Diagnosis Coding

Description

Seizures and epilepsy is a broad topic. Pediatricians care for children with a wide range of these problems including typically developing children with infrequent seizures as well as severely impaired children with complicated medical regimens. This module attempts to present information that a primary care provider can use to help care for children with uncomplicated clinical situations involving seizures and epileptic syndromes. Less common seizure types and epilepsy syndromes are not covered here; more information can be found in Seizure Syndrome Type. The emergency evaluation and management of seizures is not covered here.

Seizure: A sudden, involuntary, time-limited alteration in behavior, motor activity, autonomic function, consciousness, or sensation, accompanied by an abnormal electrical discharge in the brain. Seizures can be provoked by acute medical conditions (e.g., trauma, electrolyte disturbances, meningitis) or they can occur without provocation.

Epilepsy: A condition in which an individual has recurrent, unprovoked seizures.

Status epilepticus: Traditionally, status epilepticus has been defined as more than thirty minutes of continuous seizure activity or recurrent seizures without intercurrent recovery of consciousness. Most seizures will stop on their own within five minutes of onset. A child with a seizure lasting 5 minutes or more will likely require medical intervention to stop it. [Jenssen: 2006] (See Status Epilepticus).

Prevalence

Pediatric epilepsy incidence in Singapore was 24 per 100000 person-years, and was highest in early childhood. Focal epilepsy was found to be more common than generalized epilepsy. [Chan: 2010] Looking at different parameters, a study of pediatric epilepsy in Brazil found an incidence rate of 7/100000 children with a prevalence of 65.2/10000 children. [Nunes: 2011] Demographic information - seizures (Epilepsy Foundation of America) presents information from the Epilepsy Foundation of America, but it is unclear from this information how those numbers were derived.

Genetics

Although seizures may run in families, inheritance patterns are hard to predict. Epilepsy is probably a polygenic disorder, and may require a combination of genetic and environmental susceptibilities to be expressed in an individual. Sometimes the clinical presentation suggests a particular epilepsy syndrome; clinical testing is available to diagnose some of these syndromes. See Genetic testing for specific epilepsy syndromes (Gene Reviews) and [Deprez: 2009] for more information.

Prognosis

The two primary elements of prognosis include: seizure recurrence risk and likelihood for seizures to be controlled by medication. In general, prognosis will depend on the underlying etiology and the type of seizure. Prognosis for a child with seizures may vary from excellent to devastating.

• Seizure recurrence risk after a first afebrile, generalized, tonic-clonic seizure in a typically developing child is 25-50% (children with intellectual disability, cerebral palsy, and/or a family history of epilepsy are more likely to have recurrent seizures):
  • Age of the child and duration of the event do not affect the risk of recurrence.
  • Half of recurrences will occur in the first 6 months following a first seizure, two thirds will occur within one year, and 90% or more within 2 years.
  • The EEG is an important predictor of recurrence. If the EEG is normal, the 5-year recurrence risk is 25%.
  • The first afebrile seizure in a typically developing child is not usually treated with anticonvulsants.
• Likelihood for seizures to be controlled by medication:
  • 50% of children with epilepsy will respond to the first medication.
  • 20-30 % of children will not respond completely, will require two medications for control, or will change medication before control is reached.
  • 20-30% of patients will have intractable epilepsy that doesn't respond completely to multiple medications and/or other treatments such as the ketogenic diet, surgery, and the vagal nerve stimulator. Demographic information - seizures (Epilepsy Foundation of America)
• Up to 75% of children will experience a prolonged remission from seizures and will be able to stop medication after two or more years.

Roles Of The Medical Home

Children with febrile seizures are usually managed by their primary care clinician. Children with uncomplicated genetic epilepsy syndromes such as childhood absence epilepsy or benign Rolandic epilepsy, may also be managed by their primary care clinician, often following an initial visit to a neurologist to confirm the diagnosis. Many of the other epilepsies, including syndromes such as Lennox-Gaustaut, will require concurrent care with a pediatric neurologist. A primary care clinician might wish to refer a patient to neurology when discontinuation of AEDs is being considered.

