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Epilepsy

What is Epilepsy?

Seizures are caused by a sudden burst of electric activity in the brain. There are different types of seizures ranging from subtle absences up to life threatening tonic clonic movement (shaking) in which sudden stiffening and shaking of body is associated with unresponsiveness. In epilepsy, the normal pattern of neuronal activity becomes disturbed, causing strange sensations, emotions, and behaviour or sometimes convulsions, muscle spasms, and loss of consciousness. The epilepsies have many possible causes and there are several types of seizures. Anything that disturbs the normal pattern of neuron activity-from illness to brain damage to abnormal brain development-can lead to seizures.

Epilepsy may develop because of an abnormality in brain wiring, an imbalance of nerve signaling chemicals called neurotransmitters, changes in important features of brain cells called channels, or some combination of these and other factors.

Is there any treatment?

Once epilepsy is diagnosed, it is important to begin treatment as soon as possible. For about 70-80% of those diagnosed with epilepsy, seizures can be controlled with modern medicines, special diet and surgical techniques depending upon the diagnosis. Some drugs are more effective for specific types of seizures. An individual with seizures, particularly those that are not easily controlled, may want to see a neurologist specifically trained to treat epilepsy. In some children, special diets (Ketogenic diet) may help to control seizures when medications are either not effective or cause serious side effects.

What is the prognosis?

While epilepsy cannot be cured, for some people the seizures can be controlled with medication, diet, devices, and/or surgery. Most seizures do not cause brain damage, but on-going uncontrolled seizures may cause brain damage. Issues may also arise as a result of the stigma attached to having epilepsy, which can led to embarrassment and frustration or bullying, teasing, or avoidance in school and other social settings.

What should I do if my child starts fits at home?

  1. During the seizure, do not panic; take a note of time if possible.
  2. Please do not put anything, water, spoon, finger, food, etc.) into the mouth to prevent the clenching of teeth.
  3. Try to keep the child in a safe and flat surface and in right or left lateral position
  4. Do not restrain the child.
  5. Loosen any tight clothes.
  6. Take help from another person; keep yourself ready to take the child to the hospital if seizure does not stop within five minutes. Your doctor may have given some nasal spray which you can use at this stage in the doses advised and make your way to the hospital, if this is first seizure at home.
  7. If the seizure settles, keep the child in left lateral position, recovery position.
  8. Inform your local pediatric neurologist

Causes of Epilepsy

The causes of epilepsy can be put into three different categories, and each category includes a number of different types of epilepsy, including:

  • Symptomatic Epilepsy is when there is a known cause for a person's epilepsy, such as a head injury, infection of the brain like meningitis, or a stroke.
  • Idiopathic Epilepsy is when there is no clear cause for the seizures suddenly starting.
  • Cryptogenic Epilepsy is when a known cause for a person's epilepsy starting cannot be proved, but one is suspected.

Epilepsy cannot be cured, but seizures are controllable with medication in about 70% of cases.In those whose seizures do not respond to medication, surgery, neurostimulation or dietary changes may be considered. Not all cases of epilepsy are lifelong, and a substantial number of people improve to the point that medication is no longer needed.

About 1% of people worldwide (65 million) have epilepsy,and nearly 80% of cases occur in developing countries.Epilepsy becomes more common as people age.In the developed world, onset of new cases occurs most frequently in infants and the elderly;in the developing world this is in older children and young adults, due to differences in the frequency of the underlying causes. About 5-10% of all people will have an unprovoked seizure by the age of 80, and the chance of experiencing a second seizure is between 40 and 50%.In many areas of the world those with epilepsy either have their ability to drive restricted or disallowed,but most are able to return to driving after a period of time without seizures.

Signs and symptoms

Epilepsy is characterized by a long-term risk of recurrent seizures.These seizures may present in several ways depending on the part of the brain involved and the person's age.

Seizures

The most common type (60%) of seizures are convulsive. Of these, two-thirds begin as focal seizures (which may then become generalized) while one-third begin as generalized seizures. The remaining 40% of seizures are non-convulsive. An example of this type is the absence seizure, which presents as a decreased level of consciousness and usually lasts about 10 seconds.

Focal seizures are often preceded by certain experiences, known as an aura. These may include sensory (visual, hearing or smell), psychic, autonomic, or motor phenomena. Jerking activity may start in a specific muscle group and spread to surrounding muscle groups in which case it is known as a Jacksonian march. Automatisms may occur; these are non-consciously generated activities and mostly simple repetitive movements like smacking of the lips or more complex activities such as attempts to pick something up.

