Seizures/Epilepsy

Description

ICD-9

780.3x, seizures

345.xx, epilepsy

ICD-9-CM uses two general classifications for seizure disorders. Those in the 780 series are for convulsions, which may or may not be epileptic seizures, certain kinds of seizures (e.g, febrile seizures), or seizures not otherwise specified, whereas epilepsy and epileptic syndromes are coded for in the 345 group. Most require fourth and even fifth digits (see Seizures ICD9 (PDF Document 64 KB)).

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

Clinical Assessment

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 (PDF Document 88 KB) may help guide your seizure history and physical exam.

Screening

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 (PDF Document 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.

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 (PDF Document 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.

History & Examination

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

General

Observe generally the appearance and interaction of the patient. Absence seizures may be obvious to an examiner. Children with autism spectrum disorders have a higher incidence of seizures. [Tuchman: 2011]

Growth Parameters

Check height, weight, and head circumference for decreased growth that may be due to a genetic syndrome. AEDs may cause weight gain or loss. A head circumference changing percentile lines may reflect an underlying etiology for seizures.

Skin

Look for evidence of tuberous sclerosis.

HEENT

Look for distinctive features. Perform visual and fundoscopic exams. An unexplained bite mark on the front lateral edge of the tongue might be due to a generalized seizure.

Neurologic Exam

Look for focal abnormalities such as asymmetric or focal weakness or sensation, asymmetric reflexes or impaired coordination that would suggest an intracranial abnormality (tumor, vascular or traumatic injury, etc.).

New focal abnormalities (such as weakness, altered reflexes or sensation, impaired coordination) may suggest complications of severe seizures, injury during a seizure, progression of an underlying syndrome, or a new diagnosis.

Testing

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.

Subspecialist Collaborations & Other Resources

Pediatric Neurology (see Services below for relevant providers)

Depending on the comfort of the Medical Home clinician, a visit to pediatric neurology to confirm the diagnosis and for further evaluation may be helpful. Depending on the seizure type, the spectrum of involvement with neurology may range from a one-time visit to concurrent care. Depending on the clinical circumstances, refer to a pediatric neurologist for diagnosis, occasional follow-ups, or concurrent management.

Pediatric Genetics (see Services below for relevant providers)

A consult with genetics may be helpful if a genetic or metabolic basis for the seizures is being considered.

Medical Imaging (see Services below for relevant providers)

Although brain imaging is available in most hospitals, most small children need sedation for brain MRIs and may need referral to a pediatric hospital. MRI is often part of the initial management of the child presenting with seizures and in most cases will not need to be repeated unless seizures change in quality or frequency.

Electroencephalography (EEG) (see Services below for relevant providers)

EEG, overnight video EEG, ambulatory monitoring (when available) will sometimes need to be repeated after the initial evaluation if seizures are proving difficult to control An EEG is also sometimes performed when the patient has been seizure-free on medication for a few years and stopping the medication is being considered.

Sleep Studies/Polysomnography (see Services below for relevant providers)

Sleep studies may be helpful for the child with epilepsy and sleep problems and may be ordered with concurrent EEG if seizures during sleep are suspected

Treatment & Management

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 (PDF Document 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) (PDF Document 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

Neurology

End of Visit Instructions including a seizure action plan should be given to all families in a child with epilepsy. These should include seizure precautions and what to do if there is a seizure. See Evaluation and treatment of a first unprovoked seizure with end of visit information.


Initial Treatment: Some children with seizures might not require any medical therapy. These include those with a first-time generalized seizure, febrile seizures, benign myoclonic epilepsy, and some patients with benign Rolandic epilepsy. For those requiring medication levetiracetam is useful for most children with generalized seizures except absence seizures and oxcarbazepine is useful for children with focal onset seizures as determined by semiology (description of the event) or EEG.
  • Levetiracetam is started at 10 mg/kg twice daily, raised to 20 mg/kg twice daily after 1 week, then 30 mg/kg twice daily. Labs are not needed before or after starting this medication; levels are only rarely checked. There are very few drug interactions and the main side effect, seen in a small percentage of children, is behavioral. (Note behavior may worsen initially but then go back to baseline in some children.) Levetiracetam comes as a liquid (100 mg/ml) and tablets ( ) and is available as a generic.
  • Oxcarbazepine (Trileptal), also available as a generic, is started at and then increased ). It comes as a liquid (300 mg/5 ml) and in tablets. Although some providers will check a blood sodium level before and after starting this medication, no other labs or levels are necessary in the majority of cases.
  • Patients and families should be instructed to call if a rash develops after starting medication. If a rash occurs, the patient should be seen emergently and, if it is probable that it is a drug rash, the medication should be stopped.
  • Patients who have absence epilepsy or juvenile absence epilepsy are exceptions to the rule of levetiracetam as first choice for generalized seizures. Instead, evidence-based medicine suggests they may be treated with ethosuximide, valproic acid, or lamotrigine. Note that ethosuximide, while effective for absence seizures, will not control the generalized tonic-clonic seizures that may occur in juvenile absence epilepsy.
  • Children with infantile spasms and seizures not responding to first line treatment should generally be referred to pediatric neurology. Children with intractable seizures may have one of the epilepsy syndromes that are very difficult to manage. These include Dravet and Lennox-Gastaut syndrome and some of the partial epilepsies. Patients with infantile spasms are initially treated with high-dose steroids or, sometimes, vigabatrin. See Infantile Spasms, Treatment & Management for specific information. See Lennox Gastaut syndrome for specific information. Children with refractory epilepsy may be referred to an epilepsy center for management of multiple AEDs, implantation of a vagal nerve stimulator, or epilepsy surgery. Epilepsy surgery may be the treatment of choice early on for some types of epilepsy and, over the long run, might be less costly and more effective than AEDs. ([Langfitt: 2007])

