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Epilepsy (sometimes referred to as a seizure disorder) is a common chronic neurological condition that is characterized by recurrent unprovoked epileptic seizures.

It affects approximately 50 million people worldwide.[1] It is usually controlled, but not cured, with medication although surgery may be considered in difficult cases.

Classification
Epilepsies are classified five ways: 1. 2. 3. 4. 5. By their first cause (or etiology). By the observable manifestations of the seizures, known as "semiology." By the location in the brain where the seizures originate. As a part of discrete, identifiable medical syndromes. By the event that triggers the seizures, as in primary reading epilepsy.

In 1981, the International League Against Epilepsy (ILAE) proposed a classification scheme for individual seizures that remains in common use.[2] This classification is based on observation (clinical and EEG) rather than the underlying pathophysiology or anatomy and is outlined later on in this article. In 1989, the ILAE proposed a classification scheme for epilepsies and epileptic syndromes.[3] This can be broadly described as a two-axis scheme having the cause on one axis and the extent of localisation within the brain on the other. Since 1997, the ILAE have been working on a new scheme that has five axes: ictal phenomenon, seizure type, syndrome, etiology and impairment.[4]

Diagnosis
The diagnosis of epilepsy requires the presence of recurrent, unprovoked seizures; accordingly, it is usually made based on the medical history. EEG, brain MRI, SPECT, PET, and magnetoencephalography may be useful to discover an etiology for the epilepsy, discover the affected brain region, or classify the epileptic syndrome, but these studies are not useful in making the initial diagnosis. Long-term video-EEG monitoring for epilepsy is the gold standard for diagnosis, but it is not routinely employed owing to its high cost and inconvenience. Convulsive or other seizure-like activity, non-epileptic in origin, can be observed in many other medical conditions. These non-epileptic seizures can be hard to differentiate and may lead to misdiagnosis. Epilepsy covers conditions with different etiologies, natural histories and prognoses, each requiring different management strategies. A full medical diagnosis requires a definite categorisation of seizure and syndrome types.[5]

Causes

The cause of an individual's epilepsy can be divided into two categories: symptomatic and idiopathic.[3] Symptomatic epilepsies originate due to some structural or metabolic abnormality in the brain. This may be the result of:

genetic conditions such as tuberous sclerosis complications during pregnancy or birth stroke head injury neurosurgical operations bacterial or viral encephalitis parasitical infection

The term cryptogenic is used to describe epilepsy where the cause is suspected to be symptomatic but the underlying illness or damage has not been identified. The term idiopathic means "a disorder unto itself", and not "cause unknown". No other condition has been implicated as the cause of the epilepsy. Idiopathic epilepsies are often but not exclusively genetic and generalised - for example Juvenile Absence Epilepsy.

Triggers
Many definitions of epilepsy require that the seizures be "unprovoked", with the implication that the provocant is assumed to be something obviously harmful. However, some epilepsy syndromes the provocant can reasonably be considered to be part of normal daily life. Examples of these normal provocants include reading, hot water on the head, hyperventilation and flashing or flickering lights. This last provocant is a special type of reflex epilepsy called photosensitive epilepsy. Certain circumstances can lead to an increased likelihood of seizures in someone with epilepsy or in certain syndromes. For example:

during sleep the transition between sleep and awake tireness illness constipation menstruation stress

Epidemiology
The most common ages of incidence are under the age of 18 and over the age of 65. It has been estimated that about 1% of the population meets the diagnostic criteria for epilepsy at any given time, but some theorize that the prevalence may be much higher in fact.

Seizure types
Main article: Seizure types Seizure types are organised firstly according to whether the source of the seizure within the brain is localised (partial or focal onset seizures) or distributed (generalised seizures). Partial seizures are further divided on the extent to which consciousness is affected. If it is unaffected, then it is a simple partial seizure; otherwise it is a complex partial seizure. A partial seizure may spread within the brain - a process known as secondary generalisation. Generalised seizures are divided according to the effect on the body but all involve loss of consciousness. These include absence (peitit mal), myoclonic, clonic, tonic, tonic-clonic (grand mal) and atonic seizures.