Practice Guidelines

Hirtz D, Ashwal S, Berg A, Bettis D, Camfield C, Camfield P, Crumrine P, Elterman R, Schneider S, Shinnar S.
Practice parameter: evaluating a first nonfebrile seizure in children: report of the quality standards subcommittee of the American Academy of Neurology, The Child Neurology Society, and The American Epilepsy Society.
Neurology. 2000;55(5):616-23. PubMed abstract / Full Text

Subcommittee on febrile seizures.
Neurodiagnostic evaluation of the child with a simple febrile seizure.
Pediatrics. 2011;127(2):389-94. PubMed abstract

Chadwick D, Marson T.
Choosing a First Drug Treatment for Epilepsy after SANAD: Randomized Controlled Trials, Systematic Reviews, Guidelines and Treating Patients.
Epilepsia. 2007. PubMed abstract

Marson AG, Al-Kharusi AM, Alwaidh M, Appleton R, Baker GA, Chadwick DW, Cramp C, Cockerell OC, Cooper PN, Doughty J, Eaton B, Gamble C, Goulding PJ, Howell SJ, Hughes A, Jackson M, Jacoby A, Kellett M, Lawson GR, Leach JP, Nicolaides P, Roberts R, Shackley P, Shen J, Smith DF, Smith PE, Smith CT, Vanoli A, Williamson PR.
The SANAD study of effectiveness of valproate, lamotrigine, or topiramate for generalised and unclassifiable epilepsy: an unblinded randomised controlled trial.
Lancet. 2007;369(9566):1016-26. PubMed abstract / Full Text

Marson AG, Al-Kharusi AM, Alwaidh M, Appleton R, Baker GA, Chadwick DW, Cramp C, Cockerell OC, Cooper PN, Doughty J, Eaton B, Gamble C, Goulding PJ, Howell SJ, Hughes A, Jackson M, Jacoby A, Kellett M, Lawson GR, Leach JP, Nicolaides P, Roberts R, Shackley P, Shen J, Smith DF, Smith PE, Smith CT, Vanoli A, Williamson PR.
The SANAD study of effectiveness of carbamazepine, gabapentin, lamotrigine, oxcarbazepine, or topiramate for treatment of partial epilepsy: an unblinded randomised controlled trial.
Lancet. 2007;369(9566):1000-15. PubMed abstract / Full Text

Baumann RJ.
Technical report: treatment of the child with simple febrile seizures.
Pediatrics. 1999;103(6):e86. PubMed abstract / Full Text

Hirtz D, Berg A, Bettis D, Camfield C, Camfield P, Crumrine P, Gaillard WD, Schneider S, Shinnar S.
Practice parameter: treatment of the child with a first unprovoked seizure: Report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society.
Neurology. 2003;60(2):166-75. PubMed abstract / Full Text

Mackay MT, Weiss SK, Adams-Webber T, Ashwal S, Stephens D, Ballaban-Gill K, Baram TZ, Duchowny M, Hirtz D, Pellock JM, Shields WD, Shinnar S, Wyllie E, Snead OC 3rd.
Practice parameter: medical treatment of infantile spasms: report of the American Academy of Neurology and the Child Neurology Society.
Neurology. 2004;62(10):1668-81. PubMed abstract / Full Text

Steering Committee on Quality Improvement and Management, Subcommittee on Febrile Seizures American Academy of Pediatrics.
Febrile seizures: clinical practice guideline for the long-term management of the child with simple febrile seizures.
Pediatrics. 2008;121(6):1281-6. PubMed abstract

Helpful Articles

PubMed search for practice guidelines for seizures or epilepsy in children

Hauser WA, Beghi E.
First seizure definitions and worldwide incidence and mortality.
Epilepsia. 2008;49 Suppl 1:8-12. PubMed abstract

Neville BG, Chin RF, Scott RC.
Childhood convulsive status epilepticus: epidemiology, management and outcome.
Acta Neurol Scand Suppl. 2007;186:21-4. PubMed abstract

Subcommittee on febrile seizures.
Neurodiagnostic evaluation of the child with a simple febrile seizure.
Pediatrics. 2011;127(2):389-94. PubMed abstract

Overview

When a child presents having experienced a paroxysmal event, the clinician must determine if the event is a seizure. Many paroxysmal events are not seizures, and should not be treated with antiepileptic medication. See Differential diagnosis of paroxysmal events. Generally jerking/stiffening after a syncopal episode or breath-holding spell is not a seizure and will not respond to anti-epileptic medication. If the child has had a seizure, classify it by type – provoked or unprovoked, focal (localized) or generalized, etc. – to determine the appropriate diagnostic workup and treatment. The Seizure history & physical form ( 88 KB) may help guide your seizure history and physical exam.

