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Basic Information

AUTHOR: 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 18% of all pediatric epilepsy syndromes

Predominant Sex and Age

More common in girls than in boys, absences typically begin between 4 and 10 yr and remit by age 201

Peak Incidence

6 to 7 yr

Physical Findings & Clinical Presentation

  • Patients with absence seizures usually have normal physical and neurologic examinations.
  • During the seizures, the patients are unresponsive and can have motor phenomena (automatisms, eye blinks, mouth and hand movements).
  • 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
Workup

  • 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.

TABLE E1 Comparison of Absence and Complex Partial Seizures

FeatureAbsence SeizureComplex Partial Seizure
Neurological statusNormalMay have positive history or examination
Age at onsetChildhood or adolescenceAny age
Aura or warningNoCommon
OnsetAbruptGradual
DurationSecondsTypically 1-2 minutes
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.

TABLE E2 Differentiating Absence Seizures and Focal Seizures with Impaired Awareness

FeatureAbsence SeizureFocal Seizure with Impaired Awareness
DurationSecondsMinutes
Provoking maneuverHyperventilation
Photic stimulation
Variable, but often none
Post-ictal phaseNone (return immediately to baseline)Confusion, sleepiness
Number of seizuresMany per dayInfrequent (rarely >1/day)
EEG featuresInterictal: 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
NeuroimagingNormalNormal, 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.

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: 10 mg/kg/day; then after 7 days, 20 mg/kg
  • Divalproex sodium (Depakote): Initial dose: 5 to 10 mg/kg/day (divided bid), maximum dose: 60 mg/kg/day
  • Lamotrigine (Lamictal): Dose for patients 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 yr.
  • 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 T.A. : Ethosuximide, valproic acid, and lamotrigine in childhood absence epilepsyN Engl J Med. ;362(9):790-799, 2010.