Seizures/Epilepsy

Overview

Pediatricians care for a wide range of children with seizures and epilepsy including the typically developing child with infrequent seizures and the severely impaired child with complicated medical regimens. This module focuses on assessing and managing children with uncomplicated clinical situations involving seizures and epileptic syndromes. Less common seizure types and epilepsy syndromes, as well as the emergency evaluation and management of seizures, are not covered here. The Portal provides information about some specific seizure syndromes, including: Seizure (or convulsion) involves a sudden, involuntary, time-limited alteration in behavior, motor activity, autonomic function, consciousness, or sensation, and it is 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 is a condition in which an individual has recurrent, unprovoked seizures. While most seizure syndromes are associated with abnormal EEGs, not all abnormal EEGs are associated with seizures. The aim of seizure treatment is preventing recurrent alterations in behavior, motor activity, or consciousness that are caused by abnormal, episodic brain activity.

Status epilepticus is a common, life-threatening neurologic disorder that is defined as more than 5 minutes of continuous seizure activity or shorter recurrent seizures without recovery of consciousness between seizures. Status epilepticus most commonly occurs in children with known epilepsy following medication changes or missed doses, but it also can occur as the first manifestation of an epileptic syndrome or with a neurologic insult such as a stroke. Most seizures will stop on their own within 5 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.)

Other Names & Coding

Convulsions
Fits
ICD-10 coding

R56.xx, convulsions

G40.xxx, epilepsy

ICD-10 uses 2 general classifications for seizure disorders. The R56 series is for convulsions (which may or may not be epileptic seizures), certain kinds of seizures (e.g., febrile seizures), or seizures not otherwise specified. The G40 series is for epilepsy and epileptic syndromes. The x indicates additional digits that are required for billing. For more specific codes, see Coding for Convulsions (icd10data.com) and Coding for Epilepsy (icd10data.com).

Prevalence

Pediatric epilepsy incidence in Norway was reported as 144 per 100,000 in the first year of life and 58 per 100,000 for those 1-10 years old. [Aaberg: 2017] The cumulative incidence of epilepsy at age 10 years was 0.66% with the majority (0.62%) having active epilepsy. These numbers were similar to those reported in another developed country where incidence of epilepsy appears to have stabilized. [Meeraus: 2013] In 2015, 1.2% of the US population had active epilepsy (95% CI = 1.1-1.4), which translates to about 3 million adults and 470,000 children. [National: 2018]

Genetics

Although seizures may run in families, inheritance patterns are hard to predict. In some children and syndromes, epilepsy is likely a polygenic disorder and may require a combination of genetic and environmental susceptibilities to be expressed. Sometimes the clinical presentation suggests the epilepsy syndrome; clinical testing is available to diagnose some of these syndromes. See [Deprez: 2009] and [Steinlein: 2008] for more information.

Prognosis

The 2 primary elements of prognosis are 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 1 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 2 medications for control, or will change medication before control is reached.
    • 20-30% of patients will have intractable epilepsy that does not respond completely to multiple medications and/or other treatments (e.g., ketogenic diet, surgery, or vagal nerve stimulator).
  • Up to 75% of children will experience prolonged remission from seizures and will be able to stop medication after 2 or more years.

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

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

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

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

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 after an initial visit to a neurologist to confirm the diagnosis. Many of the other epilepsies, including syndromes such as Lennox-Gastaut, will require concurrent care with a pediatric neurologist. A primary care clinician may wish to refer a patient to neurology when discontinuation of anti-epileptic drugs (AEDs) is being considered.

The medical home should also assist the child and family with counseling about safety, educating others about responding to seizures (extended family, other caregivers, school teachers, and classmates, etc.), coordinating care with specialists, assessing and managing medication side effects and secondary impacts of seizures or their underlying cause, and supporting decisions regarding treatment or tapering medications.

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 the Medical Home Portal's 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 and Physical Exam Form (PDF Document 88 KB) may help guide seizure history and physical exam.

Pearls & Alerts for Assessment

Flashing lights can trigger seizures

Seizures triggered by flashing lights occur in approximately 9% of people with epilepsy. It 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, but it is often between 15 and 80 Hertz. Sensitivity to flashing lights may be somewhat predicted by an EEG, which usually involves provocation with lights flashing at varying frequencies. [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 a 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 seizures

A syncopal event, followed by some jerking after loss of consciousness, is not considered a seizure and the cause of the jerking differs from that associated with seizures. These children do not generally need to be seen by pediatric neurology.