No screening is recommended (or possible) for seizures/epilepsy. After being diagnosed with epilepsy, children should receive developmental screening and screening for mood disorders.

Clinical Classification

If there is reasonable certainty that the child has had a seizure, the next questions are why and what kind? A complete description of the event, including age of the patient and description of the seizure (focal vs. generalized) and related events (e.g. sleep deprivation before, a period of profound sleepiness afterward, associated fever, etc.), is needed. The Seizure assessment tool ( 41 KB) adapted from [Hirtz: 2000]] may be helpful. Seizure type will guide the search for etiology and treatment choices if antiepileptic drug (AED) therapy is considered. A seizure is classified as a focal seizure either by the description of the seizures, for example, "first his face was twitching and then he started jerking all over his body" or by EEG (abnormalities in EEG activity localized to one part of the brain, at least at the start of the seizure). Examples of normal (EEG - normal), focal (EEG - focal abnormalities (arrows)) and generalized seizure activity (EEG - generalized seizure activity (absence)) are shown in these links and may be helpful.

• Focal onset seizures (also called localized or partial seizures): symptoms at onset are convincingly localized in the motor, somatosensory, special sensory, autonomic, or limbic systems. Examples of focal onset seizures include: a seizure that starts with limb jerking on one side of the body, with tingling on one side of the body, with a sense of fear, or with vomiting. Because focal seizures may be associated with focal brain pathology (e.g., stroke or tumor), imaging is almost always indicated. The exception to imaging is if there is a diagnosis of a benign epilepsy syndrome such as benign Rolandic epilepsy. Seizures with focal onset before generalization are classified as focal seizures with secondary generalization and for purposes of evaluation, should be treated as focal seizures. The focal onset may be subtle and must be asked about – for instance, did the child's eyes go to one side before the tonic-clonic activity was noted?
• Generalized seizures begin with widespread manifestations, caused by widespread electrical dysfunction of the entire cortex. Types of generalized seizures include absence seizures and generalized tonic-clonic seizures.

Epilepsy Syndromes If a thorough history and physical exam does not suggest any provoking cause for a seizure, the child might have an epilepsy syndrome. Epilepsy syndromes can be classified using the age of onset and seizure type as follows:

• Neonatal seizures with onset between birth and 2 months of age - epilepsy syndromes include benign neonatal convulsions, benign myoclonic epilepsy of infancy, and others. Children with seizures occurring in this age group should be urgently evaluated for a provoking cause of the seizures, and if none is found, then seen by a pediatric neurologist.
• Seizures with onset from 2 months to 3 years of age - in this age group, epilepsy syndromes can range in severity of impact from benign to devastating.
  • Febrile seizures - generally benign, and very common. These are often familial, and may be part of the recently described syndrome Generalized Epilepsy with Febrile Seizures Plus (GEFS+) [Scheffer: 2005].
  • Benign myoclonic seizures - generally benign
  • Early infantile epileptic encephalopathy and early myoclonic epilepsy - generally severe and with a very poor prognosis
  • Infantile spasms (See also Infantile Spasms) - generally severe and with a very poor prognosis
  • Lennox Gastaut syndrome - generally severe and with a very poor prognosis