There are six main types of generalized seizures: tonic-clonic, tonic, clonic, myoclonic, absence, and atonic seizures.They all involve loss of consciousness and typically happen without warning. Tonic-clonic seizures present with a contraction of the limbs followed by their extension along with arching of the back which lasts 10-30 seconds (the tonic phase). A cry may be heard due to contraction of the chest muscles. This is then followed by a shaking of the limbs in unison (clonic phase). Tonic seizures produce constant contractions of the muscles. A person often turns blue as breathing is stopped. In clonic seizures there is shaking of the limbs in unison. After the shaking has stopped it may take 10-30minutes for the person to return to normal; this period is called the "postictal phase".

Loss of bowel or bladder control may occur during a seizure. The tongue may be bitten at either the tip or on the sides during a seizure. In tonic-clonic seizure, bites to the sides are more common. Tongue bites are also relatively common in psychogenic non-epileptic seizures.

Myoclonic seizures involve spasms of muscles in either a few areas or all over. Absence seizures can be subtle with only a slight turn of the head or eye blinking. The person does not fall over and returns to normal right after it ends. Atonic seizures involve the loss of muscle activity for greater than one second. This typically occurs on both sides of the body.

About 6% of those with epilepsy have seizures that are often triggered by specific events and are known as reflex seizures. Those with reflex epilepsy have seizures that are only triggered by specific stimuli. Common triggers include flashing lights and sudden noises. In certain types of epilepsy, seizures happen more often during sleep, and in other types they occur almost only when sleeping.

Causes and symptoms

Epilepsy has many causes that, in part, have an affect on the clinical presentation of symptoms. In order for epilepsy to occur, there must be an underlying physical problem in the brain. The problem can be so mild that an individual is perfectly normal other than seizures. The brain has roughly 50–100 billion neurons. Each neuron can have up to 10,000 contacts with neighboring neurons. Hence, trillions of connections exist. However, only a very small area of dysfunctional brain tissue is necessary to create a persistent generator of seizures and, hence, epilepsy. The following are potential causes of epilepsy:

  • genetic and/or hereditary
  • perinatal neurological insults
  • trauma with brain injury
  • stroke
  • brain tumors
  • infections such as meningitis and encephalitis
  • multiple sclerosis
  • ideopathic (unknown or genetic)

Any of the above conditions have the potential for causing the brain or a portion of it to be dysfunctional and produce recurrent seizures. Regardless of the exact cause, epilepsy is a paroxysmal (sudden) condition. It involves the synchronous discharging of a population of neurons. This is an abnormal event that, depending on the location in the brain, will correspond to the particular symptoms of a seizure. The International League Against Epilepsy (ILAE) issued a classification of types of seizures. The list gives the kind of seizures that can occur. Individual seizure types are based on the clinical behavior (semiology) and electrophysiological characteristics as seen on an electroencephalogram (EEG). Generalized seizures included in the list are:

  • tonic-clonic seizures (includes variations beginning with a clonic or myoclonic phase)
  • clonic seizures, including without tonic features and with tonic features
  • typical absence seizures
  • atypical absence seizures
  • myoclonic absence seizures
  • tonic seizures
  • spasms
  • myoclonic seizures
  • eyelid myoclonia, including without absences and with absences
  • myoclonic atonic seizures
  • negative myoclonus atonic seizures
  • reflex seizures in generalized epilepsy syndromes

Focal seizures included in the ILAE list are:

  • focal sensory seizures with elementary sensory symptoms (e.g., occipital and parietal lobe seizures) and experiential sensory symptoms (e.g., temporo-parieto-occipital junction seizures)
  • focal motor seizures with elementary clonic motor signs, asymmetrical tonic motor seizures (e.g., supplementary motor seizures), typical (temporal lobe) automatisms (e.g., mesial temporal lobe seizures), hyperkinetic automatisms, focal negative myoclonus, and inhibitory motor seizures
  • gelastic seizures
  • hemiclonic seizures
  • secondarily generalized seizures
  • reflex seizures in focal epilepsy syndromes

In 1989, the International League Against Epilepsy also issued the following classification of epilepsies and epileptic syndromes:

  • benign familial neonatal seizures
  • early myoclonic encephalopathy
  • Ohtahara syndrome
  • migrating partial seizures of infancy (syndrome in development)
  • West syndrome
  • benign myoclonic epilepsy in infancy
  • benign familial and non-familial infantile seizures
  • Dravet's syndrome
  • HH syndrome
  • myoclonic status in nonprogressive encephalopathies (syndrome in development)
  • benign childhood epilepsy with centrotemporal spikes
  • early onset benign childhood occipital epilepsy (Panayiotopoulos type)
  • late-onset childhood occipital epilepsy (Gastaut type)
  • epilepsy with myoclonic absences
  • epilepsy with myoclonic-astatic seizures
  • Lennox-Gastaut syndrome
  • Landau-Kleffner syndrome (LKS)
  • epilepsy with continuous spike-and-waves during slow-wave sleep (other than LKS)
  • childhood absence epilepsy
  • progressive myoclonus epilepsies
  • idiopathic generalized epilepsies with variable phenotypes include juvenile absence epilepsy, juvenile myoclonic epilepsy, and epilepsy with generalized tonic-clonic seizures only
  • reflex epilepsies
  • idiopathic photosensitive occipital lobe epilepsy
  • other visual sensitive epilepsies
  • primary reading epilepsy
  • startle epilepsy
  • autosomal dominant nocturnal frontal lobe epilepsy
  • familial temporal lobe epilepsies
  • generalized epilepsies with febrile seizures plus (syndrome in development)
  • familial focal epilepsy with variable foci (syndrome in development)
  • symptomatic focal epilepsies
  • limbic epilepsies
  • mesial temporal lobe epilepsy with hippocampal sclerosis
  • mesial temporal lobe epilepsy defined by specific etiologies
  • neocortical epilepsies
  • Rasmussen syndrome

Classifying epilepsy can help in the evaluation and management of patients with seizure disorders. The combination of seizure type(s), etiology (cause), age of onset,

family history, and other medical or neurological conditions can be used to identify an epilepsy syndrome. Classification helps clinicians and researchers understand the broader picture of seizure disorders. On a practical level, syndrome identification can help in planning the management of patients. Syndrome classification schemes are revised periodically as individual components of particular categories are better understood.

The term idiopathic refers to a cause that is suspected to be, if not genetic, then unknown. Cryptogenic is a term that suggests that an underlying cause is suspected, but not yet fully understood. Symptomatic is a term that is applied to epilepsies that are a result of understood underlying pathologies.

The management and prognosis vary considerably among these differing syndromes. Epilepsies that have a genetic basis can be inherited or occur spontaneously. A detailed family history can often identify other family members who have had seizures. However, because seizures are common, it is possible to have more than one family member with epilepsy, though the etiologies may not be related. To say that a particular type of epilepsy is genetic does not mean that it is necessarily transmitted by heredity. Often, disorders can have a genetic cause, but be spontaneously occurring in only one member of a family. In this case, there may simply be a random mutation in that particular person's genes.

There are several mechanisms in which epilepsies can be inherited. So-called simple Mendelian inheritance occurs with benign familial neonatal convulsions and autosomal dominant nocturnal frontal lobe epilepsy. On the other hand, complex inheritance mechanisms can involve more than one gene, or a gene mutation in combination with environmental or acquired factors such as juvenile myoclonic epilepsy. As the genetics of the epilepsies become better understood, the classification scheme will evolve.

With epilepsy, symptoms vary considerably depending on the type. The common link among the epilepsies is, of course, seizures. The different epilepsies can sometimes be associated with more than one seizure type. This is the case with Lennox-Gastaut syndrome.

Diagnosis

Arriving at a diagnosis of epilepsy is relatively straightforward: when people suffer two or more seizures, they would be considered to have epilepsy. However, diagnosing the specific epilepsy syndrome is much more complex. The first step in the evaluation process is to obtain a very detailed history of the illness, not only from the patient but from the family as well. Since seizures can impair consciousness, the patient may not be able to recall the specifics of the attacks. In these cases, family or friends that have witnessed the episodes can fill in the gaps about the particulars of the seizure. The description of the behaviors during a seizure can go a long way to categorizing the type of seizure and help with the overall diagnosis. Moreover, in the initial visit with the physician, the entire history of the patient is obtained. In a child, this would include birth history, complications, if any, maternal history, and developmental milestones. At any age, socalled co-morbidities (other medical problems) are considered. Medications that have been taken or currently being prescribed are documented.