Screening

For the Condition

No screening is recommended (or possible) for seizures/epilepsy.

For Complications

Children with epilepsy should receive periodic 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 (AAN) (PDF Document 41 KB) adapted from [Hirtz: 2000] may be helpful.

Seizure Type
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 (e.g., 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).
  • Focal onset seizures (also called localized or partial seizures) have symptoms at onset that 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, tingling on one side of the body, a sense of fear, or 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 do 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 are common and generally benign. These often are familial and may be part of Generalized Epilepsy with Febrile Seizures Plus (GEFS+), a syndrome that is described further in [Scheffer: 2005].
    • Benign myoclonic seizures - generally benign
    • Early infantile epileptic encephalopathy and early myoclonic epilepsy - usually severe with very poor prognosis (Dravet syndrome)
    • Infantile Spasms - usually severe with very poor prognosis
    • Lennox-Gastaut Syndrome - usually severe with 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
    • Benign Rolandic epilepsy (BRE) or Benign Epilepsy with Centrotemporal Spikes (BECTS) are focal onset seizures of the face and arm, occur mostly at night, often familial, and often not treated with AEDs .
    • Epilepsy with Generalized Tonic-Clonic (Grand Mal) Seizures Upon Awakening (EGMA) is 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 they 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 (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, such as Syncope, in a typically-developing child. Families can be given a Seizure Diary (PDF Document 75 KB) to record descriptions of events and determine patterns. 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 may be the best approach. Because it is difficult to be certain that a seizure has not occurred, the child should be reevaluated if a new or similar event occurs. 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 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 Condition

Possible underlying conditions include brain tumors, congenital brain malformations, and metabolic disorders. External and underlying caused should be ruled out before making a diagnosis of primary seizure disorder or epilepsy.

Comorbid & Secondary Conditions

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

History & Examination

Current & Past Medical History

Head trauma is an uncommon cause of seizures but an important diagnosis to consider in the acute setting because subdural or epidural hemorrhage can be life-threatening. Brain tumors are an even less common cause of seizures and also may present 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 is 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,
    • Autonomic – pupillary dilation, drooling, change in heart or respiratory rate, incontinence, pallor, vomiting; loss of consciousness or inability to understand or speak
  • Generalized or focal movements; respiration
    • Change in breathing pattern, cessation of breathing, cyanosis
  • Postictal symptoms (appearance following the seizure)
    • Amnesia for events, confusion, lethargy, sleepiness, headaches, and muscle aches
    • Transient focal weakness (Todd's paralysis), nausea/vomiting, bitten tongue
Important information to guide management will include the number of seizures since the last visit, similarity to past seizures, new medical problems, daily functioning, and school performance (if applicable). An app, like Seizure Tracker (SeizureTracker.com) or others, may be helpful for families to track the number and type of seizures. For children on an AED, ask about the doses the child is taking. Don't assume that the family has continued the AED(s); a number of issues can lead to families changing the dosage or frequency of administration. Sleep problems are common in individuals with epilepsy. [French: 2004]

Family History

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

Pregnancy/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 increases the risk of seizures and epilepsy.

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 psychosocial 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 a substantial impact on families including fear of underlying diagnoses, fear of injury or death during seizures, concerns about the future, and more.

Physical Exam

General

Observe the general 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/Oral

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 (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

An EEG is performed usually on an outpatient basis and not emergently to obtain additional information. The type of seizure and the EEG results will determine if imaging should be performed. An EEG can show the specific area of onset in a focal onset seizure and can confirm the diagnosis of an epilepsy syndrome.

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 seizures.

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

A follow-up EEG may be helpful when seizures change in character or frequency or stop responding to a previously effective AED. EEGs do not need to be performed routinely. 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.

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. These would usually be performed in an emergent setting unless there are signs/symptoms of these disorders in the primary care clinician's office. 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 comprehensive metabolic profiles and CBCs before they are initiated and periodically during treatment, along with drug levels.

Imaging

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 (e.g., abscess or tumor).