Seizure syndromes
There are many different epilepsy syndromes, each presenting with its own unique combination of seizure type, typical age of onset, EEG findings, treatment, and prognosis. Below are some common seizure syndromes:

Infantile spasms (West syndrome) is associated with brain development abnormalities, tuberous sclerosis, and perinatal insults to the brain. It affects infants (as implied by its name), which by definition is between 30 days to 1 year of life. It carries a poor prognosis such that only 5-10% of children with infantile spasms will develop normal to near-normal function, while more than two-thirds will have severe deficits. The typical seizures are characterized by sudden flexor and extensor spasms of head, trunk, and extremities. The key EEG finding in these patients is a hypsarrhythmia, or a high-voltage slow wave with multifocal spikes. The first line treatment for these patients is adrenocorticotropic hormone (ACTH or corticotropin) since traditional antiepileptic drugs generally cannot adequately control seizure activity. Vigabatrin is also used in many countries, and is particularly effective when tuberous sclerosis is the cause of seizures.

Generalized 3 Hz spike and wave discharges in EEG

Childhood absence epilepsy affects children between the ages of 4 and 12 years of age. These patients have recurrent absence seizures that can occur hundreds of times a day. On EEG, one finds the stereotyped generalized 3 Hz spike and wave discharges. A subset of these patients will also develop generalized tonic-clonic seizures. This condition carries a fairly good prognosis in that these children do not usually show cognitive decline or neurological deficits. First line treatment for pure absence seizures is ethosuximide. If patients do not respond or have mixed seizures along with their absence seizures, then valproic acid can be used. Benign focal epilepsy of childhood (Benign Rolandic epilepsy) begins in children between the ages of 4 and 13 years. Apart from their seizure disorder, these patients are otherwise normal. Seizures occur at night and sleep promotes secondary generalization. As such, parents only report generalized seizures because focal manifestations are often subtle and go unnoticed. Between seizures, patients have a stereotyped EEG pattern that includes di- or triphasic sharp waves over the central-midtemporal (Rolandic) regions. Prognosis is uniformly good with seizures disappearing by adolescence. Carbamazepine is the first line treatment, though phenytoin and phenobarbital have also been used with some efficacy. Juvenile myoclonic epilepsy (JME) begins in patients aged 8 to 20 years. These patients have normal IQ and are otherwise neurologically intact. There is usually a family history of similar seizures. The seizures are morning myoclonic jerks often with generalized tonic-clonic seizures that occur just after waking. EEG readings reveal generalized spikes with 4-6 Hz spike wave discharges and multiple spike discharges. Interestingly, these patients are often first diagnosed when they have their first generalized tonic-clonic seizure later in life when they experience sleep deprivation (e.g., freshman year in college after staying up late to study for exams). Valproic acid is the first line treatment. This condition is lifelong, thus patients must be taught appropriate sleep hygiene to prevent generalized tonic-clonic seizures. Temporal lobe epilepsy is the most common epilepsy of adults. In most cases, the epileptogenic region is found in the mesial temporal structures (e.g., the hippocampus, amygdala, and parahippocampal gyrus). Seizures begin in late childhood and adolescence. There is an association with febrile seizures in childhood, and some studies have shown herpes simplex virus (HSV) DNA in these regions, suggesting that perhaps this epilepsy has an infectious etiology. Most of these patients have complex partial seizures often preceded by an aura. Frontal lobe epilepsy Lennox-Gastaut syndrome

Treatment
Epilepsy is usually treated with medication prescribed by a physician; primary caregivers, neurologists, and neurosurgeons all frequently care for people with epilepsy. In some cases the implantation of a stimulator of the vagus nerve, or a special diet can be helpful. Neurosurgical operations for epilepsy can be palliative, reducing the frequency or severity of seizures; or, in some patients, an operation can be curative.