• Seizures with onset between 3 and 10 years of age - the majority of children in this group have genetic epilepsies that carry a good prognosis.
  • Childhood absence epilepsy (CAE; see also Absence Epilepsy)
  • Benign Rolandic epilepsy (BRE) or benign epilepsy with centro-temporal spikes (BECTS) - focal onset seizures of the face and arm, occur mostly at night, often familial, often not treated with AEDs (see Benign rolandic epilepsy (Benign childhood epilepsy with centrotemporal spikes - BECTS).
  • Epilepsy with Generalized Tonic Clonic (Grand Mal) Seizures Upon Awakening (EGMA) - a genetic variety of tonic-clonic epilepsy that has a predilection for occurring upon awakening. It may resolve by puberty.
• Seizures with onset over the age of 10 years - these epilepsy syndromes may also have a genetic origin but usually don't resolve spontaneously. Youths with these epileptic syndromes usually have normal lives but may need to be on medication indefinitely.
  • Juvenile absence epilepsy (JAE)
  • Juvenile myoclonic epilepsy (see Juvenile myoclonic epilepsy (JME)

Differential Diagnosis

Common events confused with seizures are syncope, breath-holding spells, and movement disorders such as tics and stereotypies. See Differential diagnosis of paroxysmal events. The clinical history of the event(s) is the most useful information in arriving at the diagnosis, but even a detailed history may not be sufficient to distinguish between a seizure or some other type of episode in a typically-developing child. Families can be given a (Seizure diary ( 75 KB)) to record descriptions of events and to help determine patterns over time. The family can also be asked to video an event, which is sometimes very helpful. For a one-time event in a child with a normal clinical evaluation, reassurance and reevaluation of the child if another similar event occurs may be the best approach. Because it is difficult to be certain that a seizure has not occurred, the child should be reevaluated if new events occur. If there is uncertainty, seizure precautions should be recommended (see Activity Restrictions in Children with Seizures) and documented. An EEG may not always help since mildly abnormal EEGs are common in the general population and many children with seizures have normal EEGs. If the child has neurologic abnormalities as a baseline, seizures are much more likely and the threshold for referral to pediatric neurology for further evaluation and management should be lower. If uncertainty continues after a period of observation, and if the events are occurring frequently enough to warrant testing, a 2 day video EEG as an inpatient or a prolonged ambulatory EEG where the patient continues to do his or her usual activities may be helpful. In episodes where this degree of uncertainty exists, a consultation with pediatric neurology may be useful.

Medical Conditions Causing Seizures/Epilepsy

Seizures when they first appear may be due to a primary seizure disorder (epilepsy), they may occur without an obvious underlying etiology, or they may be due to a number of underlying conditions. These include brain tumors, congenital brain malformations, and metabolic disorders. A diagnosis of a primary seizure disorder, or epilepsy, is made after other causes are ruled out.

Comorbid Conditions

Children with epilepsy have a higher incidence of mood disorders and learning and attention disorders, all of which may be due to the underlying epileptic syndrome, the seizures themselves, or the effects of the antiepileptic medications.

Pearls & Alerts

Flashing lights can trigger seizures

Flashing lights can trigger seizures in approximately 9% of people with epilepsy. This is more common in children and adolescents and becomes less common with age. The frequency of flashing that is most likely to cause seizures varies from person to person but is generally between 15 and 80 Hertz. [Hughes: 2008]

Non-epileptic seizures

Non-epileptic seizures (NES) are sometimes seen in older children and adolescents, and can be found in individuals who also have epileptic seizures. They should be considered when seizures are not responding to medications, particularly in older children and adolescents with a history of somatiform illnesses or with long, detailed responses to the review of systems. [Benbadis: 2007] Patients in whom non-epileptic seizures are suspected are likely best referred to pediatric neurology since the diagnosis may require video-EEG monitoring.

The term ‘pseudoseizure’ is generally avoided because of its negative connotation. NES is a diagnosable disorder, not a diagnosis of exclusion, with potentially severe consequences if untreated. Most children and adolescents will improve once the correct diagnosis is made and appropriate treatment is initiated.

Syncope is not seizure

If the child has had a syncopal event but has some jerking after they have passed out, this is not considered a seizure and the jerking does not have the same etiology as jerking with seizures. These children do not generally need to be seen by pediatric neurology.

Family History

Children may have a heritable epilepsy syndrome – there are now more than 70 gene mutations associated with epilepsy [Noebels: 2003] [Reid: 2009] - or an underlying condition that predisposes them to seizures, e.g., tuberous sclerosis.