Genetic Testing

Genetic testing may be indicated if there is a genetic condition, such as tuberous sclerosis, a metabolic condition causing seizures, or the possibility of a genetic epilepsy syndrome. 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 other epilepsy syndromes are diagnosed clinically. Genetic testing is generally directed by neurology and/or genetics with genetic counseling available for the family. See [Steinlein: 2008] and [Ottman: 2010] for discussions of the use of genetic testing in epilepsy. In a recent article, 24% of children tested with a 110 gene epilepsy panel had either pathogenic or probably pathogenic mutations, with 87 variants found in 30 different genes. [Butler: 2017] Genetic testing for epilepsy will continue to improve and increase in frequency.

Other Testing

Children with epilepsy often have concurrent sleep disorders, and a sleep study with or without overnight EEG may be helpful if there are signs or symptoms of a sleep disorder. [Wilner: 2007]

Specialty Collaborations & Other Services

Pediatric Neurology (see Services below for local providers)

Depending on the comfort of the medical home clinician, a visit to pediatric neurology to confirm the diagnosis and evaluate further 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 local 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 local 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 local providers)

EEG, overnight video EEG, or 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 local 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 to initiate therapy with an anti-epileptic drug (AED) known to be effective for the given seizure type or epilepsy syndrome, and then increase 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.

Pearls & Alerts for Treatment & Management

Families of children with seizures or possible seizures need to know seizure precautions

Children should be observed whenever near water. This includes showering rather than taking a bath unless an adult is present. One-to-one observation by an adult is needed for a child with seizures swimming or in a hot tub. Children should also be observed when around water heaters, campfires, saunas, and cooking areas. 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 (Intermountain Healthcare) for more information.

Seizure action plans

All children with seizures should have an action plan so families and providers know what to do in the case of a breakthrough seizure. See Sample Seizure Action Plan (University of Utah) (PDF Document 67 KB) for an example.

Seizures and driving

Rules for driving after a seizure vary by state. A few states require reporting by the treating physician; most others require the individual with seizures to report to them. There are varying amounts of times that individuals must be seizure free, with or without AEDs, before driving is allowed. State Driving Laws (Epilepsy Foundation) provides a searchable database.

Rescue drugs for breakthrough seizures

A description of rescue drugs should be part of a seizure action plan if the child has prolonged seizures or clusters of seizures. Rescue drugs may include nasal midazolam and rectal valium (Diastat). Nasal midazolam is given to each nostril by a mucosal atomizer device. One “kit,” which is what is used at each administration, consists of the total dosage divided into 2 syringes that each have 2 mucosal atomizer devices (MADs). Pharmacies need to special order the MADs.

Drug interactions and AEDs

Children on carbamazepine should avoid macrolide antibiotics and grapefruit juice because this slows metabolism of the medication. Oral contraceptives may interfere with AED efficacy and vice versa. See [Perucca: 2006] for more information.

Consider possible pregnancy when choosing medication

Certain anti-epileptic drugs (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 pregnant or considering pregnancy to a neurologist.

New treatment on the horizon

Over the next few years, the responsive neurostimulation (RNS) system will likely be used more often. RNS involves a neurostimulator that is implanted in the seizure onset areas of the brain. It consists of a stimulator, implanted leads, a wireless programming wand and associated computer hardware and software. It monitors brain activity in real-time and delivers electrical stimulation in response to seizure activity. It is approved in the US as adjunctive therapy for medically resistant focal onset epilepsy. See Responsive Neurostimulation (Epilepsy Foundation).

New medication for children with Dravet and Lennox Gastaut syndromes

Epidiolex is a new, FDA-approved prescription cannabidiol for the treatment of seizures associated with Lennox-Gastaut syndrome (LGS) or Dravet syndrome. It is structurally different from other antiepileptic medications and offers hope for individuals with these 2 difficult to control epilepsy syndromes. For more information, see CBD for Neurologic Conditions in Children.

How should common problems be managed differently in children with Seizures/Epilepsy?

Development (cognitive, Motor, Language, Social-Emotional)

Children with epilepsy may have delayed development due to either very frequent seizures, the condition causing their epilepsy, or epilepsy medications. Enhanced screening is recommended.

Over the Counter Medications

Many over-the-counter medications, including herbal medications, should be avoided. They can affect epilepsy medication metabolism and cause higher or lower concentrations of the seizure medications in the blood. Acetaminophen is likely safer than ibuprofen for individuals on epilepsy medications. Over-the-Counter Medications and Seizures (Epilepsy Foundation) has information about what medications are considered safe for use by people with epilepsy.