Responding to a seizure
In most cases, the proper emergency response to a generalized tonic-clonic epileptic seizure is simply to prevent the patient from self-injury by moving him or her away from sharp edges, placing something soft beneath the head, and carefully rolling the person onto his or her side to avoid asphyxiation. Should the person regurgitate, the material should be allowed to drip out the side of the patient's mouth by itself. If the seizure lasts longer than 5 minutes, Emergency Medical Services should be contacted. Prolonged seizures may develop into status epilepticus, a dangerous condition requiring hospitalization and emergency treatment. Objects should never be placed in a person's mouth during a seizure as this could result in injury to the person's mouth or obstruction of the airway. Despite common folklore, it is not possible for a person to swallow their own tongue during a seizure. After a seizure, it is typical for a person to be confused, disoriented, and possibly agitated or sleepy. It is important to stay with the person until this passes; people should not eat or drink until they have returned to their normal level of awareness, and they should not be allowed to wander about unsupervised. Many patients will sleep deeply for a few hours after a seizure; this is not dangerous. In about 50% of people with epilepsy, headaches may occur after a seizure. These headaches share many features with migraines, and respond to the same medications. If it seems to have been a first seizure, it is likely to be noticeably helpful to make a written or otherwise recorded note of the sequence and nature of events. The doctor deciding on further management will probably find this helpful.

Pharmacologic treatment
Some medications can be taken daily in order to prevent seizures altogether or reduce the frequency of their occurrence. These are termed "anticonvulsant" or "antiepileptic" drugs (sometimes AEDs). All such drugs have side effects which are idiosyncratic and others which are dose-dependent; it is not possible to predict who will suffer from side effects or at what dose the side effects will appear. Some people with epilepsy will experience a complete remission when treated with an anticonvulsant medication. If this does not occur, the dose of medication may be increased, or another medication may be added to the first. The general strategy is to increase the medication dose until either the seizures are controlled, or until dose-limiting side effects appear; at which point the medication dose is reduced to the highest amount that did not produce undesirable side effects. Serum levels of AEDs can be checked to determine medication compliance and to assess the effects of drug-drug interactions; serum levels are generally not useful to predict anticonvulsant efficacy in an individual patient, though in some cases (such as a seizure flurry) it can be useful to know if the level is very high or very low.

If a person's epilepsy cannot be brought under control after adequate trials of two different drugs, that person's epilepsy is generally said to be 'medically refractory.' Various drugs may prevent seizures or reduce seizure frequency: these include carbamazepine (common brand name Tegretol), clobazam (Frisium), clonazepam (Klonopin), ethosuximide (Zarontin), felbamate (Felbatol), fosphenytoin (Cerebyx), flurazepam (Dalmane), gabapentin (Neurontin), lamotrigine (Lamictal), levetiracetam (Keppra), oxcarbazepine (Trileptal), mephenytoin (Mesantoin), phenobarbital (Luminal), phenytoin (Dilantin), pregabalin (Lyrica), primidone (Mysoline), sodium valproate (Epilim), tiagabine (Gabitril), topiramate (Topamax), valproate semisodium (Depakote), valproic acid (Depakene, Convulex), and vigabatrin (Sabril). Other drugs are commonly used to abort an active seizure or interrupt a seizure flurry; these include diazepam (Valium) and lorazepam (Ativan). Drugs used only in the treatment of refractory status epilepticus include paraldehyde (Paral) and pentobarbital (Nembutal). Bromides were the first of the effective anticonvulsant pure compounds, but are no longer used in humans[6] due to their toxicities and low efficacy.