Pregnancy Or Perinatal History

Children who experienced infections in utero, such as toxoplasmosis or CMV, are at greater risk for seizures. Brain injury associated with perinatal asphyxia or significant prematurity also increase the risk of seizures and epilepsy.

Current & Past Medical History

Head trauma is an uncommon cause of seizures, but an important diagnosis to consider in the acute setting – subdural or epidural hemorrhage can be life-threatening. Brain tumors are an even less common cause of seizures and may present also with behavior or personality change and vomiting (particularly early morning). What is the child's basic state of health? Illness, sleep-deprivation, alcohol and certain medications and illegal substances may lower the seizure threshold. Features of an event that are more associated with seizures than with non-seizure events:

• Precipitating events: head trauma, conditions that cause abrupt electrolyte changes (gastroenteritis, diabetes), febrile illness
• Pre-ictal symptoms: behavior or mood change or an aura minutes before a seizure may be a symptom to a focal onset
• Ictal description (appearance during the seizure): vocal – cry or gasp, slurring of words, garbled speech; motor – head or eye turning, eye deviation, posturing, jerking, stiffening, automatisms, generalized or focal movements; respiration – change in breathing pattern, cessation of breathing, cyanosis; autonomic – pupillary dilation, drooling, change in heart or respiratory rate, incontinence, pallor, vomiting; loss of consciousness or inability to understand or speak
• Postictal symptoms (appearance following the seizure): – amnesia for events, confusion, lethargy, sleepiness, headaches and muscle aches, transient focal weakness (Todd's paralysis), nausea or vomiting, bitten tongue

Important information to guide management will include the number of seizures since the last visit, their similarity to past seizures, any new medical problems, and daily functioning and school performance if applicable. Seizure Tracker may be helpful for families to track the number and type of their children's seizures. For children on an AED, ask about the AED(s) and doses the child is taking. Don't assume that the family has continued the AED(s) as a number of issues can lead to their changing the dosage or frequency of administration. Sleep problems are common in individuals with epilepsy. [French: 2004]

Developmental & Educational Progress

Developmental screening should be performed for young children and IQ and school performance assessment should be considered in older children. Developmental delays may be a manifestation of an underlying condition. A decrease in school performance may be due to undiagnosed seizures, especially absence seizures. Mood disorders and attention problems are common in children with epilepsy.

Developmental delays or a decrease in school performance may be due to increased seizures, including seizures not clinically apparent (such as absence seizures), side effects of AEDs, the presence of a neurodegenerative disorder, the presence of psycho-social stressors or the presence of a mental health disorder.

Maturational Progress

Oral contraceptives may interfere with antiepileptic medications or vice versa. In young women who may become pregnant, certain seizure medications should be avoided. Some AEDs are associated with teratogenic effects.

Social & Family Functioning

Seizures can interfere with social functioning in many ways. Frequent seizures, side effects of AEDs, and school absences may make it difficult for children with epilepsy to function well socially. Poor self-esteem is often found in children with epilepsy and may contribute to frequent mood disorders. Many children with epilepsy and their families perceive a social stigma associated with the condition.
Seizures and epilepsy can have substantial impact on families including fear of underlying diagnoses, fear of injury/death during seizures, concern about the future, financial problems, etc.

Physical Exam

In a child with a new onset seizure where acute causes such as meningitis has been ruled out, an EEG scheduled non-emergently is often helpful. The type of seizure and the EEG results will determine if imaging should be performed. If the child had a focal seizure, focal findings on EEG, or an abnormal neurologic exam, or is very young, MRI brain is usually performed.

Laboratory Testing

Depending on the presentation, laboratory testing may be helpful to identify a metabolic, electrolyte, or acid-base derangement that could cause seizures or be associated with a seizure-causing diagnosis. Examples include hypo- or hypernatremia, severe acidosis, hyperammonemia, and many others. Baseline labs for seizures in a neonate might include comprehensive metabolic profiles, CBC with differential, serum amino acids, urine organic acids, blood PH, lactate, pyruvate, ammonia, and biotinidase.
Some medications (e.g., valproic acid, carbamazepine) require the child to have baseline comprehensive metabolic profiles and CBCs before they are initiated and periodically during treatment as well as drug levels.

