surgery

is often an option for patients with TLE. Temporal lobectomy is the most

common and successful form of epilepsy surgery. Vagus nerve stimulation

can also be beneficial in cases where temporal lobectomy is not

recommended or has failed.

Frontal lobe epilepsy is the next most common form of epilepsy after

temporal lobe epilepsy (TLE), and involves the frontal lobes of the brain. As

in temporal lobe epilepsy, seizures in frontal lobe epilepsy are partial,

though seizure symptoms differ depending on the frontal lobe area involved.

Frontal Lobe Epilepsy

Since the frontal lobes are responsible for a wide array of functions including

motor function, language, impulse control, memory, judgment, problem

solving, and social behavior, seizure symptoms in the frontal lobes vary

widely. Also, the frontal lobes are large and include many areas that do not

have a precisely known function. Therefore, when a seizure begins in these

areas, there may be no symptoms until it spreads to other or most areas of

the brain, causing a tonic-clonic seizure. When motor areas controlling motor

movement are affected, abnormal movements occur on the opposite side of

the body. Seizures beginning in frontal lobe motor areas can result in

weakness or the inability to use certain muscles, such as the muscles that

allow someone to speak.

Complex partial seizures of frontal lobe origin are usually quite different from

temporal lobe seizures. Frontal lobe seizures tend to be short (less than 1

minute), and occur in clusters and during sleep. They include strange

automatisms such as bicycling movements, screaming, or even sexual

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activity, followed by confusion or tiredness. Sometimes a person will remain

fully aware during a frontal lobe seizure, while at the same time having wild

movements of the arms and legs. In fact, a seizure from the frontal lobe

may even involve laughing or crying as the only symptom, though both

laughing (gelastic) and crying (dacrystic) seizures could come from the

temporal lobe as well. The EEG might be the only way to determine which

lobe is involved in these cases.

In many cases, frontal lobe seizures can be well controlled with medications

for partial seizures. If antiepileptic drugs are not effective, surgery to

remove the seizure focus may be an option in selected cases. Those patients

with abnormalities on the brain MRI or CT scans limited to one frontal lobe

are the best candidates, but even those with normal imaging studies may be

successfully treated with surgery. Vagus nerve stimulation can also be

beneficial in cases where brain surgery is not recommended or fails.

Parietal Lobe Epilepsy

Parietal lobe epilepsy is a relatively rare form of epilepsy, comprising about

5% of all epilepsy, in which seizures arise from the parietal lobe of the brain.

Parietal lobe epilepsy can start at any age and occurs in both males and

females equally. It may be a result of head trauma, birth difficulties, stroke,

or tumor, though the cause is unknown in 20% of patients.

The parietal lobe is located just behind the frontal lobe and it plays

important roles in touch perception, the integration of sensory information

and in visual perception of spatial relationships among objects (visuospatial

processing). In the language dominant side of the brain (the left side for

most right-handed individuals), the parietal lobe is also involved with

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language, planned movements such as writing, as well as mathematical

skills.

Since the parietal lobe involves the processing and integration of sensory

and visual perception, seizures originating from the parietal lobe can involve

both sensory and visual sensations. Seizure duration varies, from a few

seconds in some patients to a few minutes in others. The following are the

different types of symptoms associated with parietal lobe seizures.

Somatosensory seizures

These are the most common type of seizures in parietal epilepsies. Patients

with these types of seizures describe feeling physical sensations of

numbness and tingling, heat, pressure, electricity and/or pain. Pain, though

a rare symptom in seizures overall, is quite common in parietal seizures,

occurring in up to one quarter of patients. Some patients describe a typical

“Jacksonian march”, in which the sensation marches in a predictable pattern

from the face to the hand up the arm and down the leg. Rarely, a patient will

describe a sensation in the genitalia, occasionally leading to orgasm.

Somatic illusions

During a somatic illusion, another common symptom of parietal seizures,

patients may experience a feeling like their posture is distorted, that their

arms or legs are in a weird position or are in motion when they are not, or

that a part of their body is missing or feels like it does not belong.

Patients with parietal seizures may also experience vertigo, a sensation of

movement or spinning of the environment, or of their body within the

environment.

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Visual illusions and hallucinations

Patients with visual illusions report a distortion of visual perception. Objects

seem too close, too far, too large, too small, slanted, moving or otherwise

not right. A patient with hallucinations describes seeing objects that seem

very real, though in fact they do not exist. Rarely, a patient with a parietal

seizure will report difficulty understanding spoken words or language,

difficulty reading or performing simple math.

Treatment with antiepileptic medication is usually effective in controlling

seizures in parietal lobe epilepsy. In severe cases, surgery may be an

option.

Occipital Lobe Epilepsy

In occipital lobe epilepsy, seizures arise from the occipital lobe of the brain,

which sits at the back of the brain, just below the parietal lobe and just

behind the temporal lobe. The occipital lobe is the main center of the visual

system. Occipital lobe epilepsy accounts for about 5-10% of all epilepsy

syndromes. This kind of epilepsy can be either idiopathic (of unknown,

presumed genetic, cause) or symptomatic (associated with a known or

suspected underlying lesion). Benign occipital epilepsies usually begin in

childhood and are discussed elsewhere.

Occipital seizures usually begin with visual hallucinations like flickering or

colored lights, rapid blinking, or other symptoms related to the eyes and

vision. They may occur spontaneously but can often be triggered by

particular visual stimuli, such as seeing flashing lights or a repeating pattern.

Occipital seizures are often mistaken for migraine headache because they

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share similar symptoms including visual disturbances, partial blindness,

nausea and vomiting, and headache. The following are the different types of

seizure symptoms associated with occipital lobe seizures:

Visual hallucinations and/or illusions

Blindness or decreased vision

Pallinopsia or image repetition (image replayed again and again) can occur:

Sensation of eye movements

Eye pain

Involuntary eye movement to one or other side

Nystagmus or eye jerking to one or other side (rapid involuntary

rhythmic eye movement, with the eyes moving quickly in one direction

(quick phase), and then slowly in the other (slow phase),

Eyelid fluttering

As with any epilepsy syndrome, detailed patient history, neurological

examination, and EEG are very important. In occipital lobe epilepsy, the EEG

may provide information that is very helpful in making the correct diagnosis.

An abnormal response in the EEG to intermittent photic stimulation (rapidly

flashing strobe light) often occurs in occipital lobe epilepsy; however, this

response can occur in other epilepsy syndromes as well.

Treatment with a drug used for partial epilepsy, often carbamazepine, is

usually effective. In intractable cases (those that do not respond to

medication), surgical options may be considered.

Primary Generalized Epilepsy

Primary Generalized Epilepsy (PGE), also called Idiopathic Generalized

Epilepsy (IGE), refers to an epilepsy syndrome of idiopathic or unknown

cause. An idiopathic disease is a primary or intrinsic disorder that cannot be

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attributed to a known underlying condition. So, while other types of epilepsy

may be caused by a brain tumor, stroke, or other neurological disorder,

idiopathic epilepsy is a primary brain disorder of unknown cause. In fact,

most idiopathic epilepsy syndromes are presumed to be due to a genetic

cause, but in most cases the specific genetic defect is not known and a

family history of epilepsy may not be present. There are a number of

different PGE syndromes. Each syndrome has its own characteristic seizure

type(s), typical age of onset, and specific EEG patterns. Some of these

syndromes are:

Childhood absence epilepsy

Juvenile myoclonic epilepsy

Juvenile absence epilepsy

Epilepsy with generalized tonic-clonic seizures on awakening

Generalized epilepsies with febrile seizures

PGE is a generalized type of epilepsy, which means there is no single part of

the brain where seizures originate. In fact, EEG results may show epileptic

discharges affecting the entire brain. The types of seizures patients with PGE

exhibit may include myoclonic seizures and absence seizures.

Generalized tonic-clonic seizures

The seizures in PGE usually respond well to medication. Some of the more

commonly prescribed medications for these syndromes include: valproate,

lamotrigine, topiramate, levetiracetam; and, in Childhood Absence Epilepsy,

ethosuximide.

Nearly all patients with PGE begin having seizures in childhood or

adolescence. Most patients with childhood absence epilepsy (CAE) start

having seizures before age 10, and “outgrow” their seizures within a few

years, meaning that they no longer need medication to control their

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seizures. On the other hand, juvenile myoclonic epilepsy (JME) is generally

considered a life-long disease. Once seizures start, usually in adolescence,

most patients need medication treatment for life to prevent seizure

recurrence. Individuals with PGE syndromes usually have normal

development and intelligence.

Idiopathic Partial Epilepsy

Just as there are generalized epilepsies of unidentifiable, presumably

genetic, cause, there are also partial epilepsy syndromes of unknown or

idiopathic cause, or Idiopathic Partial Epilepsies. An idiopathic disease is a

disorder that cannot be attributed to a known underlying condition. So, while

other types of epilepsy may be caused by a brain tumor, stroke, or other

neurological disorder, idiopathic epilepsy is a primary brain disorder of

unknown cause. In fact, most idiopathic epilepsy syndromes are presumed

to be due to a genetic cause, but in most cases the specific genetic defect is

not known and a family history of epilepsy may not be present.

Benign rolandic epilepsy

There are a few idiopathic partial epilepsy syndromes. Each individual

syndrome generally has its own characteristic seizure type(s), typical age of

onset, and specific EEG patterns. Some of these syndromes are known as:

benign rolandic epilepsy, is also known as benign epilepsy of childhood with

centrotemporal spikes, early onset benign childhood occipital epilepsy, and,

late onset benign childhood occipital epilepsy.

The seizures in idiopathic partial epilepsy typically respond well to

medications used for other partial epilepsy syndromes. However, depending

on the seizure type, time of day, and frequency, some providers and parents

choose not to treat the individual with medication at all. For example, a

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patient with benign rolandic epilepsy who experiences rare nocturnal

seizures consisting of only brief face and arm twitching may do well without

any medication treatment.

Though the prognosis of these syndromes varies by syndrome type, it is

usually quite good. Younger patients with these syndromes most often

“outgrow” their seizures by teenage years or young adulthood, and also

have normal intelligence and motor skills.

Symptomatic Generalized Epilepsy

Symptomatic Generalized Epilepsy (SGE) refers to epilepsy syndromes in

which the majority of seizures are generalized, but partial onset seizures can

also occur. The types of generalized seizures that occur in SGE include

myoclonic, tonic, atonic, atypical absence, and generalized tonic-clonic.

Virtually any type of partial onset seizure can also occur, depending on the

underlying brain pathology. Usually (but not always) there is a known

underlying brain disorder or injury, which is often severe. These syndromes

may occur in the setting of certain neurological diseases, such as Tuberous

Sclerosis (a rare genetic mutation that affects several organ systems), or

may be due to lack of oxygen at birth, trauma, infection, developmental

malformations, chromosomal abnormalities or other causes. SGE syndromes

typically begin in early life.

The following is a list of some symptomatic generalized epilepsy syndromes:

West Syndrome

Lennox-Gastaut Syndrome

Epilepsy with myoclonic-astatic seizures

Epilepsy with myoclonic absences

Early myoclonic encephalopathy

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Early infantile epileptic encephalopathy with suppression burst

Progressive myoclonic epilepsies

Antiepileptic medications are the mainstay of treatment in SGE, though

certain syndromes may require additional treatments including

adrenocorticotropic hormone (ACTH) or Immunoglobulin. The ketogenic diet

may be helpful in some patients. The vagus nerve stimulator has been

studied extensively in patients with SGE. In some patients it has been very

helpful, while others have experienced no benefit. In patients with atonic, or

drop seizures, a surgical procedure called corpus callosotomy may help

reduce the falls that may result from seizures.

There are, however, some SGE syndromes in which other surgical options

may be considered. In Tuberous Sclerosis, for example, where the epilepsy

is often considered a SGE syndrome, certain tubers may be more

epileptogenic than others. If such a tuber is found to be the cause of the

most disabling seizures, removal of it could reduce the frequency of

seizures.

The prognosis of SGE depends largely on the underlying cause of the

seizures. For example, up to 15-30% of patients with West Syndrome,

affecting infants, without known cause become seizure free and have normal

or near normal intelligence. However, patients with Lennox-Gastaut

Syndrome or progressive myoclonic epilepsy tend to have seizures

throughout life, and some level of cognitive impairment.

Progressive Myoclonic Epilepsy

Progressive myoclonic epilepsies are rare and frequently result from

hereditary metabolic disorders. They feature a combination of myoclonic and

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tonic-clonic seizures. Unsteadiness, muscle rigidity, and mental deterioration

are often also present.

Progressive myoclonic epilepsies are treated with medication, which usually

proves to be successful for a short period of time (months to years).

However, as the disorder progresses, drugs become less effective and

adverse effects may be more severe as more drugs are used at higher

doses. Valproate and zonisamide are most commonly used. Other commonly

prescribed drugs include clonazepam, lamotrigine, topiramate, phenobarbital

and carbamazepine. Types of Progressive Myoclonic Epilepsies include:

Mitchondrial Disorders, involving mutation of genes, and;

Unverricht-Lundborg Syndrome, a myoclonic disorder.

Reflex Epilepsy

In reflex epilepsies, seizures are triggered by specific stimuli in the

environment. In the most common type of reflex epilepsy, flashing lights

trigger absence, myoclonic or tonic-clonic seizures. This is called

photosensitive epilepsy, which usually begins in childhood and is often

outgrown by adulthood. Other environmental triggers in reflex epilepsy

include sounds such as church bells, a certain type of music or song, or a

person’s voice. For some people, activities such as arithmetic, reading,

writing, and even thinking about specific topics can provoke seizures. These

non-visual stimuli may trigger generalized or partial-onset seizures. Some

patients with reflex epilepsy can have spontaneous seizures that occur

without exposure to their specific trigger.

A two-pronged approach is usually best in treating reflex epilepsy: avoiding

the triggering stimulus as much as possible, and treatment with antiepileptic

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drugs. Valproate, carbamazepine and clonazepam have been most

commonly prescribed to control reflex seizures, though lamotrigine,

levetiracetam and other newer antiepileptic medication are promising.

