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Basic Information

Author: Naomi R. Kass, BA and Joseph S. Kass, MD, JD, FAAN

Definition

Absence seizures are a type of generalized seizure characterized by brief episodes of staring with impairment of consciousness (absence). They usually last no more than 20 to 30 sec. The onset and the end of the seizures are sudden. Patients are typically unaware of the seizure and resume the activity they were performing before the seizure. The electroencephalogram signature of absence seizures consists of a generalized 3-Hz spike and slow wave discharges.

Synonyms

  • Childhood absence epilepsy
  • Seizures, absence
ICD-10CM CODE
G40.309Generalized idiopathic epilepsy and epileptic syndromes, not intractable, without status epilepticus
Epidemiology & Demographics
Incidence:

1 to 10 cases per 100,000 population

Prevalence:

Represents up to 12% to 18% of all pediatric epilepsy syndromes

Predominant Sex & Age:

More common in girls than in boys, absences typically begin between 4 and 10 yr and remit by age 20 yr.1

Peak Incidence:

6 to 7 yr

Physical Findings & Clinical Presentation

  • Patients with absence seizures usually have normal physical and neurologic examinations. However, attention deficit hyperactivity disorder (ADHD), anxiety, mood disorders, and learning disorders are common comorbidities in children with absence seizures.
  • During the seizures, the patients are unresponsive and can have motor phenomena (automatisms, eye blinks, mouth and hand movements).
  • Typical seizure duration is 4 to 20 sec.
  • Absence seizures are not associated with post-ictal confusion.
  • They may be triggered by hyperventilation associated with activity.
  • Tonic clonic seizures are not usually a feature of this syndrome. If the patient also experiences tonic clonic seizures, other etiologies should be investigated, such as juvenile absence epilepsy, juvenile myoclonic epilepsy, complex partial seizures, etc.
Etiology

Genetic

Diagnosis

Differential Diagnosis

It is important to distinguish between focal onset impaired awareness seizures (previously called complex partial seizures) and absence seizures (Tables E1 and E2).

  • Juvenile absence epilepsy
  • Juvenile myoclonic epilepsy
  • Complex partial seizures
  • Focal seizures with altered consciousness
  • Nonepileptic spells comprised of staring
  • GLUT1 deficiency syndrome
  • Epilepsy with eyelid myoclonia
  • Epilepsy with myoclonic absences
  • Atypical absence seizures

TABLE E2 Differentiating Absence Seizures and Focal Seizures With Impaired Awareness

FeatureAbsence SeizureFocal Seizure With Impaired Awareness
DurationSecondsMinutes
Provoking maneuverHyperventilation Photic stimulationVariable, but often none
Post-ictal phaseNone (return immediately to baseline)Confusion, sleepiness
Number of seizuresMany per dayInfrequent (rarely >1/day)
EEG features
  • Interictal: normal except bursts of generalized spike wave and sometimes occipital intermittent rhythmic delta activity
  • Ictal: 3-Hz generalized spike-wave
  • Interictal: normal or focal slowing, sharp waves, or spikes
  • Ictal: focal discharges (with or without spread to contiguous regions or the contralateral hemisphere)
Neurologic examinationNormalNormal, or focal deficits
NeuroimagingNormal*Normal, or focal abnormalities (mesial temporal sclerosis, focal cortical dysplasia, neoplasm, encephalomalacia)
First-line treatmentEthosuximide or valproic acidOften levetiracetam, oxcarbazepine, or lamotrigine are used

EEG, Electroencephalographic.

* In the proper clinical context, and with an appropriate EEG, a diagnosis of absence epilepsy should be made without neuroimaging.

Oxcarbazepine and carbamazepine are relatively contraindicated for children with typical absence epilepsy, as their seizures can be exacerbated by these medications.

From Marcdante KJ et al: Nelson essentials of pediatrics, ed 9, Philadelphia, 2023, Elsevier.

TABLE E1 Comparison of Absence and Complex Partial Seizures

FeatureAbsence SeizureComplex Partial Seizure
Neurologic statusNormalMay have positive history or examination
Age at onsetChildhood or adolescenceAny age
Aura or warningNoCommon
OnsetAbruptGradual
DurationSecondsTypically 1-2 min
AutomatismsSimpleMore complex
Provocation by hyperventilationCommonUncommon
TerminationAbruptGradual
Post-ictal phaseNoConfusion, fatigue
FrequencyPossibly multiple seizures per dayOccasional
Electroencephalogram3 Hz generalized spike-and-waveFocal epileptic discharges or focal slowing
NeuroimagingUsually normal findingsMay demonstrate focal lesions

From Jankovic J et al: Bradley and Daroff’s neurology in clinical practice, ed 8, Philadelphia 2022, Elsevier.