Imaging

EEG - After the child is clinically diagnosed with one or more seizures, EEG is performed to obtain additional information, usually on an outpatient basis and not emergently. EEG can show the specific area of onset in a focal onset seizure, and can confirm the diagnosis of an epilepsy syndrome, for example.
EEGs should not be used in the diagnosis of seizures because non-specific abnormalities in background activity can be seen in 10% of children without seizures, and 2-3% of healthy children may have epileptiform patterns on EEG (e.g.spikes or sharp waves) but never have a seizure. Conversely, normal EEGs do not rule out seizure.

The American Academy of Neurology recommends that all children with a first afebrile seizure undergo EEG [Hirtz: 2000]; this is usuallly part of the evaluation in children who are thought to have had a seizure.

A follow-up EEG may be helpful when seizures change in character or frequency or stop responding to a previously effective AED. Sometimes overnight video EEGs are necessary to determine whether a given frequent event is seizure or behavior, particularly in a child with known developmental delay or an abnormal neurological exam.

Imaging is often performed as part of the seizure workup unless there is a clear clinical diagnosis of a benign or genetic etiology (e.g. absence epilepsy or benign Rolandic epilepsy). [Berg: 2000] [Gaillard: 2009] Findings that are likely to increase the yield of an imaging study include: [Sharma: 2003]

• Seizure with focal onset
• Seizures in a newborn or young infant
• Status epilepticus at any age
• Focal abnormality on EEG

In children with new onset seizures that had localizing features, approximately 50% of imaging studies were reported to be abnormal. [Gaillard: 2009]

Brain MRI is preferred over a head CT when looking for cause of seizure or epilepsy. When a particular etiology for seizures is suspected, e.g., prenatal stroke, an MRI is useful to confirm the diagnosis and rule out other possibilities such as a developmental brain malformation, e.g., schizencephaly, or a new condition such as an abscess or tumor.

Genetic Testing

Referral to a geneticist may be indicated if there is a genetic condition such as tuberous sclerosis or a metabolic condition causing seizures. Some epilepsy syndromes are known to be genetic and specific testing is available. Currently, diagnostic testing is available for 16 major epilepsy syndromes. A number of others are diagnosed clinically. See Genetic epilepsy syndromes diagnostic testing. Genetic testing is generally directed by neurology and/or genetics with genetic counseling available for the family.

Other Testing

Sleep study - Children with epilepsy often have concurrent sleep disorders [Wilner: 2007] and a sleep study, with or without overnight EEG, may be helpful.

Overview

A fundamental principle in the treatment of seizures is initiation with an agent known to be effective in treating the given seizure type or epilepsy syndrome, followed by increasing the dose until the seizures are controlled or undesirable side effects occur. This implies that drug levels should not be used to define treatment failure – in the absence of adverse effects, a serum level that exceeds a 'therapeutic range' does not justify switching to another treatment. Drug levels are sometimes useful once seizures have been controlled to determine the level required for seizure control for a particular patient. Multiple practice guidelines are cited below.

Pearls & Alerts

Seizure precautions

Families of children with seizures or possible seizures need to learn about seizure precautions. Children need to avoid being alone near water (shower rather than bathe, unless an adult is present; an adult should watch only the child with seizures in swimming pools or hot tubs). Children should also be careful around hot water heaters, campfires, saunas, and cooking. In young children with seizures, babysitters should be aware of seizure precautions and what to do if there is a seizure. See the Let's Talk about...Seizures ( 128 KB) for more information.

Seizure action plans

Seizure action plans should be developed for all children with seizures so that families and providers know what to do in the case of a breakthrough seizure. See Seizure action plan (Pediatric Neurology division, Univ Utah Health Sciences Center) ( 67 KB).

Seizures and driving

Rules for driving after a seizure vary in different states. Only a few states have mandatory reporting by the treating physician; other states require the individual with seizures to report them. There are varying amounts of times that individuals must be seizure free, with or without AEDs, before driving is allowed. See Epilepsy and driving: physician issues (Epilepsy Foundation) for specifics.