Epilepsy Syndromes In Children

Febrile Seizures

Children aged 6 months to 5 - 6 years may have tonic-clonic seizures when

they have a high fever. These are called febrile seizures and occur in 2% to

5% of children. There is a slight familial (hereditary) tendency toward febrile

seizures. In other words, the chances are slightly increased that a child will

have febrile seizures if their parents, brothers or sisters, or other close

relatives have had them.

The peak age of febrile seizures is about 18 months. The usual situation is a

healthy child with normal development, who has a viral illness with high

fever. As the child's temperature rapidly rises, he or she has a tonic-clonic

seizure. The seizure usually involves muscles on both sides of the body.

Febrile seizures can be as short as a minute or two, or as long as 30 minutes

or more. They also can be repetitive. In most instances, hospitalization is

not necessary, although a prompt medical consultation is essential after the

first seizure.

Most children with recurrent febrile seizures do not require daily antiepileptic

drug therapy. Children who have had more than three febrile seizures or

prolonged febrile seizures, or who have seizures when they have no fever,

are usually treated with antiepileptic drugs including phenobarbital and/or

valproate. Diazepam (Valium), if given by mouth or rectum at the time of

fever, has been used effectively to both treat and prevent recurrent febrile

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seizures. However, the dose that is effective when given by mouth can cause

irritability, insomnia, or other troublesome side effects that last for days.

The prognosis for febrile seizures is excellent. There is no reason for a child

who has had a single febrile seizure to receive antiepileptic drugs unless the

seizure was unusually long or other medical conditions warrant it.

Recurrence rates (the chances of having another seizure) vary from 50% if

the seizure occurred before age one year to 25% if the seizure occurred

after that age. In addition, 25% to 50% of recurrent febrile seizures are not

preceded by a fever. In some cases, the seizure is the first sign of an illness

(usually viral) and the fever comes later.

The vast majority of children with febrile seizures do not have seizures

without fever after age five. Risk factors for later epilepsy include:

Abnormal development before the febrile seizure

Complex febrile seizures (seizures lasting longer than 15 minutes,

more than one seizure in 24 hours, or body movements during the

seizure restricted to one side)

A history of seizures without fever in a parent or a brother or sister.

If none of these risk factors is present, the chances of later epilepsy are the

same or nearly the same as in the general population; if one risk factor is

present, the chances of later epilepsy are 2.5%; if two or more risk factors

are present, the chances of later epilepsy range from 5% to over 10%.

Rarely, febrile seizures that last more than 30 minutes may cause scar

tissue in the temporal lobe and chronic epilepsy that can be effectively

treated with medication or a temporal lobectomy.

Benign Rolandic Epilepsy

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Benign rolandic (sylvian) epilepsy (BRE, also called BECTS (benign epilepsy

of childhood with centrotemporal spikes), is a common childhood seizure

syndrome, with seizures beginning between 2 and 13 years of age. A

hereditary factor is often present. The seizures most commonly observed in

BRE are partial motor seizures (twitching) or a sensory seizure (numbness or

tingling sensation) involving the face or tongue and which may cause

garbled speech. In addition, tonic-clonic seizures may occur, especially

during sleep. Although the seizures are often infrequent, or may occur in

infrequent clusters, some patients need medication. These include children,

in addition to the typical seizure disorder, that have daytime seizures, a

learning disorder, a mild mental handicap, or multiple seizures at night,

which leave the child lethargic in the morning.

The EEG shows a characteristic pattern of abnormal spikes over the central

and temporal regions of the brain, especially during sleep. Despite the

abundant abnormal spike activity, the child may have only one or a few

seizures. This illustrates that the amount or frequency of abnormal spike

activity in the EEG is not necessarily related to the severity of the epileptic

disorder. Siblings or close relatives may have the same EEG pattern during

childhood without ever having seizures.

The seizures are usually easily controlled with low to moderate doses of

carbamazepine, oxcarbazepine, or gabapentin (or, outside the United States,

clobazam). Medication is usually continued until age 15, when the seizures

spontaneously stop in almost all patients.

Juvenile Myoclonic Epilepsy

Juvenile myoclonic epilepsy (JME) accounts for about 7% of the cases of

epilepsy, making it one of the most common epilepsy syndromes. The

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syndrome is defined by myoclonic seizures (jerks) with or without tonic-

clonic or absence seizures. The EEG usually shows a pattern of intermittent

spike-and-wave or polyspike-and-wave, even in between seizures. CT and

MRI scans of the brain are normal and typically are not needed.

Seizures usually begin shortly before or after puberty, or sometimes in early

adulthood. They usually occur in the early morning, within a couple hours of

awakening. Persons with JME often have photosensitive myoclonic seizures

in addition to spontaneous seizures. The intellectual functions of persons

with JME are the same as those in the general population.

Juvenile myoclonic epilepsy often has a genetic basis. In some families,

genes associated with an increased risk of JME are located on chromosomes

6, 8, or 15. The chance that a child born to a parent with JME will also have

JME is about 15%. In most cases, the seizures are well controlled with

medication, but the disorder is lifelong. Valproate is the treatment of choice.

Other options include lamotrigine, levetiracetam, or topiramate.

Carbamazepine may actually worsen the myoclonic jerks.

Infantile Spasms

Infantile spasms (West's syndrome), a very uncommon form of epilepsy,

begins between 3 and 12 months of age. The seizures, or spasms, consist of

a sudden jerk followed by stiffening. With some spells, the arms are flung

out as the body bends forward (also called jackknife seizures). Other spells

have more subtle movements limited to the neck or other body parts. A

brain disorder or brain injury, such as birth trauma with oxygen deprivation,

precedes the seizures in 60% of these infants, but in the other 40% no

cause can be determined, and development is normal prior to the onset of

seizures.

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Several antiepileptic drugs and hormonal therapy can be used to treat

infantile spasms. Some experts recommend a trial of an antiepileptic drug

(e.g., vigabatrin, valproate, topiramate) before hormonal therapy, but

others use hormonal therapy as the first treatment. In countries where it is

available, vigabatrin (Sabril) is often used as the initial therapy because it is

relatively safe (especially for short-term use) and effective. Vigabatrin is

especially effective in children with infantile spasms due to tuberous

sclerosis (a disorder associated with abnormalities involving the brain, skin,

heart, and other parts of the body).

If vigabatrin does not control the seizures in 3 or 4 days, adrenocorticotropic

hormone (ACTH) is usually used next. ACTH is a hormone made by the

pituitary gland. It stimulates the adrenal glands to make and release

additional cortisol, which acts much like prednisone. ACTH has been proven

to be slightly more effective than prednisone, but it must be given as an

injection, once a day for the first several weeks, then every other day.

Steroid hormones such as prednisone, on the other hand, can be given by

mouth. ACTH stops seizures in more than half of children with infantile

spasms.

In the United States, ACTH is often used as the first therapy and is typically

given for 1 month. The dosage is highest during the first 2 weeks and then

usually lowered gradually. The adverse effects of ACTH depend on the dose

used, the duration of therapy, and the baby’s sensitivity to the drug.

Although rare allergic reactions may occur, all other adverse effects occur

because ACTH stimulates the infant’s body to produce cortisol, a steroid

hormone. Excessive cortisol can cause the following:

Irritability

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Increased appetite

High blood pressure

Kidney problems

Redistribution of body fat to make the face and trunk fatter and the

arms and legs thinner

Increased risk of infection or gastrointestinal bleeding

Metabolic changes that alter the concentrations of glucose (sugar),

sodium, and potassium in the blood.

For most babies with infantile spasms, the adverse effects of ACTH can be

safely managed. Often the baby will be given another anti-epileptic drug

after the spasms have stopped and the ACTH therapy has been completed.

The future course of the disorder and of the child's development is related to

the cause of the seizures, the child's intellectual and neurological

development before the seizures began (the better the condition at that

time, the better the outlook), and whether they are controlled quickly. The

sooner therapy is begun, the better the results.

When the spasms stop, some children will later develop other types of

seizure. Untreated children often have frequent spasms for many years, and

later develop partial and generalized seizures. Approximately one-fifth of the

cases of West’s syndrome will evolve into Lennox-Gastaut syndrome.

Lennox-Gastaut Syndrome

Lennox-Gastaut syndrome is serious but uncommon. Three things define it:

Difficult-to-control generalized seizures

Mental handicap

Slow spike-and-wave pattern on the EEG

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The seizures usually begin between 1 and 6 years of age, but can begin

later. The syndrome involves some combination of tonic, atonic, atypical

absence, myoclonic, and tonic-clonic seizures that are usually resistant to

medications. Useful medications for controlling the seizures of patients with

Lennox-Gastaut syndrome include valproate, carbamazepine, clobazam (not

available in the US), lamotrigine, and topiramate. Felbamate is also an

effective drug and can often improve behavior and quality of life, but it

carries a risk of life-threatening blood or liver disorders and must be used

carefully.

In children or adults with frequent, poorly controlled seizures, it is often wise

to avoid high doses of antiepileptic drugs because they may intensify the

behavioral, social, and intellectual problems, especially when two or more

drugs are used together. It may be better to tolerate slightly more frequent

seizures in order to have a more alert and attentive family member.

In those patients whose seizures are not controlled with medication, there

are other options. These include the vagus nerve stimulator, the ketogenic

diet or corpus callosotomy (a palliative surgical procedure). Vagus nerve

stimulation or corpus callosotomy can be helpful treatments for some

patients. However, experts typically recommend vagus nerve stimulation

before consideration of corpus callosotomy because of lower risks.

Most children with Lennox-Gastaut syndrome have intellectual impairment

ranging from mild to severe. Behavioral problems are also common and

probably relate to a combination of the brain dysfunction, seizures, and

antiepileptic drugs. The course of the seizures varies greatly. Some children

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will later have fairly good seizure control. Others will continue to have

multiple types of poorly controlled seizures throughout life.

The intellectual and behavioral development of children whose seizures come

under fair to good control may be almost normal, but the development of

those who have frequent seizures and are given high doses of more than

one drug may be severely delayed. This syndrome usually persists into

adulthood and affected persons often need to live in a residential (adult

foster care) group home when their parents are no longer able to care for

them.

Childhood Absence Epilepsy

Absence seizures are generalized seizures that occur in school-aged children

usually between the ages of 5 and 9. Sometimes childhood absence epilepsy

(CAE) can be inherited, but it can also occur as a sporadic event. Typical

absence seizures consist of sudden cessation of movement, staring, and

sometimes blinking. Sometimes, there may be a mild loss of body tone,

causing the child to lean forwards or backwards slightly. Unlike other types

of seizures, absence seizures occur without an aura or warning. When

diagnosing CAE, typical absence seizures need to be differentiated from

atypical absence seizures, which can occur at an earlier age. An EEG of a

child with CAE will show a typical pattern known as 3-Hz generalized spike

and wave complexes.

Many children with CAE have normal neurological examinations and

intellectual abilities. However, some children may have developmental and

intellectual impairments and may have other types of seizures including, but

not limited to, tonic clonic seizures. The medications that are usually used to

treat CAE include ethosuximide and valproic acid, but other medications can

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also be used successfully. Usually children are treated for a minimum of 2

years.

The prognosis for CAE is excellent. Remission can be achieved in

approximately 80% of patients. Close attention must be paid to seizure

control to avoid academic or social difficulties.

Benign Occipital Epilepsy

In this epilepsy syndrome, seizures usually begin between the ages of 5 and

7, and originate in the occipital lobe. Seizure symptoms often include the

following:

visual hallucinations

loss of vision, or forced deviation of the eyes

vomiting

The hallucinations can take any form, but tend to be of brightly colored

shapes of all sizes. Children may then complain of intense headache and

may have extended periods of nausea and/or vomiting. Benign occipital

epilepsy (BOE) can sometimes be mistaken for migraines due to the visual

changes and headaches associated with this type of epilepsy. In addition to

hallucinations and visual disturbances children may also experience jerking

movements on one side of their body.

The EEG of a child with BOE shows spikes in the occipital region of the head

during sleep, or when the eyes are closed during wakefulness. An MRI scan

of the brain will be normal. By definition, BOE is not caused by a structural

lesion or abnormality. Since the seizures are of partial origin, medications

such as carbamazepine and oxcarbazepine are good treatment options.

Children with BOE are usually neurologically normal and complete seizure

control can be attained in 60% of patients.

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Mitochondrial Disorders

Mitochondria are the energy factories of the cell. Abnormalities in

mitochondrial DNA or genes produce metabolic disorders that affect different

parts of the body, including muscle and brain. Mitochondrial disorders can be

inherited or sporadic. When inherited, the abnormal genes always come

from the mother, since all mitochondria are of maternal origin. Two

mitochondrial disorders can be associated with epileptic seizures:

one is MELAS (which stands for mitochondrial encephalopathy), lactic

acidosis (too much lactic acid in the blood), and stroke-like episodes. MELAS

can lead to stroke-like episodes at a young age (usually before 40), seizures,

dementia, headaches, vomiting, unsteadiness, and ill effects from exercise.

Persons with MELAS can have both generalized (including myoclonic and

tonic-clonic) and partial seizures.

The other mitochondrial disorder with epileptic seizures is MERRF, which

stands for myoclonic epilepsy with ragged red muscle fibers. MERRF is one of

the progressive myoclonic epilepsies. It can also be associated with hearing

loss, unsteadiness, dementia, and ill effects from exercise. In addition to

myoclonic seizures, patients with MERRF often have generalized tonic-clonic

seizures. There are other mitochondrial disorders that do not fit clearly into

the MELAS or MERRF syndromes but which can cause epilepsy and additional

neurological problems.

There is no specific cure yet for mitochondrial disorders. Treatment is geared

towards controlling symptoms and slowing the progression of the disease. A

medical provider may prescribe a combination of supplements such as Co-

enzyme Q 10 or L-Carnitine in addition to other supplements. For patients

who have isolated deafness, evaluation for a cochlear implant may be

possible. For patients with seizures, standard antiepileptic medications are

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used, such as those mentioned below in the section on Anti-Epileptic

Medications.