When seizures are refractory to the initial AED, it may be that the family is confused about the dosing, or not giving the full dose due to concern about side effects or that the seizures are refractory to treatment. Non-epileptic seizures are also a possibility. A referral to pediatric neurology may be helpful to sort out these possibilities.

Stopping medication After a child has been seizure free for 2 years, they may be able to stop taking anti-epileptic medication. For information about when and how to stop AEDs, see Tapering Antiepileptic Medication.

Subspecialist Collaborations & Other Resources

Pediatric Neurology (see Services below for relevant providers)

When, and how often, to consult a pediatric neurologist will depend on the seizure type and the child's response to medication. Diagnosis and treatment of some seizure types, such as childhood absence epilepsy, are straightforward, but the family might benefit from a single consultation to confirm the diagnosis. Children with some of the mixed seizure type epilepsy syndromes will usually need concurrent care by pediatric neurology.

Learning/Education/Schools

School performance should be monitored at all Medical Home visits. Learning may be affected by whatever is causing the seizures or by their treatment. Frequent seizures may interrupt learning and social functioning. AEDs may cause problems in learning and may also cause fatigue that impairs school performance. Children with epilepsy will often need to have a 504 plan in place (See Education and Schools). See additional resources below. Children who are doing poorly in school may benefit from IQ and achievement testing by child psychology or neuropsychology.

Subspecialist Collaborations & Other Resources

Psychologist, Child-18 (see Services below for relevant providers)

Evaluation and psychometric testing including IQ and achievement testing.

Neuropsychology (see Services below for relevant providers)

In a child with a more complicated clinical picture, a full neuropyschological profile may be warranted.

Mental Health/Behavior

Even typically developing children with epilepsy are known to demonstrate an increased incidence of mood disorders. Children with some of the epilepsy syndromes might have associated intellectual disability and demonstrate behaviors that are difficult for parents and teachers. If these problems are noted, a referral to child psychiatry and/or child psychology for medications and/or counseling might be helpful.

Subspecialist Collaborations & Other Resources

Psychologist, Child-18 (see Services below for relevant providers)

Evaluation and management of mood disorders in children with seizures.

Psychiatrist, Child-18 (see Services below for relevant providers)

Evaluation for and treatment of behavior problems and mood disorders is important in children with epilepsy. Although many Medical Home clinicians may feel comfortable prescribing medications for uncomplicated attention problems and depression, issues surrounding these diagnoses and medications are more complicated in children with seizures on medications.

Maturation/Sexual/Reproductive

There are several issues in reproductive medicine that need to be followed and/or managed in some children with seizures. Phenytoin, particularly, can cause excess hair growth that might be confused with precocious puberty. Many of the older drugs increase the metabolism of oral contraceptives, decreasing their efficacy. If AEDs and oral contraceptives are to be used concurrently, the possibility of drug interactions needs to be addressed.

Some seizure medications, such as phenytoin, have been associated with fetal anticonvulsant syndrome, which includes minor facial anomalies that become less obvious as the child matures. [Tomson: 2005] Major anomalies (e.g., cardiac defects, cleft lip/palate, microcephaly) may also be increased when AEDs are taken by the mother. Valproic acid and carbamazepine have been associated with an increased risk of neural tube defects. Valproic acid is also associated with other teratogenic effects and are best avoided when possible in girls who may become pregnant. [French: 2007]

Subspecialist Collaborations & Other Resources

Gynecology (Ped/Adol, Special Needs) (see Services below for relevant providers)

Providers who offer gynecological care for girls with special needs.