Rescue drugs for breakthrough seizures

Rescue drugs for breakthrough seizures should be part of a seizure action plan if the child has prolonged seizures, or clusters of, seizures. These include nasal midazolam and rectal valium (Diastat). See the Evaluation and treatment of a first unprovoked seizure with end of visit information issue for more specific information.

Carbamazepine special considerations

Use of carbamazepine entails special considerations. Carbamazepine is sometimes used when clinicians are unsure of the seizure type because it is inexpensive, relatively safe, and well-known. However, it can worsen seizures that are generalized in nature. During the first few weeks, carbamazepine "autoinduces" liver enzymes, increasing its metabolism. Carbamazepine levels that were initially effective gradually fall, resulting in seizures. Children who are on carbamazepine should not drink grapefruit juice.

Drug interactions

Drug interactions should be considered when a child/adolescent is on an AED. For instance children on carbamazepine should avoid macrolide antibiotics and grapefruit juice because they slow metabolism of the medication. Oral contraceptives may interfere with AED efficacy and vice versa. See [Perucca: 2006] for more information.

Pregnancy

AED choice should take into account possible pregnancy. Certain AEDs are more strongly associated with major malformations of the fetus than others. Some examples include facial abnormalities (phenytoin, phenobarbital) and neural tube defects (valproic acid). No AEDs have proven to be completely safe in pregnancy, but seizures are also a potential risk to the mother and fetus. A primary care clinician should consider referring a patient with seizures who is also pregnant or is considering becoming pregnant to a neurologist.

Systems

Clinical Assessment

Seizure Syndrome Type

Neurology

Absence Epilepsy
Activity Restrictions in Children with Seizures
Benign rolandic epilepsy (Benign childhood epilepsy with centrotemporal spikes - BECTS)
Differential diagnosis of paroxysmal events
Epilepsy surgery
Evaluation and treatment of a first unprovoked seizure with end of visit information
Febrile seizures
Infantile spasms (West syndrome)
Juvenile myoclonic epilepsy (JME)
Ketogenic Diet
Lennox Gastaut syndrome
Status Epilepticus
Tapering Antiepileptic Medication
Types of Generalized Seizures
Vagus Nerve Stimulator (VNS)

Information for Clinicians

Epilepsy guidelines (NICE)
Guidance documents for the diagnosis and managment of epilepsy in children from the National Institute for Health and Clinical Excellence (National Health Service, UK).

Helpful Articles

PubMed search for practice guidelines for seizures or epilepsy in children

Hauser WA, Beghi E.
First seizure definitions and worldwide incidence and mortality.
Epilepsia. 2008;49 Suppl 1:8-12. PubMed abstract

Neville BG, Chin RF, Scott RC.
Childhood convulsive status epilepticus: epidemiology, management and outcome.
Acta Neurol Scand Suppl. 2007;186:21-4. PubMed abstract

Subcommittee on febrile seizures.
Neurodiagnostic evaluation of the child with a simple febrile seizure.
Pediatrics. 2011;127(2):389-94. PubMed abstract

Toolkits

Quality improvement in epilepsy-a toolkit for families (NICHQ)
This toolkit provides different resources and tips that can assist families with children with epilepsy to be part of quality improvement projects.

Other

Seizure Tracker
The easy-to-use tools found at SeizureTracker.com allow patients to create personalized reports of logged seizure activity and treatment history that can be easily shared with their medical team.

Information on the Web

Epilepsy Foundation
A national organization that provides information about epilepsy; programs to improve epilepsy treatment; materials to assist in helping people with epilepsy find jobs; activities in schools to educate the public; activities to educate policymakers; funds for research; and news about conferences and other items of interest.

Epilepsy (MedlinePlus)
Offers numerous links to patient/consumer information about epilepsy; from the National Library of Medicine.

Seizures, convulsions, and epilepsy (healthychildren.org)
General information about convulsions, seizures, and epilepsy from Healthychildren.org.

Epilepsy (ninds.nih.gov)
Detailed information about epilepsy and treatment from the NIH.

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Services for Patients & Families

For other services related to this condition, browse our Services categories or search our database.