Landau-Kleffner Syndrome

The Landau-Kleffner syndrome (acquired epileptic aphasia) is another rare

disorder. Acquired aphasia means the loss of language abilities that had

been present. In the typical case, a child between 3 and 7 years of age

experiences progressive language problems, with or without seizures. The

language disorder may start suddenly or slowly. It usually affects auditory

comprehension (understanding spoken language) the most, but it may affect

both understanding speech and speaking ability, or it may affect speaking

only. Seizures are usually rare and often occur during sleep. Simple partial

motor seizures are most common, but tonic-clonic seizures can also occur.

Seizure control is rarely a problem.

The EEG is often the key to the diagnosis. A normal EEG, especially one

done when the child is awake, does not rule out this disorder. Sleep

activates the abnormal spike activity, and therefore sleep recordings are

extremely important.

The boundaries of the Landau-Kleffner syndrome are imprecise. Some

children may first have a delay in language development followed by a loss

of speech abilities. Landau-Kleffner syndrome (or a variant of it) may also

occur in some children in whom language function never develops, or in

others whose language skills move backward but who very seldom have

spike-wave discharges on the EEG. The exact relationship between the EEG

findings and the language disorder is imprecise, although in some cases the

epilepsy activity may contribute to the language problems.

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Standard antiepileptic drugs may help the seizures but are ineffective in

treating the language disorder. Steroids are effective in some children,

improving both the EEG abnormalities and the language problems. A form of

epilepsy surgery, multiple subpial transections, may improve both the EEG

abnormalities and the language disorder in a small number of children, but

results to confirm this finding are still coming in from various epilepsy

centers. In some cases, intravenous immunoglobulin (IVIG) has proven to

be helpful.

Rasmussen Syndrome

Rasmussen syndrome usually begins between 14 months and 14 years of

age and is associated with slowly progressive neurologic deterioration and

seizures. Seizures are often the first problem to appear. Simple partial motor

seizures are the most common type, but in one-fifth of these children, the

first seizure is an episode of partial or tonic-clonic status epilepticus.

Although Rasmussen syndrome is rarely fatal, its effects are devastating.

Progressive weakness on one side (hemiparesis) and mental handicap are

common, and language disorder (aphasia) often occurs if the disorder affects

the side of the brain that controls most language functions, which is usually

the left side. Mild weakness of an arm or leg is the most common initial

symptom besides seizures. The weakness and other neurologic problems

often begin 1 to 3 years after the seizures start. CT and MRI scans of the

brain show evidence of a slow loss (atrophy) of brain substance. Recent

studies suggest that the cause of Rasmussen’s syndrome is an autoimmune

disorder (antibodies are produced against the body’s own tissues) directed

against receptors on the brain cells. The process may be triggered by a viral

infection.

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Treatment of this disease with antiepileptic drugs has been disappointing.

Steroids may be effective, but additional studies are needed. Immunologic

therapies (gamma globulin, plasmapheresis, prednisone) may be helpful in

some cases. In children with severe weakness and loss of touch sensation

and vision on the side of the body opposite to the involved hemisphere of

the brain, a surgical procedure called a functional hemispherectomy may be

successful.

Hypothalamic Hamartoma & Epilepsy

Small tumors in the base of the brain that affect the hypothalamus can

cause a syndrome consisting of abnormally early puberty, partial seizures

with laughing as a frequent feature, and increased irritability and aggression

between the seizures. The partial seizures may be simple or complex and

there may be secondary generalized tonic-clonic seizures.

Affected individuals are often short and have mild abnormalities in their

physical features (dysmorphisms). A high-quality MRI brain scan is

necessary for diagnosis. If the tumor extends beyond the hypothalamus and

below the brain, treatment with surgery may be an option. Antiepileptic

drugs can also be beneficial, as well as drugs aimed at hormonal and

behavioral problems, if needed.

Treatment

Treatment is typically required to control the seizures associated with

epilepsy. However, some patients may not require treatment. The initiation

and continuation of treatment will depend on a number of factors, including

the severity of the condition, the extent and duration of seizures, the

presence of other physical conditions, and the patient’s individual needs.

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Therefore, it is important for providers to work with each patient to

determine what type of treatment will best meet the needs of the patient.

In addition, regular monitoring is crucial once treatment is initiated, as the

patient may require adjustments depending on how he or she responds to

the therapy. This is especially crucial when treating the patient

pharmacologically (17).

Some patients will require lifelong treatment to manage their seizures, while

others will only require short term, intermittent treatment to manage their

symptoms. In many instances, patients will only experience seizures during

specific periods in their lifetime. In fact, a number of cases of epilepsy will

include seizures that present in childhood and diminish over time (18). In

these instances, treatment will only be required during the time that the

patient is experiencing seizures. The following guidelines are typically used

when determining if treatment is required: (15)

Usually, Anti-Epileptic Drug (AED) treatment will not begin until after an individual has

had a second seizure. This is because a single seizure is not a reliable indicator that an

individual has epilepsy. In some cases, treatment will begin after a first seizure if:

An electroencephalogram (EEG) test shows brain activity associated with epilepsy.

A magnetic resonance imaging (MRI) scan shows damage to the brain.

The patient has a condition that has damaged the brain, such as a stroke.

For some people, surgery may be an option. However, this is only the case if removing

the area of the brain where epileptic activity starts would not cause damage or disability.

If successful, there is a chance the epilepsy will be cured.

If surgery is not an option, an alternative may be to implant a small device under the skin

of the chest. The device sends electrical messages to the brain. This is called vagus nerve

stimulation.

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A variety of treatment options are available to patients experiencing epileptic

seizures. Most patients will attempt to manage their symptoms through

non-pharmacologic therapies. If these treatments are not successful, the

patient will begin pharmacologic treatment (19).

Diet

Some patients will attempt to manage the symptoms of epilepsy through a

change in diet. The ketogenic diet is a high fat, low carbohydrate diet that

has been shown to reduce symptoms of epilepsy, especially in children (20).

While the diet is effective, it is also very difficult to manage and can be quite

limiting for the patient. The success of the ketogenic diet relies on strict

adherence to carbohydrate restriction. Therefore, patients cannot allow any

flexibility in their daily eating patterns (21).

When excess amounts of carbohydrates are consumed, the patient will

“reset” ketone metabolism for up to two weeks, which will minimize the

efficacy of the diet in managing seizure activity (22). Many patients find the

diet too restrictive and are unable to fully adhere to it. In fact, less than ten

percent of patients are able to commit to the diet for more than a year (23).

Ketogenic, and in some instances,

modified Atkins diets have been

shown to reduce epileptic seizures

by approximately fifty percent (24).

The most significant results occur in

patients who reduce daily

carbohydrate levels to ten grams or

(Photo courtesy of: http://www.ketogenic-

diet-resource.com/images/ketoratios.jpg)

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less per day. However, some patients will still experience a reduction in

seizures while allowing for a higher number of carbohydrates each day. In

these patients, twenty to thirty grams of carbohydrates appears to be an

appropriate number (25). The diet is especially successful in children, but

does appear to be helpful in adults experiencing epileptic seizures (26).

In most cases, patients will require a period of adjustment to determine if

the diet will reduce symptoms. Often, physicians will require patients to

adhere to the diet for three months before making a determination regarding

its effectiveness (23). In the early stages of the diet, the patient will continue

medication. However, once the patient has had time to adjust to the diet,

medication will be tapered. The eventual goal is complete discontinuation,

but, in some instances, the patient will still require low doses of medication

(27).

While the ketogenic diet is quite effective, there are some potential side

effects (28). Reported side effects include dehydration, constipation, and,

sometimes, complications from kidney stones or gall stones. Adult women

on the diet may have menstrual irregularities. Pancreatitis (inflammation

of the pancreas), decreased bone density and certain eye problems have

also been reported. Again, this is why the medical team closely follows

children or adults who are on the diet.

The diet lacks several important vitamins, which have to be added

through supplements. Sometimes high levels of fat build up in the blood,

especially if a child has an inborn defect in his ability to process fat. This

possibility can lead to serious effects, which is another reason for careful

monitoring.

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The ketogenic diet is very effective, but it is not the right treatment for all

patients. If a patient will be unable to adhere to the strict guidelines

required of the diet, it is not considered an appropriate method of treatment.

Therefore, the treating provider must work with the patient to determine of

if he or she is a viable candidate for diet therapy. If it is determined that the

patient is not suited for this type of treatment, other methods must be

considered.

Electroencephalography Biofeedback

Electroencephalography (EEG) biofeedback has been used to treat many

forms of epilepsy since the early 1970’s. It is especially helpful in treating

petit mal, grand mal, and complex partial seizures (29). In earlier years, the

technique was used infrequently, as it was quite expensive. In addition,

training for the procedure required a long term commitment and was not

easily accessible (30). However, recent advances in technology and

methodology have made the procedure more affordable, while also reducing

the cost and length of training. Therefore, biofeedback is utilized more

frequently as a treatment for

epilepsy (31).

Although access to the procedure

has increased the number of

individuals who revive biofeedback

treatment, there are still

discrepancies in the outcomes

experienced. Some patients will

respond to treatment quickly,

requiring only a few sessions to

experience a reduction in seizures.

(Photo courtesy of:

http://heartzine.com/diagrams/eeg-system.jpg)

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Other patients may require a more extensive treatment period, often

requiring 80 – 100 treatment sessions before experiencing any reduction in

seizures (32). Therefore, the procedure is still not a viable option for some

patients. In addition, many patients will require complementary treatment

with other therapies in conjunction with biofeedback (33).

In most instances, biofeedback is used as part of a comprehensive treatment

program that includes other therapies such as dietary management, lifestyle

changes, and pharmacologic intervention. This multi-faceted approach to

treatment typically produces the greatest results in patients who have more

severe cases of epilepsy. In patients with less severe cases, a single

treatment such as biofeedback is often adequate for reducing seizures (30).

Biofeedback can help regulate behavioral disturbances in patients with

epilepsy, even when it does not eliminate seizures. In addition, it can help

reduce the dose of medication the patient requires to achieve seizure

elimination (34).

The neurons in the brain are divided into bands, some slow, some moderate

and some fast, measured by cycles per second (30).

Delta (.05-3 hertz)

Produced in deep, dreamless sleep

Theta (4-7 hertz)

Drowsiness, inattention, deep meditation. A person with epilepsy will often

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produce bursts of theta.

Alpha (8-12 hertz)

General relaxation and meditation

SMR (sensorimotor rhythm) (12-15 hertz)

Relaxed concentration. Often used for seizure control.

Beta (15-18 hertz)

Focused attention

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Gamma (24 hertz and above)

Intense concentration or anxiety

EEGs of people with epilepsy appear as follows:

Spike-and-slow-wave

3-second spike-and-wave (Absence or Petit Mal)

During Tonic Clonic seizure

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An EEG of a person without epilepsy appears as:

Melatonin

Melatonin is a hormone secreted by the pineal gland in the brain. It helps

regulate other hormones and maintains the body's circadian rhythm. It also

plays an important role in epilepsy treatment and management. Many

individuals with epilepsy have lower than normal melatonin levels. In fact,

seizure activity may be linked to the body’s need to increase melatonin

levels, as the individual experiences a significant increase of melatonin when

a seizure occurs (35). Therefore, some recent clinical studies have attempted

to link melatonin supplementation with reduced seizure activity. In some

studies, there was a direct link between melatonin supplementation and a

decrease in seizure activity, especially in children (36). However, other trials

have been inconclusive (37).

Since melatonin supplementation is relatively new, there is no standard

dosage amount that is recommended. Some individuals may only require

low doses, while others will benefit from a larger dose. The physician will

need to experiment with dosage amounts to identify the appropriate amount

for each patient (38).

Melatonin can cause side effects in individuals. Therefore, the patient should

be closely monitored to ensure the side effects do not become problematic.

The most common side effects include: (39)

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Some people may have vivid dreams or nightmares when they take

melatonin. Taking too much melatonin may disrupt circadian rhythms

(“body clock”).

Melatonin can cause drowsiness if taken during the day. If an

individual is drowsy the morning after taking melatonin, a lower dose

may be necessary.

Additional side effects include stomach cramps, dizziness, headache,

irritability, decreased libido, breast enlargement in men (called

gynecomastia), and decreased sperm count.

Pregnant or nursing women should not take melatonin because it could

interfere with fertility.

Some studies show that melatonin supplements worsened symptoms

of depression. For this reason, people with depression should consult

their doctor before using melatonin supplements.

Melatonin may interact with various medications. The following table

provides an overview of the drugs that have the highest risk of interacting

with melatonin: (40)

Antidepressant

medications

In an animal study, melatonin supplements reduced the

antidepressant effects of desipramine and fluoxetine (Prozac).

More research is needed to know if the same thing would happen

in people. In addition, fluoxetine (a member of a class of drugs

called selective serotonin reuptake inhibitors, or SSRIs) can

cause low levels of melatonin in people.

Antipsychotic

medications

A common side effect of antipsychotic medications used to treat

schizophrenia is a condition called tardive dyskinesia, which

causes involuntary movements. In a study of 22 people with

schizophrenia and tardive dyskinesia caused by antipsychotic

medications, those who took melatonin supplements had fewer

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symptoms compared to those who did not take the supplements.

Benzodiazepines The combination of melatonin and triazolam (Halcion) improved

sleep quality in one study. In addition, a few reports have

suggested that melatonin supplements may help people stop

using long-term benzodiazepine therapy, which is habit-forming.

Blood pressure

medications

Melatonin may make blood pressure medications like

methoxamine (Vasoxyl) and clonidine (Catapress) less effective.

In addition, medications in a class called calcium channel

blockers may lower melatonin levels. Calcium channel blockers

include:

Nifedipine (Procardia)

Amlodipine (Norvasc)

Verapamil (Calan, Isoptin)

Diltiazem (Cardizem)

Felodipine (Plendil)

Nisoldipine (Sular)

Bepridil (Vascor)

Beta-blockers Use of beta-blockers may lower melatonin levels in the body.

Beta-blockers include:

Acebutolol (Sectral)

Atenolol (Tenormin)

Bisoprolol (Zebeta)

Carteolol (Cartrol)

Metoprolol (Lopressor, Toprol XL)

Nadolol (Corgard)

Propranolol (Inderal)

Anticoagulant

medications

Melatonin may increase the risk of bleeding from anticoagulant

medications such as warfarin (Coumadin).

