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Encyclopedia of Clinical Neuropsychology pp 6–10Cite as

Absence Epilepsy

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Synonyms

Childhood absence epilepsy; Juvenile absence epilepsy; Petit mal epilepsy

Definition

Childhood and juvenile absence epilepsy are forms of idiopathic (genetic) generalized epilepsy that are characterized by seizures that involve sudden arrest in activity, awareness, and responsiveness, and may include some mild motor features. Typical absence seizures usually last less than 20 s and end as abruptly as they start. Patients have no recollection of the event and often return immediately to their previous activity with little or no postictal alterations in functioning. Generalized 3 Hz spike-and-wave discharges on EEG are required for the diagnosis and are strongly correlated with the clinical events.

Categorization

  • Childhood absence epilepsy (CAE)

  • Juvenile absence epilepsy (JAE)

Epidemiology

The incidence of CAE is estimated at 6–8% per 100,000 in children younger than 15 years, and the estimated prevalence is 10–12% in children younger than 16 years with epilepsy. The incidence...

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References and Readings

  • Berkovic, S. F., & Benbadis, S. (2001). Childhood and juvenile absence epilepsy. In E. Wyllie (Ed.), The treatment of epilepsy: Principles and practice (3rd ed., pp. 485–490). Philadelphia: Lippincott Williams & Wilkins.

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  • Caplan, R., Siddarth, P., Stahl, L., Lanphier, E., Vona, P., Gurbani, S., et al. (2008). Childhood absence epilepsy: Behavioral, cognitive, and linguistic comorbidities. Epilepsia, 49(11), 1838–1846.

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  • Holtkamp, M. (2017). Genetic generalized epilepsies with adolescent onset. In J. M. Pellock, D. R. Nordli Jr., R. Sankar, & J. W. Wheless (Eds.), Pellock’s pediatric epilepsy: Diagnosis and therapy (4th ed., pp. 337–346).

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  • Masur, D., Shinnar, S., Cnaan, A., Shinnar, R. C., Clark, P., Wang, J., Weiss, E. F., Hirtz, D. G., Glauser, T. A., & Childhood Absence Epilepsy Study Group. (2013). Pretreatment cognitive deficits and treatment effects on attention in childhood absence epilepsy. Neurology, 81, 1572–1580.

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  • Pearl, P. L., & Holmes, G. L. (2017). Childhood absence epilepsy. In J. M. Pellock, D. R. Nordli Jr., R. Sankar, & J. W. Wheless (Eds.), Pellock’s pediatric epilepsy: Diagnosis and therapy, (4th ed) pp. 309–336).

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  • Schwartz, J. K., & Titus, J. B. (2015). Executive functioning and behavioral profiles in childhood absence epilepsy and juvenile myoclonic epilepsy [Abstract]. Journal of the International Neuropsychological Society, 21(s1), 87–88.

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  • Verrotti, A., Matricardi, S., Rinaldi, V. E., Prezioso, G., & Coppola, G. (2015). Neuropsychological impairment in childhood absence epilepsy: Review of the literature. Journal of the Neurological Sciences, 359(1–2), 59–66.

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Author information

Authors and Affiliations

  1. Comprehensive Epilepsy Program, Dell Children’s Medical Center of Central Texas, Austin, TX, USA

    Jeffrey B. Titus & Dave Clarke

  2. Department of Psychology, The University of Texas at Austin, Austin, TX, USA

    Jeffrey B. Titus

  3. Department of Educational Psychology, The University of Texas at Austin, Austin, TX, USA

    Hayley Loblein

  4. Department of Pediatrics, Dell Medical School, The University of Texas at Austin, Austin, TX, USA

    Dave Clarke

Authors
  1. Jeffrey B. Titus
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  2. Hayley Loblein
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  3. Dave Clarke
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Corresponding author

Correspondence to Jeffrey B. Titus .

Editor information

Editors and Affiliations

  1. Department of Physical Medicine and Rehabilitation, Virginia Commonwealth University, Richmond, VA, USA

    Jeffrey S. Kreutzer

  2. Kessler Foundation, Pleasant Valley Way, West Orange, NJ, USA

    John DeLuca

  3. Independent Practice, Wynnewood, PA, USA

    Bruce Caplan

Absence Epilepsy – CAE and JAE

Absence Epilepsy – CAE and JAE

EEG signature

Characteristic generalized 3 Hz spike and slow wave (may slow to 2.5 Hz during the absence and may be faster in JAE).

Activated by hyperventilation is more common than photic stimulation.

Age of onset

CAE: 3–10 years

JAE: 10–17 years

Clinical features

Typical events last 4–20 s.

Seizure semiology:

(a) Frequent staring spells with abrupt onset and offset without aura (fewer seen in JAE). Occasional automatic mouth or hand movements or subtle myoclonus.

(b) Generalized tonic-clonic seizures are more common in JAE.

MRI is typically normal.

AED management

Ethosuximide

Valproic acid or lamotrigine can be helpful if generalized tonic-clonic seizures manifest.

Response to treatment with AEDs

Good

Seizure freedom

CAE: Expected by adolescence

JAE: Less common

Functional outcome

CAE: Good

JAE: Unknown

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Cite this entry

Titus, J.B., Loblein, H., Clarke, D. (2018). Absence Epilepsy. In: Kreutzer, J.S., DeLuca, J., Caplan, B. (eds) Encyclopedia of Clinical Neuropsychology. Springer, Cham. https://doi.org/10.1007/978-3-319-57111-9_1504

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