Prescription Medications

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.

Systems

Neurology

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.

End of Visit Instructions (After Visit Summaries), 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.For those requiring medication, levetiracetam is useful for most children with generalized seizures other than absence seizures, and oxcarbazepine is useful for children with focal onset seizures as determined by semiology (description of the event) or EEG. 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.

All epilepsy medications are started at a low dose and gradually increased. 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. Drug rashes are usually generalized; if there is a question, a dermatologist evaluation may be helpful.
  • Levetiracetam (Keppra), available as a generic, is the most common medication started for children with epilepsy. 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 both a liquid and tablets.
  • Oxcarbazepine (Trileptal), also available as a generic, also comes as both a liquid and 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.
  • Lamotrigine can cause a life-threatening rash (toxic epidermal necrolysis or Stevens-Johnson syndrome) and is increased more slowly than other epilepsy medications. Although this rash is rare (1% in children under 16 years of age and 0.3% in adults), families need to be aware so they may monitor for skin reactions. Symptoms that are concerning for a more severe reaction to lamotrigine include joint and muscle pain, fever, general discomfort, and blisters. Note that ethosuximide, while effective for absence seizures, will not control the generalized tonic-clonic seizures that may occur in juvenile absence epilepsy.
When seizures are refractory to the initial AED
It may be that the family is confused about the dosing, 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. Epilepsy surgery to remove lesions responsible for seizures [Conway: 2018], a Vagus Nerve Stimulator (VNS), and responsive neurostimulation are techniques increasingly being used for individuals with intractable epilepsy.

Children with infantile spasms and seizures not responding to first-line treatment should generally be referred to pediatric neurology. Patients with infantile spasms are initially treated with high-dose steroids or, sometimes, vigabatrin. See Infantile Spasms, Treatment & Management.

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. Children with refractory epilepsy may be referred to an epilepsy center for management with multiple AEDs, implantation of a Vagus Nerve Stimulator (VNS), 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])

A new therapy, responsive neurostimulation (RNS) involves a neurostimulator implanted in the seizure onset areas of the brain. It consists of a stimulator, implanted leads, a wireless programming wand and associated computer hardware and software. It monitors brain activity in real-time and delivers electrical stimulation in response to seizure activity. It is approved in the U.S. as adjunctive therapy for medically-resistant focal onset epilepsy. Responsive Neurostimulation (Epilepsy Foundation)

Cannabidiol (CBD oil) and medical marijuana
The use of CBD oil is an evolving area with new information arising frequently. Currently, there is some evidence that CBD may be helpful in certain kinds of epilepsy. A purified form available as the medication Epidiolex is now available for the treatment of epilepsy in individuals with Lennox-Gastaut and Dravet syndromes, and other clinical trials are in progress. Some children experience significant side effects (drowsiness, malaise, elevated transaminases, nausea, diarrhea) and interactions with other epilepsy medications. There is no evidence at this time that medical marijuana is helpful for children with epilepsy.

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

Specialty Collaborations & Other Services

Pediatric Neurology (see Services below for local 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

All children with epilepsy should have a school health plan in place including a Sample Seizure Action Plan (University of Utah) (PDF Document 67 KB) that gives instructions for what to do if that child has a seizure. For children with frequent seizures, nasal midazolam or rectal diazaepam may be prescribed so the child can have it available at school. Education of teachers and other students about epilepsy may be very helpful to enhance acceptance of the child whose seizures aren’t completely controlled and to teach how to best respond if a seizure occurs.

School performance should be monitored at all medical home visits. Learning may be affected by whatever is causing or treating seizures. AEDs cause may not only problems in learning but also fatigue that impairs school performance. Frequent seizures interrupt learning and social functioning. Children with epilepsy will often need to have a 504 plan that specifies needed accommodations. Children who are doing poorly in school may benefit from IQ and achievement testing by child psychology or neuropsychology. See Education & Schools for guidance in communicating with teachers and school personnel to ensure that patients receive appropriate education and school-related services.

Consider the following services:

Specialty Collaborations & Other Services

Mental Health Evaluation/Assessment (see Services below for local providers)

Refer for evaluation and psychometric testing, including IQ and achievement testing, as needed.