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Interleukin-2 In one study of 80 cancer patients, use of melatonin along with

interleukin-2 led to more tumor regression and better survival

rates than treatment with interleukin-2 alone.

Nonsteroidal anti-

inflammatory drugs

(NSAIDs)

NSAIDs such as ibuprofen (Advil, Motrin) may lower levels of

melatonin in the blood.

Steroids and

immunosuppressant

medications

Melatonin may cause these medications to lose their

effectiveness. Do not take melatonin with corticosteroids or

other medications used to suppress the immune system.

Tamoxifen Preliminary research suggests that the combination of tamoxifen

(a chemotherapy drug) and melatonin may benefit some people

with breast and other cancers. More research is needed to

confirm these results.

Other Caffeine, tobacco, and alcohol can all lower levels of melatonin in

the body.

Vitamins

Many epileptic patients will benefit from supplementation with vitamins. In

many instances, epileptic seizures and other symptoms increase if the

patient is deficient in a specific vitamin (41). In other instances, patients may

benefit from an increase in nutritional supplementation as it will improve

basic body composition and increase the patient’s ability to withstand the

negative effects of epilepsy (42). The following section provides a thorough

overview of the vitamins most beneficial in epilepsy treatment: (41,43–51)

Folic acid

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Supplementation with folic acid on a daily basis is important for both women

as well as men. The vitamin named folic acid (also known as folate) is an

important part of the production of blood cells, of the function of some

nerves and to help prevent heart disease. Low levels (deficiency) of folic acid

can be the cause of intrauterine growth delay, inherited malformations,

miscarriages and neural tube defects in women, and heart disease in both

men and women.

For patients who have epilepsy, this is especially important since some

seizure medicines can cause low levels of folic acid by changing the way it is

absorbed in the body. Patients who take more than one seizure medicine

may be advised to take higher doses of folic acid. Babies born to women

who did not get enough folic acid early in their pregnancies are more likely

to have birth defects, especially a type called neural tube defects, which

affect the brain and spinal cord. The most well-known of these is spina

bifida, in which the spinal column is not completely closed. By the time a

woman knows for sure that she is pregnant, it may be too late to prevent

these defects.

Folic acid should be added to a person’s daily diet, either as food or as a

supplement, starting in the teenage years for women, and young adulthood

for men with epilepsy. Some providers recommend up to 4 mg per day for

patients who have been taking daily anti-seizure medications for many

years.

Epileptologists are now concerned that folic acid may be too low in persons

with epilepsy taking some antiepileptic drugs. Low serum and red blood cell

levels of folic acid in women of childbearing potential increase the risk of

fetal birth defects. For men and women, low levels of folic acid are

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associated with elevated homocysteine and an increased risk for

cardiovascular disease. A convincing argument now develops that routine

folic acid supplementation is important for women and men receiving

antiepileptic drugs.

Folic acid (vitamin B9) is a water-soluble B vitamin that is essential for DNA

repair, cell division, and normal cellular growth. Low folic acid levels during

pregnancy in women with epilepsy have been associated with fetal

malformation, and older enzyme-inducing antiepileptic drugs are known to

reduce serum folate levels. As mentioned earlier, profound deficiency of folic

acid during pregnancy has been associated with neural tube defects such as

spina bifida. Deficiency in adults has been associated with megaloblastic

anemia and peripheral neuropathy. In both men and women, low serum

levels of folate can increase homocysteine levels, which are correlated with

elevated cardiovascular risk.

Certain antiepileptic drugs, but not all agents, can potentially decrease folate

levels, either via hepatic enzyme induction and/or decreased absorption.

Addressing the question of which patients on AEDs need folic acid

supplementation is challenging because it depends on whether the patient is

pregnant or has a history of epilepsy. For example, the risk of having a

pregnancy complicated by a major congenital malformation (e.g., neural

tube defect) is doubled in epileptic women taking AEDs compared with those

women with a history of epilepsy not taking these agents. In fact, that risk is

tripled with AED polypharmacy, especially when valproic acid is included.

Additionally, many AEDs are used for conditions other than epilepsy, such as

chronic pain and mood disorders, but the effect of AEDs on folate has not

been adequately assessed in this population.

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There are some general guidelines about folic acid supplementation.

Consensus statements recommend 0.4-0.8 mg of folic acid per day in all

women planning a pregnancy. Ideally, this should be started at least 1

month prior to pregnancy if possible. These guidelines recommend higher

daily folic acid doses (4 mg/day) in women with a history of neural tube

defects. In addition, enzyme-inducing anticonvulsants, such as phenytoin,

carbamazepine, primidone, and phenobarbital, are known to decrease folate

levels, and valproic acid may interfere with folate metabolism. Other AEDs,

such as oxcarbazepine, lamotrigine, and zonisamide, do not appear to alter

folate levels.

Unfortunately, the effectiveness of folic acid supplementation for the

prevention of AED-induced teratogenicity and the appropriate dose of folic

acid for specific AEDs has not been determined. Not all studies designed to

determine effects of fetal AED exposure consistently demonstrate a

protective effect against congenital malformations with folic acid

supplementation. However, this may be due in part to inadequate dosage.

Because many pregnancies are unplanned, most authorities recommend that

folic acid supplementation be given routinely to all women of childbearing

potential at 0.4 mg/day. Women who have already had a child with a neural

tube defect are encouraged to consult with their clinician regarding

appropriate dosage, and those on AEDs should receive 0.4 - 4 mg/day.

Current data are inconclusive to support high-dose folic acid use in women

without epilepsy on AEDs for other indications, though supplemental folic

acid should not be regarded as harmful. For men and women on AEDs that

reduce folate levels, such as phenytoin, carbamazepine, primidone, and

phenobarbital, it seems prudent to monitor homocysteine and folate levels

and monitor for the development of megaloblastic anemia.

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The Comprehensive Epilepsy Center has developed guidelines for folate

supplementation for women of childbearing years. These guidelines were

established to enhance patient education and awareness of the potential

vitamin deficiencies that can occur when taking antiepilepsy medications

(AED's). They help to promote the general health of women, and minimize

potential birth defects associated with folate deficiency.

Folate (or folic acid) deficiency and medications used to treat epilepsy are

associated with an increased risk of birth defects. Specifically, they are

associated with spina bifida and anencephaly, two of the most common and

severe neurologic birth defects. Clinical studies have shown that

supplementing a woman's diet with folate can reduce this risk by 50-75%.

In order to reduce the risk of neural tube defects, the Center for Disease

Control and Prevention (CDC) recommends that all women who are capable

of becoming pregnant should take 0.4 mg of folate each day. Neural tube

defects occur early in the pregnancy, often before a woman is aware that

she is pregnant. In additional, about one-half of pregnancies in the United

States are unplanned. Therefore, supplementation with folate should

continue throughout a woman's reproductive years. A woman who has a

family history of neural tube defects or has a previous child born with neural

tube defects should receive folate supplementation of 4.0 mg per day.

The Comprehensive Epilepsy Center Guidelines for Folate supplementation

are as follows:

All women should supplement their diet by taking 1 prenatal

multivitamin each day. Prenatal multivitamins are available over-the-

counter (OTC) or by prescription. OTC prenatal multivitamins contain

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0.8 mg of folate while prescription prenatal multivitamins contain 1.0

mg of folate. Generic multivitamins are generally the least expensive,

followed in order of expense by brand name over-the-counter

vitamins, and finally prescription tablets.

A woman who is planning on becoming pregnant or who is pregnant,

and has a family history of neural tube defects, or has had a previous

child born with neural tube defects, or is on either Tegretol or

Depakote, should receive 3.0 mg of folate in addition to a prenatal

multivitamin.

All other women who are planning to become pregnant or are pregnant and

taking an antiepilepsy medication other than Tegretol or Depakote should

receive 1.0 mg of folate in addition to a prenatal multivitamin.

Calcium

Calcium is an important element in the body, and so important that an

individual has more calcium in his or her body than any other mineral.

Calcium is a necessary part of bone formation, development and repair. The

majority of calcium in the body is stored within bones, while the rest is in

the blood and the body’s other fluids. Abnormal calcium levels may result

in major health problems. Both hypocalcemia (low calcium levels), and

hypercalcemia (high calcium levels) can cause seizures. The main sources of

calcium are dairy products, such as milk, yogurt and ice cream. Green leafy

vegetables, such as broccoli and kale, canned sardines and shellfish are also

good sources of calcium.

Initially, low calcium levels may not give any warning signs. However, as the

level decreases, a person may feel confused and have hallucinations,

memory loss and depression. Because of calcium’s importance in muscle

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movement and the function of the nervous system, hypocalcemia can cause

muscle aches, spasms, stiffening of the muscles, and tingling sensations in

the face, mouth, lips, fingers and toes.

Low calcium levels can also cause several types of seizures, including the

following: tonic-clonic seizures, categorized by whole body shaking and loss

of consciousness; focal muscle seizures, during which a set of muscles

contract against a person’s will; and absence seizures, during which a

person appears to be staring off into space. Certain anti-seizure medications

can contribute to lowering calcium levels, especially when taken daily for a

long time period. This happens when the medication makes the liver work

harder than usual, and it causes the elimination of the calcium deposits from

the bone, leading to what is known as brittle bones, bone loss or

osteoporosis.

From a physiological perspective, it is logical that calcium supplementation

may be indicated when myoclonic seizures are encountered. For “when the

calcium ion concentration falls below about one half of normal, a person is

likely to experience tetanic contraction of muscles throughout the body

because of spontaneous nerve impulses in the peripheral nerves” (42). Since

calcitonin and the parathyroid hormone affect serum calcium concentrations,

it is possible that problems in the production of either can lead to limited

tetanic contractions.

Significant changes in important body chemicals such as calcium and

magnesium can cause seizures; so can a lack of certain vitamins. These

chemical changes may provoke a disturbance in the brain, or a single

seizure, by influencing the thresholds for firing. Calcium is a very important

mineral for the normal functioning of brain cells, and low levels of calcium

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(hypocalcemia) can cause seizures. Hypocalcemia can be a consequence of

severe kidney disease when too much calcium escapes from the kidney into

the urine. It may also, but rarely, be caused by a hormonal problem that has

the same effects. A deficiency of magnesium, a mineral that interacts with

calcium, may cause low blood calcium and, thus, seizures. With a ketogenic

diet, a calcium supplement must be taken every day to be nutritionally

complete.

There is growing evidence that elevated extra-cellular calcium levels and

homeostatic calcium control mechanisms may be factors in developing

acquired epilepsy (epilepsy that occurred after an injury). It is important to

evaluate the possible functional consequences of altered CA 2+ dynamics in

epileptogenesis. The ability of the neuron to restore CA 2+ loads to resting

[CA 2+] is regulated by CA 2+ homeostatic mechanisms. Increased or

prolonged entry of extracellular CA 2+ could contribute to the altered CA 2+

homeostatic mechanisms in epilepsy. It is important to note that cellular

calcium levels tend to be inversely correlated with extra-cellular calcium

levels. Thus, it does not seem unreasonable to conclude that those without

injury could have seizures caused by calcium problems.

Those that were on long-term anticonvulsant medications had higher levels

of calcium than non-medicated controls. This might suggest that one of the

reasons that some of these medications are continued long-term is that for

some people, they somehow increase the retention of calcium, which may

account for some of their anticonvulsant effects.

Some forms of juvenile myoclonic epilepsy can result from mutations of a Ca

2+ channel. This line of evidence suggests the involvement of channels

expressed in the brain in the pathogenesis of certain types of epilepsy. Ca

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2+ influx into excitable cells is a prerequisite for neurotransmitter release

and regulated exocytosis. Within the group of ten-cloned voltage-gated Ca

2+ channels, the Ca(v) 2.3-containing E-type Ca 2+ channels are involved in

various physiological processes, such as neurotransmitter release and

exocytosis together with other voltage-gated Ca 2+ channels of the Ca(v)1,

Ca(v)2 and Ca(v)3 subfamily. The interaction of Ca(v) 2.3 with the EF-hand

motif containing protein EFHC1 is involved in the etiology and pathogenesis

of juvenile myoclonic epilepsy. However, E-type Ca 2+ channels also exhibit

several subunit-specific features, most of which still remain poorly

understood. While they are not fully understood, it seems apparent that

calcium control mechanisms play some role in myoclonic seizures.

Mutations in the calcium-sensing receptor gene (CaSR) may result in

disorders of calcium homeostasis manifesting as familial benign hypocalciuric

hypercalcemia (FBHH), neonatal severe hyperparathyroidism (NSHPT) or

autosomal dominant hypocalcemia with hypercalciuria (ADHH). The ADHH

condition may result in asymptomatic hypocalcemia and a minority

experience seizures in infancy, which can recur into adulthood.

Even in generalized seizures, epileptics are generally mildly hypocalcemic,

especially in the period before the seizure. Stress, which releases

epinephrine and corticotropin, results in high serum citrate concentration,

which probably contributes to decreased serum [Ca2+] just before a seizure.

Long-term treatment of epileptic children with various anticonvulsant

medications was found to raise the TSH and diminished T3 and T4.

Calcitonin levels were lowered as well. Long-term use of certain

anticonvulsant medications tended to impair at least a portion of thyroid

function.

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Myoclonic seizures tend to be resistant to drug therapies. Since many anti-

epileptic medications impair thyroid function and/or somehow result in

increased calcium levels, perhaps a partial reason for their occasional

success with myoclonic seizures is the partial suppression of the thyroid

hormone calcitonin, which results in an increase of serum calcium levels.

There are scattered reports that the anticonvulsant medications

phenobarbital, carbamazepine, valproate, lamotrigine, gabapentin, and

vigabatrin can cause or induce myoclonic seizures in epileptics who had not

been experiencing those types of seizures. It is possible this occurs because

some anticonvulsant medications can reduce vitamin D levels. Other

researchers have thus suggested supplemental vitamin D when taking

certain anticonvulsant medications.