Surgical treatment
Surgical treatment can be an option for epilepsy when an underlying brain abnormality, such as a benign tumor or an area of scar tissue (e.g. hippocampal sclerosis) can be identified. The abnormality must be removable by a neurosurgeon. Surgery is usually only offered to patients when their epilepsy has not been controlled by adequate attempts with multiple medications. Before surgery is offered, the medical team conducts many tests to assess whether removal of brain tissue will result in unacceptable problems with memory, vision, language or movement, which are controlled by different parts of the brain. These tests usually include a neuropsychological evaluation, which sometimes includes an intracarotid sodium amobarbital test (Wada test). Resective surgery, as opposed to palliative, successfully eliminates or significantly reduces seizures in about 50-90% of the patients who undergo it (the exact percentage depends on the particulars of the case in question.) Many patients decide not to undergo surgery owing to fear or the uncertainty of having a brain operation. The most common form of resective surgical treatment for epilepsy is to remove the front part of either the right or left temporal lobe. A study of 48 patients who underwent this operation, anterior temporal lobectomy, between 1965 and 1974 determined the longterm success of the procedure. Of the 48 patients, 21 had had no seizures that caused loss of consciousness since the operation. Three others had been free of seizures for at least 19 years. The rest had either never been completely free of seizures or had died between the time of the surgery and commencement of the study.[7]

Palliative surgery for epilepsy is intended to reduce the frequency or severity of seizures. Examples are callosotomy or commissurotomy to prevent seizures from generalizing (spreading to involve the entire brain), which results in a loss of consciousness. This procedure can therefore prevent injury due to the person falling to the ground after losing consciousness. It is performed only when the seizures cannot be controlled by other means. Resective surgery can be considered palliative if it is undertaken with the expectation that it will reduce but not eliminate seizures. Hemispherectomy is a drastic operation in which most or all of one half of the cerebral cortex is removed. It is reserved for people suffering from the most catastrophic epilepsies, such as those due to Rasmussen syndrome. If the surgery is performed on very young patients (2-5 years old), the remaining hemisphere may acquire some rudimentary motor control of the ipsilateral body; in older patients, paralysis results on the side of the body opposite to the part of the brain that was removed. Because of these and other side effects it is usually reserved for patients who have exhausted other treatment options.

Other treatment
Ketogenic diets may occasionally be effective in controlling some types of epilepsy; although the mechanism behind the effect is not fully understood, shifting of pH towards a metabolic acidosis and alteration of brain metabolism may be involved. Ketogenic diets are high in fat and extremely low in carbohydrates, with intake of fluids often limited. This treatment, originated as early as the 1920s at Johns Hopkins Medical Center, was largely abandoned with the discovery of modern anti-epileptic drugs, but recently has returned to the anti-epileptic treatment arsenal. Ketogenic diets are sometimes prescribed in severe cases where drugs have proven ineffective. There are several downsides to what initially seems a benign therapy, however. The ketogenic diet is not good for the heart or kidneys and medical problems resulting from the diet have been reported. In addition, the diet is extremely unpalatable and few patients are able to tolerate it for any length of time. Since a single potato chip is adequate to break the ketosis, staying on the diet requires either great willpower or perfect control of a person's dietary intake. People fed via gastrostomy or young children who receive all their food in the presence of a caregiver are better candidates. Vagus nerve stimulation is a recently developed form of seizure control which uses an implanted electrical device, similar in size, shape and implant location to a heart pacemaker, which connects to the vagus nerve in the neck. Once in place the device can be set to emit electronic pulses, stimulating the vagus nerve at pre-set intervals and milliamp levels. Treatment studies have shown that approximately 50% of those treated in this fashion will show significant seizure reduction. Some people with epilepsy receive a special dog which has the rare talent of sensing the onset of a seizure and is trained to alert the human so they can reach a safe location before their seizure puts them in danger. Other epilepsy care dogs do not sense seizures,

but serve as companions and guardians during the loss of consciousness accompanying a seizure. The Institutes for The Achievement of Human Potential promulgate a home program consisting of a healthy diet, clean air, and respiratory training. This alternative approach is regarded as dangerous and without value by most medical practitioners. Magnesium and vitamin B6 exerted a positive non-specific influence on the mental states of patients with epilepsy, depression and anxiety during an experiment.[8] A number of systematic reviews by the Cochrane Collaboration into treatments for epilepsy looked at acupuncture[9], psychological interventions[10], vitamins[11] and yoga[12] and found there is no reliable evidence to support the use of these as treatments for epilepsy.