Workup

  • Electroencephalogram (EEG) with hyperventilation and photic stimulation is crucial in the diagnosis (Fig. E1).
  • Ambulatory EEG and video EEG are recommended for patients with diagnostic uncertainty.

Figure E1 Absence epilepsy.

A generalized burst of 3 cps spike-wave complexes appears during hyperventilation.

(From Pina-Garza J, James KC: Fenichel’s clinical pediatric neurology, ed 8, Philadelphia, 2019, Elsevier.)

Laboratory Tests

No specific studies needed

Imaging Studies

  • MRI of the brain with and without contrast should be performed in all epilepsy patients, especially if the EEG does not show the typical characteristic of absence seizures (3-Hz spike and slow wave discharges).
  • Computed tomography scans of the head should be avoided in children due to unnecessary exposure to radiation and low yield of the test except when MRI cannot be obtained.

Treatment

The medication of choice based on the best current evidence available is ethosuximide, followed by valproic acid and lamotrigine.2

Nonpharmacologic Therapy

Not applicable

Acute General Rx

  • Ethosuximide: Initial dose: 5 to 10 mg/kg/day divided into two doses for children <6 yr and 250 mg/kg twice daily for children 6 yr and older; then after 7 days, the usual maintenance dose is 15 to 40 mg/kg/day in divided doses; goal therapeutic concentration is 40 to 100 micrograms/ml.
  • Divalproex sodium (Depakote): Initial dose: 15 mg/kg/day (divided od, bid, or tid); dose is increased by 5 to 10 mg/kg per day at weekly intervals until seizures controlled or side effects prevent further increases; maximum/maintenance dose: 30 to 60 mg/kg/day (divided bid or tid); therapeutic concentration is 50 to 125 micrograms/ml.
  • Lamotrigine (Lamictal): Dose for patients age 2.5 to 13 yr on no other antiepileptic drugs. Wk 1 and 2: 0.3 mg/kg/day. Wk 3 and 4: 0.6 mg/kg/day. Wk 5 onward: Increase every 1 to 2 wk by 0.6 mg/kg/day. Maintenance: 4.5 to 7.5 mg/kg/day. Warning: Should be used with caution due to the potential for toxicity and Stevens-Johnson syndrome. Patients on other antiepileptic drugs can also have severe adverse reactions (e.g., valproate can cause increased levels of lamotrigine, and lamotrigine must be titrated much more slowly in patients on valproate therapy).
Chronic Rx

  • Children with recurrent seizures require chronic treatment.
  • If children are seizure-free for a period of 1 to 2 yr, a trial on no medications should be considered; children typically outgrow childhood absence seizures.
Disposition

  • Response to treatment is excellent.
  • Absence seizures tend to remit in teenage years.
  • Epilepsy can be considered as resolved once 10 yr have elapsed since the last event, including 5 yr free from medications.
Complementary & Alternative Medicine

Not applicable

Referral

Patients with epilepsy should be referred for a consultation by a child neurologist, preferably one specializing in epilepsy.

Pearls & Considerations

Comments

  • Absence seizures can be present in other epilepsy syndromes.
  • Valproate should be avoided in girls and women with childbearing potential due to the risk of teratogenicity.
  • Carbamazepine and phenytoin should be avoided in the treatment of absence seizures since these medications may worsen seizures and provoke absence status epilepticus.
  • All women of childbearing age taking antiepileptic drugs should take folic acid supplementation (1 to 4 mg/day) for the prevention of neural tube defects.
Prevention

Sleep deprivation and alcohol consumption should be avoided.

Patient & Family Education

Patients with ongoing seizures are forbidden to drive; check state regulations and laws regarding driving and epilepsy.

Related Content

  • Absence Seizures (Patient Information)

Related Content

    1. Tolaymat A : Diagnosis and management of childhood epilepsyCurr Probl Pediatr Adolesc Health Care. 45(1):3-17, 2015.
    2. Glauser TA : Ethosuximide, valproic acid, and lamotrigine in childhood absence epilepsyN Engl J Med. 362(9):790-799, 2010.