Myoclonic seizures can have an appearance of a limited tetanic contraction

associated with insufficient calcium levels. It is important to note that

others, while not specifically discussing myoclonic seizures, have also

suggested that somehow increasing calcium levels should be looked at for

the treatment of epileptics. Hence, it may be wise to consider nutritional

interventions that affect calcium levels as a first-line treatment

Currently, this is rarely the case. Even though some antiepileptic drugs could

also worsen some types of seizures, it is known that other therapies can be

efficient in refractory epilepsies; steroids, vague nerve stimulation, ketogenic

diet and surgery, nutritional therapies (especially outside the ketogenic diet)

seem to be often overlooked. It should be noted that it is theoretically

possible that, for some types of seizures, calcium could be contraindicated.

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Yet it is not unheard of that nutrition, including calcium supplementation,

should be considered as a first-line treatment for intractable forms of

epilepsy, as others have sometimes advocated it (though this investigator

appears to be the first advocating supplemental calcium, vitamin D, etc., as

first-line nutrients, as well as first to advise nutrients specifically for

myoclonic seizures). One of the reasons that nutrition should be considered

as a front-line therapy is that it is low risk.

Consumption of calcium-containing foods and/or calcium-containing

supplements is so safe that, although calcium can react with some

medications, over dosage has not been reported with calcium supplements.

Forms other than calcium carbonate are preferred, as calcium carbonate

may cause gastrointestinal side reactions such as constipation, bloating, gas

and flatulence. Prolonged use of large doses of calcium carbonate — greater

than 12 grams daily (about 5 grams of elemental calcium) — may lead to

milk-alkali syndrome, nephrocalcinosis and renal insufficiency.

There is no specific quantitative recommendation for each possible

substance that could affect calcium levels, as the amount needed appears to

vary by individual (as well as size in the case of children). But irrespective of

the quantities, it does appear reasonable to conclude that calcium control

mechanisms can play a causal role in myoclonic seizures and that calcium

and other nutrients should be considered as possible front-line therapies for

these hard to treat myoclonic seizures.

Vitamin D

Vitamin D is a necessary part in the process of proper breakdown and use of

calcium. Because of this, vitamin D deficiency caused or worsened by daily

use of anti-seizure medications for a long time can make the bones very soft

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and brittle, causing them to break more easily. Adding vitamin D to the daily

diet can prevent this. In addition, patients taking anti-seizure medications

should increase their calcium intake every day. Exposure to sunlight is a

natural way to speed up the body’s ability to produce vitamin D.

Nearly half of people with epilepsy are also vitamin D deficient, but despite

this well-known association, only a single study has been published on the

effect of vitamin D for seizure control in the last 40 years. That study

revealed that treating epileptic patients with vitamin D2 – the far inferior

type of synthetic vitamin D – reduced the number of seizures, and in 1974

researchers concluded “the results may support the concept that epileptics

should be treated prophylactically with vitamin D (46).“

Now, nearly four decades later researchers have again revealed that “the

normalization of serum vitamin 25(OH)D [vitamin D] level has an

anticonvulsant [anti-seizure] effect (50).” The findings are even more

important given that people with epilepsy face an even greater risk of

vitamin D deficiency than the general population (and even the general

population is vastly vitamin D deficient). The reasons are two-fold, with the

first being that having frequent seizures may interfere with a person’s ability

to get outdoors and stay active.

If an individual spends most of his or her time inside, they will miss out on

regular sun exposure, which is key for the natural production of vitamin D.

Even exposing the skin to sunlight through a windowpane will prevent the

entry of the UVB rays, which are the specific wavelength that produces

vitamin D in the skin. It is crucial for epilepsy patients to get outside and

experience direct skin contact with the sunlight instead of sunning in a

sunroom, for instance. Second, anti-epileptic drugs that are often given to

epilepsy patients can interfere with vitamin D metabolism, leading to

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deficiency. If these drugs are taken, it is especially crucial that vitamin D

levels are actively monitored to avoid this side effect.

Vitamin D has a significant impact on epileptic seizures because epilepsy is a

disorder of the central nervous system, particularly of the brain. Vitamin D is

a vitamin that is also a neuroregulatory steroidal hormone that influences

nearly 3,000 different genes in the body. Vitamin D receptors can be found

in the brain, spinal cord, and central nervous system, and may enhance the

amount of important chemicals in the brain needed to protect brain cells.

Surgical Options

A number of individuals with epilepsy may benefit from surgical intervention.

There are a variety of surgical procedures that can help with various aspects

of the disorder. While medication is effective at controlling most seizure

activity, approximately thirty percent of individuals will not respond to

pharmacologic treatment and will require more advanced therapy (52). These

individuals often benefit from surgery.

There are three primary forms of surgery that are used to treat individuals

with epilepsy: (53)

Surgery to remove the area of the brain producing seizures

Surgery to interrupt the nerve pathways through which seizure

impulses spread within the brain

Surgery to implant a device used to treat epilepsy

Surgery is an invasive procedure and should only be considered if the

section of the brain where the seizures originate can be clearly identified (54).

In addition, the physician must ensure that surgery will not negatively affect

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any areas that are responsible for critical functions (55). A thorough

assessment is required before determining if surgery is a viable option.

There are a number of different surgical procedures that can be used to treat

epilepsy. The specific type of surgery performed on a patient will be

determined based upon the type of seizures the patient is experiencing and

the area of the brain where seizure activity originates (52). The following

section provides an overview of the risks and benefits of various surgical

procedures (14,56–83).

(Photo courtesy of: http://www.epilepsy.net.in/images/epilepsy_surgery.jpg)

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Procedure Description and Benefits/Risks

Vagus Nerve

Stimulation

(VNS)

VNS is a palliative technique that involves surgical implantation of a

stimulating device. VNS is FDA approved to treat medically refractory

focal-onset epilepsy in patients older than 12 years. Some studies

demonstrate its efficacy in focal-onset seizures and in a small number

of patients with primary generalized epilepsy. Randomized studies

showed modest efficacy at 3 months, but post marketing experience

showed delayed improvement in another group of patients.

In August 2013, the American Academy of Neurology issued an

update to its 1999 guidelines on the use of VNS for epilepsy. VNS is

currently indicated for patients older than 12 years with medically

intractable partial seizures who are not candidates for potentially

curative surgical resections, as well as for the adjunctive long-term

treatment of chronic or recurrent depression in patients older than 18

years with a major depressive episode not adequately relieved by 4

or more antidepressant treatments. Recent reports also indicate long-

term efficacy and successful VNS use in pediatric epilepsy and other

seizure types and syndromes.

Key recommendations of the updated guidelines include the

following:

VNS may be considered for (1) the adjunctive treatment of

partial or generalized epilepsy in children, (2) seizures

associated with Lennox-Gastaut syndrome, and (3) improving

mood in adults with epilepsy

VNS may have improved efficacy over time

Children should be carefully monitored for site infection after

VNS implantation

According to the manufacturer's registry, efficacy of the stimulating

device at 18 months is 40-50%, where efficacy is defined as a seizure

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reduction of 50% or more. Many patients report improvement in

seizure intensity and general mood. However, seizure-free rates for

pharmacologically intractable focal-onset epilepsy are less than 10%.

A meta-analysis of VNS clinical studies reported an average reduction

in seizures of at least 50% in approximately 50% of patients at last

follow-up. Although VNS was not initially FDA approved for children

or patients with generalized epilepsy, the authors also found that

these groups benefitted significantly from VNS.

Positive predictors of a favorable outcome with VNS therapy include

posttraumatic epilepsy and tuberous sclerosis. Few patients achieve

complete seizure freedom with VNS, and about a quarter of patients

receive no benefit in their seizure frequency. Some patients have

clinical improvement in terms of milder and shorter seizures.

Multiple Subpial

Transection

Multiple subpial transection was pioneered as an alternative to

removal of brain tissue. It is used to control partial seizures

originating in areas that cannot be safely removed. For example, if

the seizure focus involves the dominant temporal-lobe language area

(Wernicke’s area), which is critical for comprehension, the removal of

this area to control seizures would cause a devastating complication:

the inability to understand spoken or written language. Similarly, if

the primary motor area is part of the seizure focus, its removal would

cause permanent weakness on the opposite side of the body.

The operation involves a series of shallow cuts (transections) into the

cerebral cortex. The transections are made only as deep as the gray

matter, approximately a quarter of an inch deep. Because of the

complex way in which the brain is organized, these cuts are thought

to interrupt some fibers that connect neighboring parts of the brain,

but they do not appear to cause long-lasting impairment in the

critical functions served by these areas. Examination of brain tissue

after multiple subpial transections reveals that some nerve cells are

destroyed.

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Sometimes, brain seizures begin in a vital area of the brain -- for

example, in areas that control movement, feeling, language, or

memory. When this is the case, a relatively new epilepsy treatment

called multiple subpial transection (MST) may be an option. MST

stops the seizure impulses by cutting nerve fibers in the outer layers

of the brain (gray matter), sparing the vital functions concentrated in

the deeper layers of brain tissue (white matter).

Most people with epilepsy can control their seizures with medication.

However, about 20% of people with epilepsy do not improve with

drugs. In some cases, surgery to remove the part of the brain

causing the seizures may be recommended.

MST may be an option for people who do not respond to medication

and whose seizures begin in areas of the brain that cannot be safely

removed. In addition, there must be a reasonable chance that the

person will benefit from surgery. MST may be done alone or with the

removal of a section of brain tissue (resection). MST also may be

used as a treatment for children with Landau-Kleffner syndrome

(LKS), a rare childhood brain disorder which causes seizures and

affects the parts of the brain that control speech and comprehension.

Candidates for MST undergo an extensive pre-surgery evaluation -

including seizure monitoring, electroencephalography (EEG),

magnetic resonance imaging (MRI), and positron emission

tomography (PET). These tests help to pinpoint the area in the brain

where the seizures occur and determine if surgery is feasible.

Another test to assess electrical activity in the brain is EEG-video

monitoring, in which video cameras are used to record seizures as

they occur, while the EEG monitors the brain's activity. In some

cases, invasive monitoring - in which electrodes are placed inside the

skull over a specific area of the brain - is also used to further identify

the tissue responsible for seizures.

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MST requires exposing an area of the brain using a procedure called a

craniotomy. ("Crani" refers to the skull and "otomy" means "to cut

into.") After the patient is put to sleep with anesthesia, the surgeon

makes an incision (cut) in the scalp, removes a piece of bone and

pulls back a section of the dura, the tough membrane that covers the

brain. This creates a "window" in which the surgeon inserts his or her

surgical instruments. The surgeon utilizes information gathered

during pre-surgical brain imaging to help identify the area of

abnormal brain tissue and avoid areas of the brain responsible for

vital functions.

Using a surgical microscope to produce a magnified view of the brain,

the surgeon makes a series of parallel, shallow cuts (transections) in

gray matter, just below the pia mater (subpial), the delicate

membrane that surrounds the brain (it lies beneath the dura). The

cuts are made over the entire area identified as the source of the

seizures. After the transactions are made, the dura and bone are

fixed back into place, and the scalp is closed using stitches or staples.

There may be bleeding at the site of the transection, but the

procedure is generally well tolerated. Major complications appear to

be rare. Transections in language areas may cause mild impairments

in the language function served by that area. The risks and benefits

of multiple subpial transections need to be better defined.

Multiple subpial transections can help reduce or eliminate seizures

arising from vital functional cortical areas. Transections have been

used successfully in Landau-Kleffner syndrome, a disorder in which

language problems appear in a child whose language was previously

developing normally. One concern is that the epileptic activity may

recur after a period of 2 to 20 months. It is uncertain whether this

procedure can achieve long-term seizure control

Temporal Lobe

Resection

The most common surgical procedure performed for epilepsy is the

removal of a portion of the temporal lobe, or temporal lobectomy.

These brain structures play an important role in the generation or

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propagation of the majority of temporal lobe seizures. In most cases,

a modest portion of the brain measuring approximately 2 inches long

is removed. The temporal lobes are important in memory, emotion

and language comprehension. However, extensive preoperative

assessments (MRI, Wada tests, PET scans) ensure that removal of

the area causing seizures will not disrupt any of these critical

functions.

The largest part of the brain, the cerebrum, is divided into four paired

sections - the frontal, parietal, occipital, and temporal lobes. Each

lobe controls a specific group of activities. The temporal lobe, located

on either side of the brain just above the ear, plays an important role

in hearing, language, and memory. The most common type of

epilepsy in teens and adults originates in the temporal lobe, the

seizure focus.

Temporal Lobe Resection

A temporal lobe resection is a surgery performed on the brain to

control seizures. In this procedure, brain tissue in the temporal lobe

is resected, or cut away, to remove the seizure focus. The anterior

(front) and mesial (deep middle) portions of the temporal lobe are

the areas most often involved.

Temporal lobe resection may be an option for people with epilepsy

whose seizures are disabling and/or not controlled by medication, or

when the side effects of medication are severe and significantly affect

the person's quality of life. In addition, it must be possible to remove

the brain tissue that contains the seizure focus without causing

damage to areas of the brain responsible for vital functions, such as

movement, sensation, language, and memory.

Candidates for temporal lobe resection undergo an extensive pre-

surgery evaluation - including seizure monitoring,

electroencephalography (EEG), magnetic resonance imaging (MRI),

and positron emission tomography (PET). These tests help to pinpoint

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the seizure focus within the temporal lobe and to determine if surgery

is possible.

A temporal lobe resection requires exposing an area of the brain

using a procedure called a craniotomy. After the patient is put to

sleep with anesthesia, the surgeon makes an incision in the scalp,

removes a piece of bone and pulls back a section of the dura, the

tough membrane that covers the brain. This creates a "window" in

which the surgeon inserts special instruments for removing the brain

tissue. Surgical microscopes also are used to give the surgeon a

magnified view of the area of the brain involved. The surgeon utilizes

information gathered during the pre-operative evaluation - as well as

during surgery - to define, or map out, the route to the correct area

of the temporal lobe.

In some cases, a portion of the surgery is performed while the

patient is in a ''twilight state'' - awake but under sedation - so that

the patient can help the surgeon find and avoid areas of the brain

responsible for vital functions. While the patient is awake, the doctor

uses special probes to stimulate different areas of the brain. At the

same time, the patient is asked to count, identify pictures, or perform

other tasks. The surgeon can then determine the area of the brain

associated with each task. After the brain tissue is removed, the dura

and bone are fixed back into place, and the scalp is closed using

stitches or staples.