Pathophysiology
Mutations in several genes have been linked to some types of epilepsy. Several genes that code for protein subunits of voltage-gated and ligand-gated ion channels have been associated with forms of generalized epilepsy and infantile seizure syndromes.[13] Several ligand-gated ion channels have been linked to some types of frontal and generalized epilepsies. Epilepsy-related mutations in some non-ion channel genes have also been identified. One interesting finding in animals is that repeated low-level electrical stimulation to some brain sites can lead to permanent increases in seizure susceptibility: in other words, a permanent decrease in seizure "threshold." This phenomenon, known as kindling (by analogy with the use of burning twigs to start a larger fire) was discovered by Dr. Graham Goddard in 1967. Chemical stimulation can also induce seizures; repeated exposures to some pesticides have been shown to induce seizures in both humans and animals. One mechanism proposed for this is called excitotoxicity. The roles of kindling and excitotoxicity, if any, in human epilepsy are currently hotly debated.

History and stigma


The word epilepsy is derived from the Greek epilepsia, which in turn can be broken in to epi- (upon) and lepsis (to take hold of, or seizure)[14] In the past, epilepsy was associated with religious experiences and even demonic possession. Apocryphally, epilepsy has been called the "Sacred Disease" because people thought that epileptic seizures were a form of attack by demons, or that the visions experienced by persons with epilepsy were sent by the gods. However, in many cultures, persons with epilepsy have been stigmatized, shunned, or even imprisoned; in the Salptrire, the birthplace of modern neurology, Jean-Martin Charcot found people with epilepsy side-by-side with the mentally retarded, those with chronic syphilis, and the criminally insane. In Tanzania to this day, onlookers will not touch a person having an epileptic fit, owing to fear of

demons, even if the seizure causes the person to fall into the cooking fire (the flickering light from fire may have provoked the seizure in the first place.)[citation needed] In ancient Rome, epilepsy was known as the Morbus Comitialis ('disease of the assembly hall') and was seen as a curse from the gods. Stigma continues to this day, in both the public and private spheres, but polls suggest it is generally decreasing with time, at least in the developed world; Hippocrates remarked that epilepsy would be considered divine only until it was understood.[15]

Legal implications
Seizures have caused many fatal car accidents and plane crashes. Most people diagnosed with epilepsy are forbidden by their local laws from operating vehicles. However, there are usually exceptions for those who can prove that they have stabilized their condition. Those few whose seizures do not cause impairment of consciousness, or whose seizures only arise from sleep, may be exempt from such restrictions, depending on local laws. There is an ongoing debate in bioethics over who should bear the burden of ensuring that an epilepsy patient does not drive a car or fly an airplane. In the U.S., people with epilepsy can drive if their seizures are controlled with treatment and they meet the licensing requirements in their state. How long (they) have to be free of seizures varies in different states, but it's most likely to be between three months and a year[16] [17] . The majority of the 50 states place the burden on patients to report their condition to appropriate licensing authorities so that their privileges can be revoked where appropriate. A minority of states (including California) place the burden of reporting on the patient's physician. After reporting is carried out, it is usually the driver's licensing agency that decides to revoke or restrict a driver's license. In the UK, it is the responsibility of the patient to inform the Driver and Vehicle Licensing Agency (DVLA) if they have epilepsy[18] . The DVLA rules are quite complex[19] , but in summary[20] , those continuing to have seizures or who are within 6 months of medication change may have their license revoked. A doctor who becomes aware that a patient with uncontrolled epilepsy is continuing to drive has, after reminding the patient of their responsibility, a duty to break confidentiality and inform the DVLA. The doctor should advise the patient of the disclosure and the reasons why their failure to notify the agency obliged the doctor to act.