Neuropsychiatry/Neuropsychology (see Services below for local providers)

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

Maturation/Sexual/Reproductive

There are several issues in reproductive medicine that need to be followed 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 best avoided when possible in girls who may become pregnant. [French: 2007]

Specialty Collaborations & Other Services

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

Providers who offer gynecological care for girls with special needs.

Pediatric Endocrinology (see Services below for local providers)

Endocrinology may be helpful if precocious puberty is suspected.

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.

Specialty Collaborations & Other Services

General Counseling Services (see Services below for local providers)

Evaluation and management of mood disorders in children with seizures.

Psychiatry/Medication Management (see Services below for local 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.

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). If found to be low, a referral to endocrinology should be considered. When starting medications, Calcium and Vitamin D) intake should be optimized.

Specialty Collaborations & Other Services

Pediatric Endocrinology (see Services below for local providers)

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

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

In a child on valproic acid, periodic reevaluation of bone density may be needed.

Issues Related to Seizures/Epilepsy

Ask the Specialist

What are alternatives to medication for seizures?

Alternative therapies are sometimes offered for certain kinds of epilepsy and selected patients. These therapies include the ketogenic diet, epilepsy surgery, the vagal nerve stimulator, responsive neurostimulation, and cannabidiol (CBD) oil. 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, these alternatives may be tried for intractable seizures. Medications are successful in only about 80% of patients; these alternative and well-studied treatments have been successful additions in helping children achieve seizure control.

Should I consider recommending a ketogenic diet for my patient?

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.

Resources for Clinicians

On the Web

Epilepsy Guidelines (NICE)
Guidance documents for the diagnosis and management of epilepsy in children; National Institute for Health and Clinical Excellence (National Health Service, UK).

Helpful Articles

PubMed search for review articles in the last year about seizures or epilepsy in children or adolescents

Aaberg KM, Surén P, Søraas CL, Bakken IJ, Lossius MI, Stoltenberg C, Chin R.
Seizures, syndromes, and etiologies in childhood epilepsy: The International League Against Epilepsy 1981, 1989, and 2017 classifications used in a population-based cohort.
Epilepsia. 2017;58(11):1880-1891. PubMed abstract

Wirrell E.
Infantile, Childhood, and Adolescent Epilepsies.
Continuum (Minneap Minn). 2016;22(1 Epilepsy):60-93. PubMed abstract

Clinical Tools

Care, Action, & Self-Care Plans

Sample Seizure Action Plan (University of Utah) (PDF Document 67 KB)
One example of a seizure action plan to be written and discussed with parents of children with seizures before they leave the office or emergency department; University of Utah Pediatric Neurology Division.

Questionnaires/Diaries/Data Tools

Seizure Tracker (SeizureTracker.com)
Easy-to-use tool that allows patients to create personalized reports of logged seizure activity and treatment history that can be easily shared with their medical team -free sign-up required.

Seizure Diary (PDF Document 75 KB)
A convenient form for families to use to record seizure events; Medical Home Portal.

Patient Education & Instructions

Let's Talk About Seizures (Intermountain Healthcare)
Brief, 4-page fact sheet about seizures with information about types of seizures, safety during a seizure, and treatment, from Primary Children's Hospital. This handout is available in English and Spanish.

Resources for Patients & Families

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; links to find local and state resources; 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.

National & Local Support

Services for Patients & Families in Rhode Island (RI)

For services not listed above, browse our Services categories or search our database.

* number of provider listings may vary by how states categorize services, whether providers are listed by organization or individual, how services are organized in the state, and other factors; Nationwide (NW) providers are generally limited to web-based services, provider locator services, and organizations that serve children from across the nation.

Authors & Reviewers

Initial publication: April 2013; last update/revision: March 2019
Current Authors and Reviewers:
Author: Lynne M. Kerr, MD, PhD
Authoring history
2018: update: Lynne M. Kerr, MD, PhDA
2013: first version: Matthew Sweney, MDA; Denise Morita, MDA
AAuthor; CAContributing Author; SASenior Author; RReviewer

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Aaberg KM, Surén P, Søraas CL, Bakken IJ, Lossius MI, Stoltenberg C, Chin R.
Seizures, syndromes, and etiologies in childhood epilepsy: The International League Against Epilepsy 1981, 1989, and 2017 classifications used in a population-based cohort.
Epilepsia. 2017;58(11):1880-1891. PubMed abstract

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