Permanent complications associated with temporal lobe resection

surgery are very low. Mortality is less than 0.1% and permanent

unexpected morbidity less than 1%. In dominant hemisphere

resections, temporary language difficulties are seen in 10% of the

cases although these usually resolve. An upper quadrantanopsia

(partial upper peripheral vision loss) is expected in large temporal

resections, but seen in less than 25% of the patients. Memory

impairment rarely occurs from temporal lobectomies because of

extensive preoperative testing of language and memory functions.

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The success rate for seizure control in temporal lobectomy varies:

60%-70% of patients are free of seizures that impair

consciousness or cause abnormal movements, but some still

experience auras

20%-25% of patients have some seizures but are significantly

improved (greater than 85% reduction of complex partial and

tonic-clonic seizures)

10%-15% of patients have no worthwhile improvement

Therefore, over 85% of patients enjoy a marked improvement in

seizure control. Most of them need less medication after surgery.

Approximately 25% of those who are seizure-free eventually can

discontinue antiepileptic drugs

Lesionectomy

Twenty five percent of patients with epilepsy will have lesions

identified by MRI as the cause of recurrent seizures. Abnormalities

such as low-grade astrocytomas, cortical dysplasias, cavernous

angiomas, and areas of focal atrophy are the common causes of

refractory seizures. Since surgical removal of these lesions can result

in complete seizure control in many patients, the patient is

considered an excellent candidate for epilepsy surgery depending on

the location of the lesion and its relationship to eloquent cortex. If

the seizures have been present for many years then invasive

monitoring is often recommended to further identify the involvement

of the adjacent cortical rim around the lesion. When lesions are

within the non-dominant temporal lobe, removal of the lesion along

with a temporal lobectomy yields excellent results in over 80% of

cases.

A lesionectomy is the surgical removal of lesions. MRI identifies small

lesions as the cause of recurrent seizures in up to 25% of patients.

Common types of lesions include low grade astrocytomas, cortical

dysplasias, cavernous angiomas, and areas of focal atrophy.

Functional The largest part of the brain, the cerebrum, can be divided down the

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Hemispherectomy

middle lengthwise into two halves, called hemispheres. A deep

groove splits the left and right hemispheres, which communicate

through a thick band of nerve fibers called the corpus callosum. Each

hemisphere is further divided into four paired sections, called lobes -

the frontal, parietal, occipital, and temporal lobes.

The two different sides or hemispheres are responsible for different

types of activities. The left side of the brain controls the right side of

the body and vice versa. For most people, the ability to speak and

understand the spoken word is a function of the left side of the brain.

A functional hemispherectomy is a procedure in which portions of one

hemisphere - which are causing the seizures - are removed, and the

corpus callosum, which connects the two sides of the brain, is cut.

This disconnects communication between the two hemispheres,

preventing the spread of electrical seizures from one side of the brain

to the other. As a result, the person usually has a marked reduction

in physical seizures.

This procedure generally is used only for people with epilepsy who do

not experience improvement in their condition after taking many

different medications and who have severe, uncontrollable seizures.

This type of epilepsy is more likely to be seen in young children who

have an underlying disease, such as Rasmussen's encephalitis or

Sturge-Weber syndrome, which has damaged the hemisphere.

Candidates for functional hemispherectomy undergo an extensive

pre-surgery evaluation - including seizure monitoring,

electroencephalography (EEG), and magnetic resonance imaging

(MRI). These tests help the doctor identify the damaged parts of the

brain and confirm that it is the source of the seizures. An intracarotid

amobarbital test, also called a WADA test, is done to determine which

hemisphere is dominant for critical functions such as speech and

memory. During this test, each hemisphere is alternately injected

with a medication to put it to sleep. While one side is asleep, the

awake side is tested for memory, speech, and ability to understand

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speech.

A functional hemispherectomy requires exposing the brain using a

procedure called a craniotomy. Surgical microscopes are utilized to

give the surgeon a magnified view of the brain structures. During the

procedure, the surgeon removes portions of the affected hemisphere,

often taking all of the temporal lobe but leaving the frontal and

parietal lobes. The surgeon also gently separates the hemispheres to

access and cut the corpus callosum. After the tissue is removed, the

dura and bone are fixed back into place, and the scalp is closed using

stitches or staples.

The patient generally stays in an intensive care unit for 24 to 48

hours and then stays in a regular hospital room for three to four

days. Most people who have a functional hemispherectomy will be

able to return to their normal activities, including work or school in

six to eight weeks after surgery. Most patients will need to continue

taking anti-seizure medication, although some may eventually be

able to stop taking medication or have their dosages reduced.

About 85% of people who have a functional hemispherectomy will

experience significant improvement in their seizures, and about 60%

will become seizure-free. In many cases, especially in children, the

remaining side of the brain takes over the tasks that were controlled

by the section that was removed. This often improves a child's

functioning and quality of life, as well as reduces or eliminates the

seizures.

The following symptoms may occur after a functional

hemispherectomy, although they generally go away over time and

with therapy:

Scalp numbness.

Nausea.

Muscle weakness on the affected side of the body.

Puffy eyes.

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Feeling tired or depressed.

Difficulty speaking, remembering, or finding words.

Headaches.

The risks associated with a functional hemispherectomy include the

following:

Risks associated with surgery, including infection, bleeding,

and an allergic reaction to anesthesia.

Loss of movement or sensation on the opposite side of the

body.

Swelling in the brain.

Delayed development.

Loss of peripheral (side) vision.

Failure to control seizures.

Corpus

Callosotomy

The corpus callosum is a band of nerve fibers located deep in the

brain that connects the two halves (hemispheres) of the brain. It

helps the hemispheres share information, but it also contributes to

the spread of seizure impulses from one side of the brain to the

other. A corpus callosotomy is an operation that severs (cuts) the

corpus callosum, interrupting the spread of seizures from hemisphere

to hemisphere. Seizures generally do not completely stop after this

procedure (they continue on the side of the brain in which they

originate). However, the seizures usually become less severe, as they

cannot spread to the opposite side of the brain.

A corpus callosotomy, sometimes called split-brain surgery, may be

performed in people with the most extreme and uncontrollable forms

of epilepsy, when frequent seizures affect both sides of the brain.

People considered for corpus callosotomy are typically those who do

not respond to treatment with antiseizure medications.

Candidates for corpus callosotomy undergo an extensive pre-surgery

evaluation - including seizure monitoring, electroencephalography

(EEG), magnetic resonance imaging (MRI), and positron emission

tomography (PET). These tests help the doctor pinpoint where the

seizures begin and how they spread in the brain. It also helps the

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doctor determine if a corpus callosotomy is an appropriate treatment.

A corpus callosotomy requires exposing the brain using a procedure

called a craniotomy. After the patient is put to sleep with anesthesia,

the surgeon makes an incision in the scalp, removes a piece of bone

and pulls back a section of the dura, the tough membrane that covers

the brain. The surgeon inserts special instruments for disconnecting

the corpus callosum, gently separates the hemispheres to access the

corpus callosum. Surgical microscopes are used to give the surgeon a

magnified view of brain structures.

In some cases, a corpus callosotomy is done in two stages. In the

first operation, the front two-thirds of the structure is cut, but the

back section is preserved. This allows the hemispheres to continue

sharing visual information. If this does not control the serious

seizures, the remainder of the corpus callosum can be cut in a second

operation. After the corpus callosum is cut, the dura and bone are

fixed back into place, and the scalp is closed using stitches or staples.

The patient generally stays in the hospital for two to four days. Most

people having a corpus callosotomy will be able to return to their

normal activities, including work or school, in six to eight weeks after

surgery. The hair over the incision will grow back and hide the

surgical scar. The person will continue taking antiseizure drugs.

Complications of corpus callosotomy are greater than with frontal or

temporal lobe surgery. Behavioral, language, and other problems

may affect function and the quality of life, but serious problems are

temporary or uncommon. The potential risks of callosotomy must be

weighed against its possible benefits, such as a reduction in the

frequency of seizures that cause injury and other problems. The

persons most susceptible to behavioral problems after callosotomy

are those in whom language and motor dominance are controlled by

different hemispheres. In left-handed persons, for example, the left

side of the brain controls language, but the right side of the brain

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controls movement. Some of the problems resulting from callosotomy

are caused by injury to the frontal lobes during the operation. Since

the corpus callosum is buried deep between the frontal lobes, the

middle portions of these lobes must be separated, which poses some

risk. Surgical advances may help to minimize this risk.

Seizure frequency is reduced by an average of 70% to 80% after

partial callosotomy and 80% to 90% after complete callosotomy.

Partial seizures are often unchanged, but they may be improved or

worsened. In many cases, especially after partial callosotomy,

seizures are less frequent but persist.

Extratemporal

Cortical Resection

Extra-temporal seizure surgery constitutes about a quarter of the

surgical procedures for epilepsy and includes resection of the frontal

lobes, parietal lobes or occipital lobes. These resections are guided by

localization from invasive subdural electrodes and, if necessary,

detailed cortical functional mapping. Extra-temporal resections are

individualized to the seizure onset focus, the type of seizure or

syndrome, and the functional mapping, which defines a safe resection

boundary. Motor and sensory cortex and language cortex localization

is performed and greatly minimizes neurological deficits from

surgery.

The largest part of the brain, the cerebrum, is divided into four paired

sections, called lobes - the frontal, parietal, occipital, and temporal

lobes. Each lobe controls a specific group of activities. The temporal

lobe is the most common ''seizure focus,'' the area where most

seizures start, in teens and adults.

However, epileptic seizures can be ''extratemporal,'' or outside of the

temporal lobe, originating in the frontal, parietal or occipital lobes, or

even more than one lobe. If this is the case, extratemporal cortical

resection surgery may be warranted in some cases. An extratemporal

cortical resection is an operation to resect, or cut away, brain tissue

that contains a seizure focus. Extratemporal means the tissue is

located in an area of the brain other than the temporal lobe. The

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frontal lobe is the most common extratemporal site for seizures. In

some cases, tissue may be removed from more than one area/lobe of

the brain.

Extratemporal cortical resection may be an option for people with

epilepsy whose seizures are disabling and/or not controlled by

medications, or when the side effects of the medication are severe

and significantly affect the person's quality of life. In addition, it must

be possible to remove the brain tissue that contains the seizure focus

without causing damage to areas of the brain responsible for vital

functions, such as movement, sensation, language, and memory.

Candidates for extratemporal cortical resection undergo an extensive

pre-surgery evaluation including video electroencephalographic (EEG)

seizure monitoring, magnetic resonance imaging (MRI), and positron

emission tomography (PET). Other tests include neuropsychological

memory testing, the Wada test (to determine which side of the brain

controls language function), Single Photon Emission Computed

Tomography (SPECT), and magnetic resonance spectroscopy. These

tests help to pinpoint the seizure focus and determine if surgery is

possible.

An extratemporal cortical resection requires exposing an area of the

brain using a procedure called a craniotomy. After the patient is put

to sleep (general anesthesia), the surgeon makes an incision in the

scalp, removes a piece of bone and pulls back a section of the dura,

the tough membrane that covers the brain. The surgeon inserts

special instruments to remove brain tissue. Surgical microscopes are

used to give the surgeon a magnified view of the area of the brain

involved. The surgeon utilizes the information gathered during the

pre-operative evaluation - as well as during surgery - to define, or

map out, the route to the correct area of the brain.

In some cases, a portion of the surgery is performed while the

patient is awake, using medication to keep the person relaxed and

pain-free. This is done so that the patient can help the surgeon find

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and avoid areas in the brain responsible for vital functions such as

brain regions of language and motor control. While the patient is

awake, the doctor uses special probes to stimulate various areas of

the brain. At the same time, the patient may be asked to count,

identify pictures, or perform other tasks. The surgeon can then

identify the area of the brain associated with each task. After the

brain tissue is removed, the dura and bone are fixed back into place,

and the scalp is closed using stitches or staples.

The risk of a major complication, such as a stroke, is about 1% in

these types of surgery. The risk of behavioral changes is higher than

with temporal lobectomy although these are often difficult to measure

and define. Personality, motivation, ability to plan and to follow up on

a multistep process, ability to organize actions over time, social

graces, and demeanor are among the behaviors that the frontal lobes

help to serve. In parietal and occipital lobectomies, there may be a

risk of losing touch sensation or vision.

Results of surgical management for extratemporal epilepsy vary

depending upon seizure types, invasive mapping, and epilepsy

syndrome. Overall:

50%-60% of patients are free of seizures that impair

consciousness or cause abnormal movements.

20%-40% of patients are markedly improved (more than 90%

reduction of complex partial and tonic-clonic seizures)

20%-30% of patients have no worthwhile improvement.

Although extratemporal surgical cure rates are not as high as

temporal surgery rates, patients with well-defined epileptic

zones limited to smaller areas of the brain which can be

resected do better than in cases of widespread seizure areas.

It is in the area of extratemporal seizures that our institution

has improving success rates as these more difficult problems

are managed with the latest techniques, imaging modalities

and our greater understanding.

Implantable The NeuroPace RNS System, a device that is implanted into the

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neurostimulator

cranium, senses and records electrocorticographic patterns and

delivers short trains of current pulses to interrupt ictal discharges in

the brain. The Neurological Devices panel of the FDA concluded that

this device was safe and effective in patients with partial-onset

epilepsy in whom other antiepileptic treatment approaches have

failed and that the benefits outweigh the risks.

In November 2013, the FDA approved the NeuroPace RNS System for

the reduction of seizures in patients with drug-resistant epilepsy.

Approval was based on a clinical trial involving 191 subjects with

drug-resistant epilepsy. The neurostimulator was implanted in all of

these patients but activated in only half of them. After 3 months, the

average number of seizures per month in patients with the activated

device fell by a median of 34%, compared with an approximately

19% median reduction in patients with an unactivated device.

Anti-Epileptic Medication

Anti-epileptic medication is often a necessary component of treatment.