A complete physical examination is performed, especially a neurological exam. Because seizures are an episodic disorder, abnormal neurological findings may not be present. Frequently, people with epilepsy have a normal exam. However, in some, there can be abnormal findings that can provide clues to the underlying cause of epilepsy. For example, if someone has had a stroke that subsequently caused seizures, then the neurological exam can be expected to reveal a focal neurological deficit such as weakness or language difficulties. In some children with seizures, there can be a variety of associated neurologic abnormalities such as mental retardation and cerebral palsy that are themselves non-specific but indicate that the brain has suffered, at some point in development, an injury or malformation. Also, subtle findings on examination can lead to a diagnosis such as in tuberous sclerosis . This is an autosomal dominantly inherited disorder associated with infantile spasms in 25% of cases. On examination, patients have so-called ash-leaf spots and adenoma sebaceum on the skin. There can also be a variety of systemic abnormalities that involve the kidneys, retina, heart, and gums, depending on severity.

In the course of evaluating epilepsy, a number of tests are typically ordered. Usually, magnetic resonance image (MRI ) of the brain is obtained. This is a scan that can help in finding many known causes of epilepsy such as tumors, strokes, trauma, and congenital malformations. However, while MRI can reveal incredible details of the brain, it cannot visualize the presence of abnormalities in the microscopic neuronal environment. Another test that is routinely ordered is an electroencephalogram (EEG). Unlike the MRI scan, this can be considered a functional test of the brain. The EEG measures the electrical activity of the brain. Some seizure disorders or epilepsies have a characteristic EEG with particular abnormalities that can help in diagnosis. Other tests that are frequently ordered are various blood tests that are also ordered in many medical conditions. These blood tests help to screen for abnormalities that can be a factor in the cause of seizures. Occasionally, genetic testing is performed in those instances where a known genetic cause is suspected and can be tested. A major concern in the course of an evaluation of epilepsy is to identify the presence of life-threatening causes such as brain tumors, infections, and cerebrovascular disease. Also, an accurate diagnosis can expedite the most effective treatment plan.

The symptoms of epilepsy are dependent in part on the particular seizures that occur and other medical problems that may be associated. Seizures, themselves, can take on a variety of features. A simple sustained twitching of an extremity could be a focal seizure. If a seizure arises in the occipital lobes of the brains, then a visual experience can occur. Aura is a term often used to describe symptoms that a person may feel prior to the loss of consciousness of a seizure. However, auras are, themselves, small focal seizures that have not spread in the brain to involve consciousness. Smells, well-formed hallucinations, tingling sensations, or nausea have each occurred in auras. The particular sensation can be a clue as to the location in the brain where a seizure starts. Focal seizures can then spread to involve other areas of the brain and lead to an alteration of consciousness, and possibly convulsions. In certain epilepsy syndromes such as Lennox-Gastaut, there can be more than one type of seizure experienced, such as atonic, atypical absence, and tonic-axial seizures.

Treatment

One challenge in predicting the course of epilepsy is that for any type, there can be a variable response to treatment. Sometimes, seizures may play a rather small role in the manifestation of a medical condition. For example, a severe head injury could result in seizures that readily respond to medication, but severe neurological impairments and disabilities may still be present. On the other hand, a different head injury may result in relatively mild neurological problems, but there may be seizures that are severe and be resistant to medications.

Whatever the case, the ultimate goals when treating epilepsy are to:

  • strive for complete freedom from seizures
  • have little to no side effects from medications
  • be able to follow an easy regimen so that compliance with treatment can be maintained

Up to 60% of patients with epilepsy can be expected to achieve control of seizures with medication(s). However, in the remaining 40%, epilepsy appears to be resistant, to varying degrees, to medications. In these cases, the epilepsy is termed medically intractable.

Generally, the choice of medication is somewhat trial and error. There are, however, a number of considerations that guide the choice of treatment. Each medication has a particular side effect profile and mechanism of action. Some medications seem to be particularly effective for certain epilepsy syndromes. For example, juvenile myoclonic epilepsy responds well to valproic acid. On the other hand, ethosuxamide is primarily used for absence seizures.