Authors

Baumann RJ.
Technical report: treatment of the child with simple febrile seizures.
Pediatrics. 1999;103(6):e86. PubMed abstract / Full Text
This technical report provides in-depth information on the studies used to form the guideline recommendations. A complete bibliography is included as well as evidence tables that summarize data extracted from scientific studies. This report also provides pertinent evidence on the individual therapeutic agents studied including study results and dosing information. Readers of the clinical practice guideline are urged to review the technical report to enhance the evidence-based decision-making process.

Benbadis, SR.
Differential diagnosis of epilepsy.
Continuum Lifelong Learning Neurol . 2007;13(4):48-70.

Berg AT, Testa FM, Levy SR, Shinnar S.
Neuroimaging in children with newly diagnosed epilepsy: A community-based study.
Pediatrics. 2000;106(3):527-32. PubMed abstract

Chadwick D, Marson T.
Choosing a First Drug Treatment for Epilepsy after SANAD: Randomized Controlled Trials, Systematic Reviews, Guidelines and Treating Patients.
Epilepsia. 2007. PubMed abstract

Chan D, Phuah HK, Ng YL, Choong CT, Lim KW, Goh WH.
Pediatric epilepsy and first afebrile seizure in singapore: epidemiology and investigation yield at presentation.
J Child Neurol. 2010;25(10):1216-22. PubMed abstract

Deprez L, Jansen A, De Jonghe P.
Genetics of epilepsy syndromes starting in the first year of life.
Neurology. 2009;72(3):273-81. PubMed abstract

French JA, Kanner AM, Bautista J, Abou-Khalil B, Browne T, Harden CL, Theodore WH, Bazil C, Stern J, Schachter SC, Bergen D, Hirtz D, Montouris GD, Nespeca M, Gidal B, Marks WJ Jr, Turk WR, Fischer JH, Bourgeois B, Wilner A, Faught RE Jr, Sachdeo RC, Beydoun A, Glauser TA.
Efficacy and tolerability of the new antiepileptic drugs I: treatment of new onset epilepsy: report of the Therapeutics and Technology Assessment Subcommittee and Quality Standards Subcommittee of the American Academy of Neurology and the American Epilepsy Society.
Neurology. 2004;62(8):1252-60. PubMed abstract

French, J.
Treatment with antiepileptic drugs, new and old.
Continuum Lifelong Learning Neurol . 2007;13(4):71-90.

Gaillard WD, Chiron C, Helen Cross J, Simon Harvey A, Kuzniecky R, Hertz-Pannier L, Gilbert Vezina L.
Guidelines for imaging infants and children with recent-onset epilepsy.
Epilepsia. 2009. PubMed abstract

Hauser WA, Beghi E.
First seizure definitions and worldwide incidence and mortality.
Epilepsia. 2008;49 Suppl 1:8-12. PubMed abstract

Hirtz D, Ashwal S, Berg A, Bettis D, Camfield C, Camfield P, Crumrine P, Elterman R, Schneider S, Shinnar S.
Practice parameter: evaluating a first nonfebrile seizure in children: report of the quality standards subcommittee of the American Academy of Neurology, The Child Neurology Society, and The American Epilepsy Society.
Neurology. 2000;55(5):616-23. PubMed abstract / Full Text
Recommendations are based on a three-tiered scheme of classification of evidence found in literature review.

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This parameter reviews published literature relevant to the decision to begin treatment after a child or adolescent experiences a first unprovoked seizure and presents evidence-based practice recommendations. Reasons why treatment may be considered are discussed. Evidence is reviewed concerning risk of recurrence as well as effect of treatment on prevention of recurrence and development of chronic epilepsy. Studies of side effects of anticonvulsants commonly used to treat seizures in children are also reviewed.

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Marson AG, Al-Kharusi AM, Alwaidh M, Appleton R, Baker GA, Chadwick DW, Cramp C, Cockerell OC, Cooper PN, Doughty J, Eaton B, Gamble C, Goulding PJ, Howell SJ, Hughes A, Jackson M, Jacoby A, Kellett M, Lawson GR, Leach JP, Nicolaides P, Roberts R, Shackley P, Shen J, Smith DF, Smith PE, Smith CT, Vanoli A, Williamson PR.
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