Many patients will require pharmacologic therapy to control seizures. In

most instances, medication will be used in conjunction with other non-

pharmacologic therapies to provide a comprehensive approach to treatment

(17). Utilizing numerous options together provides the best means of seizure

control, especially in patients who experience severe or frequent seizures.

The following table provides an overview of the various types of anti-

epileptic medication: (12,55,84–101)

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Valproate

Sodium

Carbamazepine

Valproate sodium (Depacon, generic), valproic acid (Depakene,

generic), and divalproex sodium (Depakote, generic) are

anticonvulsants that are chemically very similar to each other. (In this

report, they are referred to together as valproate.) Valproate products

are the most widely prescribed anti-epileptic drugs worldwide. They are

the first choice for patients with generalized seizures and are used to

prevent nearly all other major seizures as well.

Side Effects: These drugs have a number of side effects that vary

depending on dosage and duration. Most side effects occur early in

therapy and then subside. The most common side effects are upset

stomach and weight gain. Less common side effects include dizziness,

hair thinning and loss, and difficulty concentrating.

Serious side effects include a higher risk for serious birth defects than

other AEDs especially if taken during the first trimester of pregnancy.

In particular, these drugs are associated with facial cleft deformities

(cleft lip or palate) and cognitive impairment.

Liver damage or failure is a rare but extremely dangerous side effect

that usually affects children under 2 years of age who have birth

defects and are taking more than one antiseizure drug. Pancreatitis

(inflammation of the pancreas) and kidney problems are also rare but

serious side effects.

Carbamazepine (Tegretol, Equetro, Carbatrol, generic) is used for many

types of epilepsy. It is taken alone or in combination with other drugs.

In addition to controlling seizures, it may help relieve depression and

improve alertness. A chewable form is available for children.

Side Effects: Different side effects may develop or resolve at different

points during treatment. Initial side effects may include the following:

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Double vision, headache, sleepiness, dizziness, and stomach upset.

These usually subside after a week and can be greatly reduced by

starting with a small dose and building up gradually.

Some people experience visual disturbances, ringing in the ears,

agitation, or odd movements when drug levels are at their peak. The

extended-release form of carbamazepine (Carbatrol) may help reduce

these symptoms.

Serious side effects are less common but can include: skin reactions,

including toxic epidermal necrolysis and Stevens-Johnson syndrome, so

severe the drug has to be discontinued develop in about 6% of

patients. These skin reactions cause skin lesions, blisters, fever,

itching, and other symptoms. People of Asian ancestry have a 10 times

greater risk for skin reactions than other ethnicities.

A decrease in white blood cells occurs in about 10% of those taking the

drug. This is generally not serious unless infection accompanies it.

Other blood conditions can arise that are also potentially serious.

Patients should be sure to inform their doctors if they have any sign of

irregular heartbeats, sore throat, fever, easy bruising, or unusual

bleeding.

Long-term therapy can cause bone density loss (osteoporosis) in

women, who should take preventive calcium and vitamin D

supplements to improve bone mass.

Children are at higher risk for behavioral problems.

Note: Grapefruit, Seville oranges, and tangelos can increase

carbamazepine's blood levels and risk of adverse effects.

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Phenytoin

Phenytoin (Dilantin, generic) is effective for adults who have the

following seizures or conditions:

Grand mal seizures

Partial seizures

Status epilepticus

Can be effective for people with head injuries who are at high risk for

seizures. This drug is not useful for the following seizures:

Petit mal seizures

Myoclonic seizures

Atonic seizures

Side effects are sometimes difficult to control. Some people may

develop a toxic response to normal doses, while others may require

higher doses to achieve benefits. As with any drug, side effects

generally depend on dosage and duration.

Side effects may include the following:

Excess body hair, eruptions and coarsening of the skin, and

weight loss

Gum disease

Staggering, lethargy, nausea, depression, eye-muscle problems,

anemia, and an increase in seizures can occur as a result of

excessive doses.

Liver damage may develop in rare cases.

Bone density loss from long-term therapy. Patients should take

preventive calcium and vitamin D supplements and exercise

regularly to improve bone mass.

Severe and even rare life-threatening skin reactions (Stevens-

Johnson syndrome, toxic epidermal necrosis)

A possible increased risk for birth defects (cleft palate, poor

thinking skills)

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Barbiturates

(Phenobarbital

and Primidone)

Phenobarbital (Luminal, generic), also called phenobaritone, is a

barbiturate anticonvulsant. Primidone (Mysoline, generic) is converted

in the body to phenobarbital, and has the same benefits and adverse

effects.

Barbiturates may be used to prevent grand mal (tonic-clonic) seizures

or partial seizures. They are no longer typically used as first-line drugs,

although they may be the initial drugs prescribed for newborns and

young children.

Side Effects: Phenobarbital has fewer toxic effects on other parts of the

body than most anti-epileptic drugs, and drug dependence is rare,

given the low doses used for treating epilepsy. Nevertheless, many

patients experience difficulty with side effects.

Patients sometimes describe their state as "zombie-like." The most

common and troublesome side effects are:

Drowsiness

Memory problems

Problems with tasks requiring sustained performance

Problems with motor skills

Hyperactivity in some patients, particularly in children and the

elderly

Depression in some adults

When taken during pregnancy, phenobarbital, like phenytoin and

valproate, may lead to impaired cognitive function in the child. There

has been some evidence that phenobarbitol may cause heart problems

in the fetus.

Ethosuximide

and Similar

Drugs

Ethosuximide (Zarontin, generic) is used for petit mal (absence)

seizures in children and adults when the patient has experienced no

other type of seizures. Methsuximide (Celontin), a drug similar to

ethosuximide, may be suitable as an add-on treatment for intractable

epilepsy in children.

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Side Effects: This drug can cause stomach problems, dizziness, loss of

coordination, and lethargy. In rare cases, it may cause severe and even

fatal blood abnormalities. Periodic blood counts are recommended for

patients taking this drug.

Clonazepam

Clonazepam (Klonopin, generic) is recommended for myoclonic and

atonic seizures that cannot be controlled by other drugs and for

Lennox-Gastaut epilepsy syndrome. Although clonazepam can prevent

generalized or partial seizures, patients generally develop tolerance to

the drug, which causes seizures to recur.

Side Effects: People who have had liver disease or acute angle

glaucoma should not take clonazepam, and people with lung problems

should use the drug with caution. Clonazepam can be addictive, and

abrupt withdrawal may trigger status epilepticus. Side effects include

drowsiness, imbalance and staggering, irritability, aggression,

hyperactivity in children, weight gain, eye muscle problems, slurred

speech, tremors, skin problems, and stomach problems.

Lamotrigine

Lamotrigine (Lamictal, generic) is approved as add-on (adjunctive)

therapy for partial seizures, and generalized seizures associated with

Lennox-Gastaut syndrome, in children aged 2 years and older and in

adults. Lamotrigine is also approved as add-on therapy for treatment of

primary generalized tonic-clonic (PGTC) seizures, also known as “grand

mal” seizures, in children aged 2 years and older and adults.

Lamotrigine can be used as a single drug treatment (monotherapy) for

adults with partial seizures. Birth control pills lower blood levels of

lamotrigine.

Side Effects: Common side effects include dizziness, headache, blurred

or double vision, lack of coordination, sleepiness, nausea, vomiting,

insomnia, and rash. Although most cases of rash are mild, in rare cases

the rash can become very severe. The risk of rash increases if the drug

is started at too high a dose or if the patient is also taking valproate.

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(Serious rash is more common in young children who take the drug

than it is in adults.) Rash is most likely to develop within the first 8

weeks of treatment. The medical provider should be immediately

notified for development of a rash, even if it is mild.

Lamotrigine may cause aseptic meningitis. Symptoms of meningitis

may include headache, fever, stiff neck, nausea, vomiting, rash, and

sensitivity to light. Patients who take lamotrigine should immediately

contact their doctors if they experience any of these symptoms.

Gabapentin

Gabapentin (Neurontin, generic) is an add-on drug for controlling

complex partial seizures and generalized partial seizures in both adults

and children.

Side Effects: Side effects include sleepiness, headache, fatigue, and

dizziness. Some weight gain may occur. Children may experience

hyperactivity or aggressive behavior. Long-term adverse effects are still

unknown.

Pregabalin

Pregabalin (Lyrica) is similar to gabapentin. It is approved as add-on

therapy to treat partial-onset seizures in adults with epilepsy.

Side Effects: Dizziness, sleepiness, dry mouth, swelling in hands and

feet, blurred vision, weight gain, and trouble concentrating may occur.

Topiramate

Topiramate (Topamax, generic) is similar to phenytoin and

carbamazepine and is used to treat a wide variety of seizures in adults

and children. It is approved as add-on therapy for patients 2 years and

older with generalized tonic-clonic seizures, partial-onset seizures, or

seizures associated with Lennox-Gastaut syndrome. It is also approved

as single drug therapy.

Side Effects: Most side effects are mild to moderate and can be reduced

or prevented by beginning at low doses and increasing dosage

gradually. Common side effects may include numbness and tingling,

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fatigue, abnormalities of taste, difficulty concentrating, and weight loss.

Serious side effects may include glaucoma and other eye problems.

A medical provider should be notified right away for blurred vision or

eye pain. If used during pregnancy, topiramate can increase the risk for

cleft lip or palate birth defects.

Oxcarbazepine

Oxcarbazepine (Trileptal, generic) is similar to phenytoin and

carbamazepine but generally has fewer side effects. It is approved as

single or add-on therapy for partial seizures in adults and for children

ages 4 years and older.

Side Effects: Serious side effects, while rare, include Stevens-Johnson

syndrome and toxic epidermal necrolysis. These skin reactions cause a

severe rash that can be life threatening. Rash and fever may also be a

sign of multi-organ hypersensitivity, another serious side effect

associated with this drug. Oxcarbazepine can reduce sodium levels

(hyponatremia). Serum sodium levels should be monitored. This drug

can reduce the effectiveness of birth control pills. Women who take

oxcarbazepine may need to use a different type of contraceptive.

Zonisamide

Zonisamide (Zonegran, generic) is approved as add-on therapy for

adults with partial seizures.

Side Effects: Zonisamide increases the risk for kidney stones. It may

reduce sweating and cause a sudden rise in body temperature,

especially in hot weather. Other side effects tend to decrease over time

and may include dizziness, forgetfulness, headache, weight loss, and

nausea.

Levetiracetam

Levetiracetam (Keppra, generic) is approved both in oral and

intravenous forms as add-on therapy for treating many types of

seizures in both children and adults.

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Side Effects: These tend to occur mostly in the first month. They

include sleepiness, dizziness, and fatigue.

More serious side effects may include muscle weakness and

coordination difficulties, behavioral changes, and increased risk of

infections.

Tiagabine

Tiagabine (Gabitril) has properties similar to phenytoin and

carbamazepine.

Side Effects: Tiagabine may cause significant side effects including

dizziness, fatigue, agitation, and tremor. The FDA has warned that

tiagabine may cause seizures in patients without epilepsy. Tiagabine is

only approved for use with other anti-epilepsy medicines to treat partial

seizures in adults and children 12 years and older.

Ezogabine

Ezogabine (Potiga), a potassium channel opener, was approved in 2011

for treatment of partial seizures in adults. Ezogabine is used as an add-

on (adjunctive) medication.

Its most serious side effect is urinary retention. Patients should be

monitored for symptoms such as difficulty initiating urination, weak

urine stream, or painful urination. Other side effects may include

coordination problems, memory problems, fatigue, dizziness, and

double vision.

Perampanel

Perampanel (Fycompa) was approved in 2012 as add-on treatment for

partial onset seizures in patients age 12 years and older. It is the first

in a new class of AEDs for uncontrolled partial epilepsy. Perampanel

targets the AMPA glutamate receptor, which is involved in seizure

activity. Perampanel is taken as a once-daily tablet.

Common side effects may include dizziness, drowsiness, and fatigue.

Peramanel also has a boxed warning to alert about potential risks of

serious mood changes and mental disturbances including irritability,

aggression, anxiety, and violent thoughts or behaviors.

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Less Commonly

Used AEDs

Felbamate (Felbatol) is an effective antiseizure drug. However, due to

reports of deaths from liver failure and from a serious blood condition

known as aplastic anemia, felbamate is recommended only under

certain circumstances. They include severe epilepsy, such as Lennox-

Gastaut syndrome, or as monotherapy for partial seizures in adults

when other drugs fail.

Vigabatrin (Sabril) has serious side effects, such as vision disturbances,

and is generally prescribed only in specific cases. It is sometimes given

in low doses for patients with Lennox-Gastaut syndrome. Vigabatrin is

also prescribed as a low-dose oral solution to treat infantile spasms in

children ages 1 month to 2 years.

Emotional Impact And Support

Individuals with epilepsy are more prone to behavioral and emotional

problems than their peers. In fact, mental health and behavioral problems

occur at a rate of approximately thirty to fifty percent in those with epilepsy,

while only affecting 8.5 percent of individuals who do not have epilepsy (102).

Children with epilepsy are especially prone to behavioral and emotional

problems as a result of the condition (103). These problems typically fall into

two categories: internal and external factors.

Internal factors are a direct result of complications in the affected area of

the brain. They are typically caused by structural or functional problems and

are biologically based (104). External factors are not biologically based and

occur as a result of the social response to the individual’s epilepsy. External

factors include feelings of anxiety and depression (105). In most instances,

patients will experience a combination of internal and external factors (106).

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The following section provides an overview of the main factors involved in

the development of emotional and behavioral problems.

For the person with epilepsy, a range of factors can combine to produce a

heightened sense of anxiety, depression, low self-esteem, and feelings of

isolation. While most people with the condition learn how to deal with these

feelings, some may respond to such pressures by reacting in an overly

aggressive, asocial, irritable, or introverted manner.

It is often the possibility of having a seizure, rather than the seizure itself,

which may be handicapping to the person with epilepsy. Afraid of having a

seizure in public and the very real possibility of injury, the person with

epilepsy may seclude her- or himself. As a result a person may become very

isolated. As well, the person with seizures may be anxious about other

people's reactions to a seizure. Many people who witness a seizure may

react by being afraid and embarrassed by the situation. Since the individual

who has seizures has no control over other people's reactions during a

seizure, he or she may prefer to stay alone and in isolation.