As with any medication, individuals can have very different experiences with same drug. Consequently, it is difficult to predict the efficacy of treatment in the beginning. A key concept of treatment is to first strive for monotherapy (or single drug therapy). This simplifies treatment and minimizes the chance of side effects. Sometimes, however, two or more drugs may be necessary to achieve satisfactory control of seizures. As with any treatment, potential side effects can be worse than the disease itself. Moreover, there is little point in controlling seizures if severe side effects limit quality of life. If a seizure disorder is characterized by mild, focal, or brief symptoms that do not interfere with day-to-day life, then aggressive treatments may not be justified. Epilepsy medications do not cure epilepsy; the medications can only control the frequency and severity of seizures. A list of the most commonly used medications in the management of epilepsy includes:

  • phenobarbital
  • phenytoin (Dilantin, Phenytek)
  • clonazepam (Klonipin)
  • ethosuxamide (Zarontin)
  • carbamazepine (Tegretol, Carbatrol)
  • divalproex sodium (Depakote, Depakene)
  • felbamate (Felbatol)
  • gabapentin (Neurontin)
  • lamotrigine (Lamictal)
  • topiramate (Topamax)
  • tiagabine (Gabatril)
  • zonisamide (Zonegran)
  • oxcarbazepine (Trileptal)
  • leviteracetam (Keppra)

It has been found that the initial, thoughtfully chosen medication can be expected to make almost 50% of patients seizure free for extended periods of time. If the initial drug fails, another well-chosen drug may make an additional 14% of people seizure free. If that drug fails, then the likelihood of rendering someone with epilepsy seizure free is poor. This does not mean that trying more medications or combinations of them may not be successful, but rather, these statistics give the neurologist and the patient an understanding of the realities of epilepsy treatment. In cases where medications do not fully control epilepsy, it is recommended that a more extensive evaluation at a comprehensive epilepsy center be conducted where an epileptologist (a specialist in epilepsy) will more thoroughly assess the particular aspects of the seizures. When medications are clearly ineffective, the other types of therapy that can be considered are the ketogenic diet, brain surgery, and vagal nerve stimulation.

Ketogenic diet

The ketogenic diet is based on high-fat, low-carbo-hydrate, and low-protein meals. The ketogenic diet is named because of the production of ketones by the breakdown of fatty acids. The most common version of the diet involves long-chain triglycerides. These are present in whole cream, butter, and fatty meats.

The ketogenic diet is administered with the support of a nutritionist with experience in this treatment modality. It is mostly used in children with medically intractable epilepsy and whose diet can be controlled. The ketogenic diet can be considered a pharmacologic treatment. As such, there are potential side effects that limit its tolerance. This includes hair thinning, lethargy, weight loss, kidney stones, and possibly cardiac problems. Sugar-free vitamin and mineral supplementation is necessary. The diet may not be appropriate for certain individuals, particularly in children, who may have certain metabolic diseases.

Overall, the diet has been very helpful in the control of seizures in many patients. Roughly 50% of patients can hope to achieve complete control of seizures, 25% of the patients see improvements, and another 25% are non-responders. There are some patients who have an improvement in behavior. If the diet is well tolerated with good results, then it can be maintained for up to two years, followed by a careful gradual transition to regular meals.

Epilepsy surgery

Epilepsy surgery is an option in the attempt to either cure or significantly reduce the severity of medically resistant cases. It is thought that up to 100,000 patients in the United States could be potential candidates for a surgical treatment. However, only about 5,000 cases are performed throughout the United States annually. This is likely due to several factors, including the belief that any brain surgery is a last resort, the lack of awareness or understanding of the benefits of surgery, and the false hope that some medication will come along that will be effective

There are several kinds of surgery that are available depending on the nature of the seizure disorder. A list of operations that are utilized regularly for epilepsy include:

  • lobectomy
  • lesionectomy
  • corpus collosotomy
  • multiple subpial transection
  • hemispherectomy

The type of surgery that is performed depends on the nature of the individual seizure disorder. If a seizure can be localized to a particular area in the brain, then this abnormal region can potentially be surgically removed. Epileptic brain tissue is abnormal and its removal can provide a chance of a cure. Generally, surgery should be a consideration when the risk and benefits of it outweigh the long-term risks of uncontrolled epilepsy.

The approach taken in any brain surgery for epilepsy is highly individualized and great care is taken to avoid injury to essential brain tissue. The most common epilepsy surgery performed is the temporal lobectomy. Brain tumors are frequently associated with seizures. In many cases, surgery to remove the tumor is planned so that regions that may be causing seizures are removed as well. However, in many cases, epilepsy surgery cannot be done.