Pediatric Endocrinology (see Services below for relevant providers)

Endocrinology may be helpful if precocious puberty is suspected.

Nutrition/Growth/Bone

AEDs, especially valproic acid, have been associated with decreased bone density and fractures. Bone density (by Dexa scan) should be measured as a baseline and at periodic intervals (not more than yearly) and if found to be low, a referral to endocrinology should be considered. When starting medications, calcium and vitamin D intake should be optimized (see Calcium and Vitamin D (general)).

Subspecialist Collaborations & Other Resources

Pediatric Endocrinology (see Services below for relevant providers)

Consultation with endocrinology for possible medical treatment of low bone density may be helpful.

Bone Densitometry/DEXA (see Services below for relevant providers)

In a child on valproic acid, baseline and periodic reevaluation of bone density is recommended.

Frequently Asked Questions

Are there any alternatives to medication for seizures?

Alternative therapies are sometimes offered for certain kinds of epilepsy and for selected patients. These therapies include the ketogenic diet, epilepsy surgery and the vagal nerve stimulator. Because the alternatives are not necessarily any more benign than medication, they are generally used for seizures that have been unresponsive to medications. Neurologists attempt to use single medications first, before adding a second or even third medication or trying an alternative therapy to achieve seizure control. Depending on the family preference and with neurology guidance, the ketogenic diet, the vagal nerve stimulator, or epilepsy surgery may be tried for intractable seizures. Medications are successful in only about 80% of patients and these alternative and well-studied treatments have been successful additions in helping children achieve seizure control.

Isn't the ketogenic diet safer as it doesn't involve medications?

The ketogenic diet is a very controlled low carbohydrate, high fat, and high protein diet. It probably suppresses seizures by causing changes in brain metabolism. As well as being difficult to maintain, the ketogenic diet has potentially harmful side effects. Close following of nutritional status, growth, metabolic labs, and seizures is essential for the safe use of this method of controlling seizures, Nonetheless, it may be a good alternative for patients with difficult to control seizures. See the Ketogenic Diet for more details.

Will my insurance cover these alternative therapies?

Many insurance companies are willing to cover the Ketogenic Diet, the Vagus Nerve Stimulator (VNS) and Epilepsy surgery if the medical records show that antiepileptic medications have not been successful at controlling seizures.

Does my child need to be on seizure medication?

There are many factors that go into the decision of whether to put children on seizure medication, including whether it was the first seizure, were there any provoking factors, is there a risk of further seizures, are seizures harmful to developing brains, and many more. This is a question best discussed with the child's doctor who is able to consider the many individual factors and share the decision making with the family.

Will my child always have to be on seizure medication?

This depends again on the individual child and the type of epilepsy. Many children are able to stop medication after some period of time. For some types of epilepsy, the prognosis is poor for ever getting off medication (juvenile myoclonic epilepsy, Lennox Gastaut syndrome), but other kinds of epilepsy are generally self-limited (absence epilepsy). See Infantile Spasms, Related Issues for specific information based on seizure type.

Issues Related to Seizures/Epilepsy

Clinical Assessment

Seizure Syndrome Type

Nutrition/Growth/Bone

Calcium and Vitamin D

Resources

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

Clinical Tools

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 & Support for Families

Family Diagnosis Page

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.

Services for Patients & Families

Bone Densitometry/DEXA

See all Bone Densitometry/DEXA services providers (3) in our database.

Electroencephalography (EEG)

See all Electroencephalography (EEG) services providers (1) in our database.

Gynecology (Ped/Adol, Special Needs)

See all Gynecology (Ped/Adol, Special Needs) services providers (21) in our database.

Medical Imaging

See all Medical Imaging services providers (3) in our database.

Neuropsychology

See all Neuropsychology services providers (33) in our database.

Pediatric Endocrinology

See all Pediatric Endocrinology services providers (2) in our database.

Pediatric Genetics

See all Pediatric Genetics services providers (5) in our database.

Pediatric Neurology

See all Pediatric Neurology services providers (10) in our database.

Psychiatrist, Child-18

See all Psychiatrist, Child-18 services providers (28) in our database.

Psychologist, Child-18

See all Psychologist, Child-18 services providers (151) in our database.

Seizure Clinics

See all Seizure Clinics services providers (1) in our database.

Sleep Studies/Polysomnography

See all Sleep Studies/Polysomnography services providers (8) in our database.

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

Authors

Authors: Lynne M Kerr, MD, PhD - 4/2013
Matthew Sweney, MD - 4/2013
Denise Morita, MD - 4/2013
Content Last Updated: 4/2013

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