One of the greatest concerns for the person who has recurring seizures is

the perceived loss of control, which goes along with having seizures.

Contemporary western culture has glorified the image of the controlled and

independent adult. The unpredictability of having a seizure, as well as the

very obvious loss of control during seizures clearly does not reflect this

image. By thus "failing" to meet the basic standards of our culture, a

person's sense of self-worth may well be affected. This sense of not being in

control may also extend to include other aspects of a person's life.

Being stigmatized for having epilepsy is also an important aspect. Popular

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misconceptions about epilepsy are still widespread. Again, other people's

negative responses may considerably add to the stress of the person with

epilepsy and may lead them to choose isolation over social interaction.

Sometimes, if the condition is well controlled, and a person has only a few

seizures, he or she may not feel compelled to deal with the condition. Then,

the denial of the condition can compound feelings of anxiety. In a sense, the

person does not get "used" to having seizures, and each seizure becomes

yet another traumatic experience. A person's own attitudes towards having

seizures can also very much influence their emotional state. By not

accepting the reality of having seizures, some persons may go through some

length to hide it from the people around them. The anxieties of possibly

being found out may reinforce the desire to socially isolate themselves.

Another important factor for the person with epilepsy that can greatly

increase stress and thereby emotional strain is economic hardship. High

rates of unemployment and underemployment - more than 50% for persons

with seizures - severely restricts the income of many people with epilepsy.

Thus they may have difficulty sustaining a household, not to mention the

added expenses of anticonvulsant medication.

Most persons who take anticonvulsant medication to control their seizures do

not experience serious and intolerable side effects from it. In some cases,

however, the side effects from taking medication may affect an individual's

behavior and/or emotional state. Such changes may include an impairment

of drive, mood, sociability, alertness, or concentration. People who

experience side effects in response to taking one single drug will generally

find that these effects will disappear over the first few months. However,

side effects may become a problem when the person is taking more than

one kind of anticonvulsant medication to control different types of seizures.

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It may be that the side effects of one medication are compounded by the

side effects of another. If these effects are not well tolerated, changes in

behavior and mood can occur. However, it has been found that, if the

amount of medication an individual receives is reduced, these changes are

reversed. While it is important to be aware of the possible effects of

medication, it should be recognized that they do not usually present a

serious problem to adults with epilepsy as long as they are administered in

the appropriate dosage.

The place in the brain where seizures originate may also have an effect on a

person's emotions and on her or his behavior. Seizures with temporal lobe

involvement, complex partial seizures (formerly known as psychomotor or

temporal lobe epilepsy) are most commonly associated with behavioral

changes. Such changes can include rapid fluctuations in mood, or over-

attention to detail (107–111).

The type of seizure will often impact the severity, and type, of emotional and

behavioral problems experienced by patients. The seizure type can impact

the basic functions of the brain, thereby causing internal factors that will

affect the emotional and behavioral health of the patient (12). In addition,

the severity and type of the seizure can lead to the development of external

factors such as depression and anxiety. When a patient feels impacted by

potential seizures, he or she is more apt to develop anxiety. In addition, the

limitations caused by epilepsy can cause patients to experience depression

and anger (102).

Patients will also experience stress, anxiety, and depression as a result of

treatment from others. Many patients will feel stigmatized and will allow

these feelings to affect how they perceive their situation. For some, the

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impact will cause high levels of stress and depression (112). This is especially

common in patients who are already prone to mental health issues (104).

Self-esteem issues are quite prevalent in patients who feel stigmatized, as

well as in those who are concerned with the attention they will receive when

a seizure occurs (113). Patients who are prone to frequent seizures in public

places will see an increase in self-esteem issues as a result (110).

Some patients will experience emotional and behavioral issues as a result of

the anti-epilepsy medication they take to control their seizures. Since

anticonvulsants primary function involves the inhibition of electrical activity

in the brain, they can also impact behavioral and cognitive function. This

can lead to the development of emotional and behavioral issues in some

patients, especially children (114). Some emotional and behavioral problems

are more common in those with epilepsy. The following table provides an

overview of the most common behavioral and emotional conditions in

individuals with epilepsy: (102,115–120)

Depression

Depression is the mood disorder most commonly associated with

epilepsy. However, it can often go unrecognized and untreated in

people with the disorder, especially in children. Epilepsy-related

depression can occur before, during, or after seizures, but is most

often associated with periods between seizures.

The symptoms of depression vary widely from one individual to

another. Those most often seen in children with epilepsy are sleep

disturbances, fatigue or listlessness, lack of enthusiasm, and frequent

emotional outbursts. Other behavioral issues, such as anxiety,

agitation, frustration, or impulsive behaviors, often accompany

depression.

Although the cause of depression in people with epilepsy is unknown,

it is thought to result from both internal and external factors.

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Attention

Deficit

Disorder

Attention deficit disorder with or without hyperactivity is considered a

common behavioral problem in children with epilepsy. It is estimated

that nearly 8 percent of children with epilepsy have problems with

attention. In general, attention deficit/hyperactivity disorder (ADHD) is

a neurobehavioral disorder that causes individuals to be easily

distracted, frustrated, fidgety, impulsive, and forgetful. The disorder

makes learning and social interactions difficult, regardless of an

individual's cognitive abilities. While ADHD is a clinical diagnosis made

on the basis of observation and medical history, mental health experts

and scientists agree that there are identifiable characteristics of the

disorder. Measures such as rating scales and reports from teachers

and parents can be helpful in making the diagnosis.

Anxiety

Disorders

Anxiety disorders associated with epilepsy may take the form of

chronic, generalized worrying; acute, overwhelming panic attacks; or

obsessive-compulsive tendencies. The disorders often arise in

response to the unpredictability and lack of control associated with

seizures. For some people with epilepsy, anxiety may cause them to

overestimate the threat posed by future seizures, or underestimate

their ability to cope. Such thoughts can cause physical symptoms that

accentuate the feeling of a lack of control.

Aggression

Impulse-control problems are common among children with epilepsy.

One of the most common forms of impulsivity is aggression. Although

the cause of aggression in people with epilepsy varies, the

unpredictability of seizures and the individual's lack of control over

them may contribute to frustration and irritability. In addition, children

who are more severely affected and lack good communication skills

may act out their frustration with aggressive or even violent

outbursts.

In general, aggressive behaviors tend to become less frequent and

decrease in severity as a person grows older. However, aggressive

tendencies may then be replaced by depression and anxiety.

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Autism

Autism is a spectrum disorder, or combination of symptoms,

characterized by deficits in verbal and nonverbal communication skills,

severe social dysfunction, and repetitive behaviors. Such behavioral

problems are sometimes seen in children with Lennox-Gastaut

syndrome, tuberous sclerosis complex, Angelman syndrome, and

other genetic disorders. Despite decades of research attempting to

link autism to a wide variety of potential causes, there still is no

consensus, and effective medical treatments have yet to be found.

However, there are behavioral and educational interventions that have

been developed specifically for individuals with autism.

Individuals with epilepsy will often require support in coping with the

emotional and behavioral problems associated with their condition. At the

most basic level, patients can benefit from having a support team that will

help manage the various aspects of the illness (121). Patients who have such

a network will be more involved in the care and will feel less stigmatized by

their peers. In addition, providing access to knowledge will enable patients

to make informed decisions and feel empowered and confident (122).

Beyond the basic level of support, patients will often require a combination

of medication and cognitive and behavioral interventions. This

comprehensive approach will combine pharmacological support with therapy

and peer support. Patients who receive this level of treatment often report

reduced feelings of stress, anxiety, and depression (102). In most instances,

the patient will be prescribed antianxiety medication and/or antidepressants.

If the patient is experiencing other forms of emotional or behavioral distress,

additional medications may be prescribed.

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Stigma

Many individuals experience problems as a result of the stigma attached to

epilepsy. Stigma causes social avoidance of all age groups and challenges in

an employment setting. There are various levels of stigma associated with

epilepsy: (124–127)

Internalized stigma is felt within the person with the condition and

reflects their feelings, thoughts, beliefs and fears about being

different.

Interpersonal stigma occurs in interactions with others both within and

external to the family system; and in these interactions the person

with the illness is treated differently and negatively because of the

health condition.

Institutionalized stigma reflects indirect expressions of different

treatment of persons with an illness as a group in the larger society,

e.g., discrimination of persons with epilepsy by policies of an insurance

company.

Most of the stigma associated with epilepsy is caused by a long history of

misinformation and misrepresentations of the impact of the illness. Fear of

sudden seizures and the physical impact they can have on an individual

often causes anxiety in those who have contact with them (128). Poor

portrayal of the illness in the media further enhances the stigma associated

with it (129). These misperceptions have existed for centuries. As a result,

people with epilepsy have experienced prejudice and discrimination. They

have felt stigmatized and ostracized because of their medical condition and,

as a result, have limited their social engagement and involvement in the

workforce (130). In addition, the stigma associated with epilepsy can often

lead to increased feelings of depression and anxiety in the patient (113).

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Due to the level of stigma associated with epilepsy, many individuals have

been hesitant to disclose their status due to feelings of shame and fear (131).

This can have detrimental effect on the individuals as they struggle to obtain

care and treatment without bringing attention to their medical disorder. In

fact, some patients may feel so stigmatized that they refuse to admit that

they are afflicted with epilepsy. These individuals often refrain from

receiving, medical treatment in favor of maintaining anonymity (132).

To combat the stigma associated with epilepsy, a number of education and

awareness programs have been developed. In addition, to education and

awareness programs, support networks can help patients cope with the

repercussions of the stigma (133). The Epilepsy Foundation has conducted

public campaigns since the 1970s, including efforts to reduce stigma, but

their long-term impact on attitudes is unknown (134). Advocacy campaigns for

other health conditions provide a variety of lessons and best practices for

the epilepsy community; some efforts have effectively used carefully

selected spokespeople and have achieved important policy changes. Actions

needed to improve public awareness and knowledge include informing

journalists as well as writers and producers in the entertainment industry;

engaging people with epilepsy and their families in public awareness efforts;

coordinating public awareness efforts and developing shared messaging; and

ensuring that all campaigns include rigorous formative research,

considerations for health literacy and audience demographics, and

mechanisms for evaluation and sustainability (135).

In recent years, the education and awareness campaigns aimed at reducing

the stigma associated with epilepsy have been somewhat successful. Recent

studies have shown that there has been a reduction in negative attitudes

toward the illness, especially in the social sphere. However, some negative

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attitudes still exist. They are most prevalent in the employment sector,

where individuals still experience discrimination based upon misinformation

and misrepresentations. Many individuals with epilepsy still experience

barriers to employment (136). The following Epilepsy Foundation fact sheet

provides an overview of the employment barriers individuals may experience

as a result of their epilepsy: (137)

According to the Epilepsy Foundation’s 2008 Needs Assessment Survey, 38% of adult

respondents were unemployed (as compared to the overall rate of 9.6% at the time).

Furthermore, the mean annual personal income of full-time, year-round workers with

epilepsy was $39,690, as compared to the U.S. average of $52,703 (American

Community Survey, 2007). Unemployment and underemployment among adults with

epilepsy have a significant impact on financial security and quality of life.

Perhaps the greatest barrier to employment for people with epilepsy is the inability to

reliably get to and from work because of driving restrictions and a lack of other

transportation options. Unless a person has been seizure-free for six months, he or she is

not allowed to drive and, therefore, must rely upon family members, co-workers, or

public transportation to get to work. Unfortunately, in most areas of the state, public

transportation is only an option if you work in the community in which you live, and you

live in a community that has good public transportation. In addition, it’s not always

feasible to get a ride from friends, family members, or co-workers.

The symptoms of epilepsy (e.g., seizures, medication side effects, memory problems,

depression, etc.) can also be major barriers to employment. Seizures can limit one’s

ability to safely perform certain job duties and disrupt one’s work schedule, especially if

the individual has a prolonged recovery period after seizures. Drowsiness, poor

coordination, and cognitive problems can make it difficult to perform at the level

expected by employers and can also pose safety risks. If you develop epilepsy as a

working adult, it can be difficult to adjust to new restrictions and limitations. In some

cases, you may need to consider switching the field in which you work, and this may

require additional education or training.

Despite these challenges, though, most people with epilepsy can work effectively and are

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not at significantly higher risk of injury on the job. In most cases, simple

accommodations can help people with epilepsy get around these barriers to employment;

however, this is dependent on having an employer that understands epilepsy and

employment rights.

Unfortunately, many employers have fears about epilepsy that are largely unfounded.

These fears can ultimately result in discrimination in the form of dismissal from

employment or failure to get hired in the first place. Therefore, it is important for an

individual to know when to disclose epilepsy to an employer, how to anticipate and

address employer concerns, and what your rights are.

SUMMARY

Epilepsy is a complex brain disorder that is characterized by seizures, which

are caused by disturbances in the brain’s electrical functions. The term

epilepsy encompasses a variety of different syndromes, each ranging in its

symptoms, severity, and duration. The characteristic seizures are present in

all types of epilepsy, but they differ in presentation and severity depending

on the type of epilepsy.

Epilepsy is most common in young children and the elderly, but it can affect

individuals of all ages. In 50 % of the cases, the cause of epilepsy is

unknown. In those instances when a cause is identified, we find that the

cause varies between environmental or genetic factors, or as part of

traumatic injury. Some epileptic syndromes will only last a short time,

especially those caused by trauma; however, some other epileptic

syndromes will be lifelong conditions that cannot be cured.

While many individuals will experience a single unprovoked seizure at some

point in their lives, epilepsy is not considered until the patient has had two

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or more unprovoked seizures. Once this occurs, the patient will begin the

process for assessing and diagnosing the type of epilepsy.

Epilepsy can be a frustrating and scary condition, but recent advances in

medication and surgical options have made it easier to control. Even though

the cause of this disorder is still not understood, great strides have been

made in the effort to improve care of the epileptic patient. Understanding

current trends in epilepsy care will assist medical providers and nurses to

provide best practice care to patients and ensure that they have the best

quality of life possible.

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