Important investigators of epilepsy


Galen Jean-Martin Charcot John Hughlings Jackson Hans Berger Herbert Jasper Wilder Penfield

H. Houston Merritt William G. Lennox

See also

Seizure Non-epileptic seizures List of people with epilepsy Epilepsy in animals Seizure response dog Jacksonian seizure Photosensitive epilepsy Temporal lobe epilepsy

Notes and references


1. ^ (2005-09-21) Atlas: Epilepsy care in the world 2005 (PDF), World Health Organization. 9241563036, 3. 2. ^ (1981). "Proposal for revised clinical and electroencephalographic classification of epileptic seizures. From the Commission on Classification and Terminology of the International League Against Epilepsy.". Epilepsia 22 (4): 489-501. PMID 6790275. 3. ^ a b (1989). "Proposal for revised classification of epilepsies and epileptic syndromes. Commission on Classification and Terminology of the International League Against Epilepsy.". Epilepsia 30 (4): 389-99. PMID 2502382. 4. ^ Jerome Engel. A Proposed Diagnostic Scheme For People With Epileptic Seizures And With Epilepsy: Report Of The Ilae Task Force On Classification And Terminology. ILAE. Retrieved on 2006-07-18. 5. ^ C P Panayiotopoulos and M Koutroumanidis (September 2005). The significance of the syndromic diagnosis of the epilepsies. The National Society for Epilepsy. 6. ^ Clemmons DVM, PhD, R.M. (1997). Seizure Disorders in Dogs and Cats. The Neurology Service at the VMTH. University of Floridas Veterinary Medical Teaching Hospital. Retrieved on 2006-03-29. 7. ^ Kelley K, Theodore WH (2005). "Prognosis 30 years after temporal lobectomy". Neurology 64 (11): 1974-6. PMID 15955959. 8. ^ Kalinin V, Zheleznova E, Rogacheva T, Sokolova L, Polianski D, Zemlianaia A, Nazmetdinova D (2004). "[A use of Magne-B6 in the treatment of anxietydepressive states in patients with epilepsy]". Zh Nevrol Psikhiatr Im S S Korsakova 104 (8): 51-5. PMID 15554143. 9. ^ Cheuk D, Wong V (2006). "Acupuncture for epilepsy". Cochrane Database Syst Rev (2): CD005062. PMID 16625622. 10. ^ Ramaratnam S, Baker GA, Goldstein LH (2005). "Psychological treatments for epilepsy". Cochrane Database Syst Rev (4): CD002029. PMID 16235293.

11. ^ Ranganathan LN, Ramaratnam S (2005). "Vitamins for epilepsy". Cochrane Database Syst Rev (2): CD004304. PMID 15846704. 12. ^ Ramaratnam S, Sridharan K (2000). "Yoga for epilepsy". Cochrane Database Syst Rev (3): CD001524. PMID 10908505. 13. ^ Miriam H. Meisler and Jennifer A. Kearney (2005). "Sodium channel mutations in epilepsy and other neurological disorders". Journal of Clinical Investigation 115 (8): 20102017. PMID 16075041 DOI:10.1172/JCI25466. 14. ^ Harper, Douglas (2001). epilepsy. Online Etymological Dictionary. Retrieved on 2005-06-05. 15. ^ Hippocrates quotes 16. ^ Epilepsy Foundation Driving and You - Can you drive an automobile if you have epilepsy? 17. ^ Epilepsy Foundation Driver Information by State 18. ^ UK Epilepsy Action: Driving and Epilepsy, I've had a seizure. What should I do? 19. ^ UK Driver and Vehicle Licensing Agency Guide to the Current Medical Standards Of Fitness to Drive. Full details for doctors regarding epilepsy are given in the Appendix. Information for drivers can be found in Medical Rules Group 1 Licence Holders 20. ^ UK Epilepsy Action: booklet with further details about driving PDF Resources

(National Women's Health Information Center, OWH, HHS) A Pacemaker for the Brain (Cleveland Clinic)

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