Early‐onset pure absence epilepsy: a distinct epileptic syndrome

Verrotti, Alberto; Agostinelli, Sergio; Olivieri, Cristina; Chiarelli, Francesco; Curatolo, Paolo

doi:10.1111/j.1651-2227.2011.02213.x

Cited by 11 publications

(6 citation statements)

References 24 publications

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“…Canafoglia and coworkers [ 40 ] that the enhanced outflow of frontal oscillations may be helpful to distinguish responders from non-responders. On the contrary, several other publications did not identify the EEG as a useful prognostic predictor [ 7 , 10 , 15 , 21 ]. Likewise, in our report no substantial prognostic difference was found between patients with or without focal EEG abnormalities, thus not supporting a relationship between focal dysfunctions and resistance to the ASM treatment.…”

Section: Discussionmentioning

confidence: 89%

“…The CAE subgroup was defined based on the ILAE Task Force 1989 criteria [ 14 ]: (1) onset of typical absences (TAs) before puberty in an otherwise normal child; (2) absence seizures as the predominant seizure type at time of diagnosis; (3) very frequent (several to many per day) absences; and (4) EEG showing bilateral, symmetrical spike-waves, usually 3 Hz, on normal background activity. Patients with TAs under the age of 3 were classified as “Early Onset Absence Epilepsy” (EOAE) and thus excluded from the study, since recent studies have considered it to be a distinct epileptic syndrome [ 15 , 16 ].…”

Section: Methodsmentioning

confidence: 99%

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Clinical and Instrumental Follow-Up of Childhood Absence Epilepsy (CAE): Exploration of Prognostic Factors

et al. 2022

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Background: Idiopathic generalized epilepsies (IGEs) represent 15–20% of all cases of epilepsy in children. This study explores predictors of long-term outcome in a sample of children with childhood absence epilepsy (CAE). Methods: The medical records of patients with CAE treated at a university paediatric hospital between 1995 and 2022 were systematically reviewed. Demographics and relevant clinical data, including electroencephalogram, brain imaging, and treatment outcome were extracted. Outcomes of interest included success in seizure control and seizure freedom after anti-seizure medication (ASM) discontinuation. An analysis of covariance using the diagnostic group as a confounder was performed on putative predictors. Results: We included 106 children (age 16.5 ± 6.63 years) with CAE with a mean follow-up of 5 years. Seizure control was achieved in 98.1% (in 56.6% with one ASM). Headache and generalized tonic-clonic seizures (GTCS) were more frequent in children requiring more than one ASM (p < 0.001 and p < 0.002, respectively). Of 65 who discontinued ASM, 54 (83%) remained seizure-free, while 11 (17%) relapsed (mean relapse time 9 months, range 0–18 months). Relapse was associated with GTCS (p < 0.001) and number of ASM (p < 0.002). Conclusions: A history of headache or of GTCS, along with the cumulative number of ASMs utilized, predicted seizure recurrence upon ASM discontinuation. Withdrawing ASM in patients with these characteristics requires special attention.

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Section: Discussionmentioning

confidence: 89%

Section: Methodsmentioning

confidence: 99%

Clinical and Instrumental Follow-Up of Childhood Absence Epilepsy (CAE): Exploration of Prognostic Factors

et al. 2022

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“…Our study's primary goal was to test the neurocognitive performance of patients with CAE in comparison with healthy controls. In the studied sample we included only children who developed absence seizures above the age of four years, to exclude early‐onset pure absence epilepsy, which has earlier seizure onset than the typical CAE 17 . Also, in our study we excluded patients with history of generalised tonic‐clonic seizures or myoclonus and restricted it to right‐handed individuals.…”

Section: Discussionmentioning

confidence: 99%

Assessing cognitive and executive function in childhood absence epilepsy

Salama

Saadallah

Hamed

et al. 2023

Prog Neurol Psychiatry

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Although cognitive impairment is one of the common problems among many patients with epilepsy, it is still not well defined in childhood absence epilepsy. Here, the authors assess the cognitive and executive function in childhood absence epilepsy in a group of school‐age children in Egypt. They discuss the need for early identification of neuropsychological dysfunction so that appropriate management can be implemented.

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“…Less commonly, absence seizures may arise before the 4th year of age (Early-onset Absence Epilepsy), and may be associated with other neurological disorders (other types of seizures, developmental delay, and movement disorders) [60]. Recently it has been suggested that Early-onset pure absence epilepsy (EOAE) is a distinct epilepsy characterized by absences starting from a few months to 4 years of age, normal early psychomotor development, good AEDs seizure control and normal intellectual outcome [61]. …”

Section: Introductionmentioning

confidence: 99%

"Electro-clinical syndromes" with onset in paediatric age: the highlights of the clinical-EEG, genetic and therapeutic advances

et al. 2011

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The genetic causes underlying epilepsy remain largely unknown, and the impact of available genetic data on the nosology of epilepsy is still limited. Thus, at present, classification of epileptic disorders should be mainly based on electroclinical features. Electro-clinical syndrome is a term used to identify a group of clinical entities showing a cluster of electro-clinical characteristics, with signs and symptoms that together define a distinctive, recognizable, clinical disorder. These often become the focus of treatment trials as well as of genetic, neuropsychological, and neuroimaging investigations. They are distinctive disorders identifiable on the basis of a typical age onset, specific EEG characteristics, seizure types, and often other features which, when taken together, permit a specific diagnosis which, in turn, often has implications for treatment, management, and prognosis. Each electro-clinical syndrome can be classified according to age at onset, cognitive and developmental antecedents and consequences, motor and sensory examinations, EEG features, provoking or triggering factors, and patterns of seizure occurrence with respect to sleep. Therefore, according to the age at onset, here we review the more frequently observed paediatric electro-clinical syndrome from their clinical-EEG, genetic and therapeutic point of views.

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Early‐onset pure absence epilepsy: a distinct epileptic syndrome

Abstract: Early-onset pure absence epilepsy is a distinct epilepsy characterized by absences starting from a few months to 4 years of age, normal early psychomotor development, good antiepileptic drug seizure control and normal intellectual outcome.

Cited by 11 publications

References 24 publications

Clinical and Instrumental Follow-Up of Childhood Absence Epilepsy (CAE): Exploration of Prognostic Factors

Clinical and Instrumental Follow-Up of Childhood Absence Epilepsy (CAE): Exploration of Prognostic Factors

Assessing cognitive and executive function in childhood absence epilepsy

"Electro-clinical syndromes" with onset in paediatric age: the highlights of the clinical-EEG, genetic and therapeutic advances

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