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Childhood Absence Epilepsy

CAE


Presentation

  • Review of published reports confirmed this presentation to be atypical of this developmental lesion. The authors describe their patient and discuss this unexpected association and the relevant reports briefly.[ncbi.nlm.nih.gov]
Physician
  • We call on physicians to report their experiences of V album in epilepsy and suggest further study.[ncbi.nlm.nih.gov]
Cognitive Deficit
  • In contrast, treated childhood absence epilepsy patients only exhibited cognitive deficits in visual attention. There were no significant between-group differences for other cognitive tests.[ncbi.nlm.nih.gov]
Myoclonic Jerking
  • We report a patient who at 11 months of age showed isolated myoclonic jerks occurring several times a day.[ncbi.nlm.nih.gov]
  • Similarly, subtypes have been posited including a form with only self‐remitting absence seizures, a subtype with absences and GTCS, a form with absences, GTCS and myoclonic jerks, and also eyelid myoclonia with typical absence epilepsy (EMA) ( Appleton[doi.org]
Behavior Problem
  • Abstract OBJECTIVE: To characterize pretreatment behavioral problems and differential effects of initial therapy in children with childhood absence epilepsy (CAE).[ncbi.nlm.nih.gov]
Forgetful
  • Item analysis within the subscales revealed that children with CAE demonstrate higher rates of hyperactive (overactivity and fidgetiness) and inattentive (forgetfulness and distractibility) problems, and require more supervision.[ncbi.nlm.nih.gov]

Workup

3 Hz Spikes
  • A young female presented with an epileptic syndrome resembling childhood absence epilepsy, a normal neurologic examination, generalized 3-Hz spike-and-wave discharges, and clinical absences. Her seizures responded to treatment with valproic acid.[ncbi.nlm.nih.gov]
  • Diagnosis is made upon history of absence seizures during early childhood and the observation of 3 Hz spike-and-wave discharges on an EEG.[en.wikipedia.org]
  • The phenotype was classical CAE with pyknoleptic absences affecting more girls (six out of eight), associated with typical 3 Hz spike and wave, and remission before puberty.[doi.org]
Focal Spikes
  • PURPOSE: To better understand the nature of the focal spike-wave discharges (FSWDs) and focally led generalized spike-wave discharges (GSWDs) in typical childhood absence epilepsy (CAE) and by implication their nosologic and taxonomic significance.[ncbi.nlm.nih.gov]
3-4 Hz Spikes
  • Childhood absence epilepsy with tonic–clonic seizures and electroencephalogram 34Hz spike and multispike–slow wave complexes: linkage to chromosome 8q24. Am J Hum Genet 1998 ; 63 : 1117 –29.[doi.org]
Focal Spikes
  • PURPOSE: To better understand the nature of the focal spike-wave discharges (FSWDs) and focally led generalized spike-wave discharges (GSWDs) in typical childhood absence epilepsy (CAE) and by implication their nosologic and taxonomic significance.[ncbi.nlm.nih.gov]
Focal Epileptiform Discharges
  • Among 214 patients with benign focal epileptiform discharges, 5 patients were identified with recorded EEG seizures (2.3%).[ncbi.nlm.nih.gov]

Treatment

  • Seizure frequency was not associated with baseline measures of attention, executive function, or treatment outcome.[ncbi.nlm.nih.gov]

Prognosis

  • The long term prognosis of these patients seems to be good with an excellent response to anticonvulsant therapy. Copyright 2017 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.[ncbi.nlm.nih.gov]

Etiology

  • According the ILAE classification of epileptic seizures and epilepsy the etiology could be symptomatic or cryptogenic.[ncbi.nlm.nih.gov]

Epidemiology

  • Epidemiologic data concerning early-onset CAE is scarce. We tried to specifically analyze the early-onset CAE and compare it with typical CAE.[ncbi.nlm.nih.gov]
  • Epidemiology of different types of epilepsy in school age children of Modena, Italy. Epilepsia 1980 ; 21 : 57 –62. Commission on Classification and Terminology of the International League Against Epilepsy.[doi.org]
Sex distribution
Age distribution

Pathophysiology

  • However, recent interesting observations in animal models suggest that BCECTS and CAE could be pathophysiologically related and that genetic links could play a large role.[ncbi.nlm.nih.gov]

Prevention

  • We found that the mutation in GABRG2 (encoding the gamma2-subunit) abolished in vitro sensitivity to diazepam, raising the possibility that endozepines do in fact exist and have a physiological role in preventing seizures.[ncbi.nlm.nih.gov]

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