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Care of pharmaco-resistant absence seizures in childhood
M Le Roux1, N Benallegue1, S Gueden1
1Department of Pediatric Neurology, CHU d'Angers, Angers, France.
Insights
For drug-resistant childhood absence epilepsy, ruling out glucose transporter type 1 deficiency is crucial. Prioritizing reduced cognitive side effects is key when standard treatments fail.
Area of Science:
- Neurology
- Pediatric Neurology
- Epileptology
Background:
- Childhood absence epilepsy (CAE) presents pharmaco-resistance in 20-30% of cases.
- Glucose transporter type 1 deficiency (GSD-1) should be considered in early-onset CAE with neurological signs.
- Refractory epilepsy syndromes necessitate careful management to minimize cognitive impact.
Purpose of the Study:
- To review therapeutic strategies for pharmaco-resistant childhood absence epilepsy.
- To highlight the importance of considering specific etiologies like GSD-1.
- To emphasize prioritizing cognitive side effect management in refractory CAE.
Main Methods:
- Literature review of pharmaco-resistant epilepsy syndromes in childhood.
- Analysis of treatment guidelines for absence epilepsy and related disorders.
- Discussion of diagnostic criteria for GSD-1 in the context of epilepsy.
Main Results:
- Ethosuximide, valproate, and lamotrigine monotherapy or combination failure leaves limited options for CAE.
- Myoclonic absences and epilepsy with eyelid myoclonia often exhibit pharmaco-resistance.
- Atypical absences in developmental/epileptic encephalopathies require etiological investigation for optimal treatment.
Conclusions:
- In refractory CAE, GSD-1 must be excluded, especially with early onset and neurological signs.
- Cognitive side effects of anti-epileptic drugs should be minimized in refractory CAE.
- Accurate epilepsy syndrome characterization and etiological diagnosis are vital for managing atypical absences.
Abstract:
In childhood absence epilepsy, pharmaco-resistance occurs in 20-30% of patients. In that situation, glucose transporter type 1 deficiency has to be ruled out, especially if absences started before the age of four years and if neurological signs are present. If ethosuximide, valproate and lamotrigine have failed in monotherapy or in association, there are currently no valuable therapeutic options. The same rules apply for epilepsy with myoclonic absences. Importantly, arguments supporting that making the patient seizure-free will improve eventual associated cognitive deficits such as attention deficit are very weak. Therefore, limiting the cognitive side effects of the anti-epileptic drugs has always to be a priority when faced with typical refractory absences in childhood. In epilepsy with eyelid myoclonia, the majority of patients are pharmaco-resistant. However, absence seizures, if present, tend to be very brief, and seizures are limited in many patients to eyelid myoclonia that eventually do not affect their quality of life and are well attenuated by wearing blue lenses. Atypical absences occurring in the course a developmental and/or epileptic encephalopathy are often pharmaco-resistant. In that situation, characterizing the type of epilepsy syndrome and searching for a specific genetic or structural etiology are needed to offer the best therapeutic options to the patient.
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