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Early epileptiform EEG activity in infants with tuberous sclerosis complex predicts epilepsy and neurodevelopmental
Jessie De Ridder1, Birgit Verhelle1, Jan Vervisch1
1Department of Development and Regeneration, Section Pediatric Neurology, Catholic University of Leuven (KU Leuven), Leuven, Belgium.
Insights
Early electroencephalography (EEG) findings in infants with tuberous sclerosis complex (TSC) can predict epilepsy and neurodevelopmental delays. Identifying epileptiform discharges (ED-EEG) early may guide preventive vigabatrin treatment for better outcomes.
Area of Science:
- Neurology
- Pediatrics
- Genetics
Background:
- Tuberous sclerosis complex (TSC) is a genetic disorder associated with epilepsy and neurodevelopmental challenges.
- Early identification of epilepsy risk and neurodevelopmental trajectories in infants with TSC is crucial for timely intervention.
Purpose of the Study:
- To investigate the association between the timing and characteristics of the first electroencephalography (EEG) with epileptiform discharges (ED-EEG) and the development of epilepsy and neurodevelopmental outcomes at 24 months in infants with TSC.
- To evaluate the predictive value of early EEG findings for epilepsy severity and response to treatment.
Main Methods:
- Prospective enrollment of infants with TSC in the EPISTOP trial with serial EEG monitoring up to 24 months.
- Analysis of the timing and characteristics of the first ED-EEG in relation to clinical outcomes, including epilepsy development and neurodevelopmental quotients.
- Comparison of epilepsy outcomes between a conventionally treated group and a preventive treatment group receiving vigabatrin.
Main Results:
- 95% of infants developed epileptiform discharges, with a median age of 77 days.
- Earlier onset of ED-EEG was significantly associated with lower cognitive, language, and motor development at 24 months.
- Earlier and multifocal ED-EEG predicted a higher risk of drug-resistant epilepsy, while preventive vigabatrin treatment delayed seizure onset when multifocal ED-EEG was present.
Conclusions:
- Early EEG findings in infants with TSC are valuable for identifying those at high risk for severe epilepsy and neurodevelopmental delay.
- These findings can inform the initiation of preventive vigabatrin treatment to potentially improve epilepsy outcomes.
Objective:
To study the association between timing and characteristics of the first electroencephalography (EEG) with epileptiform discharges (ED-EEG) and epilepsy and neurodevelopment at 24 months in infants with tuberous sclerosis complex (TSC).
Methods:
Patients enrolled in the prospective Epileptogenesis in a genetic model of epilepsy - Tuberous sclerosis complex (EPISTOP) trial, had serial EEG monitoring until the age of 24 months. The timing and characteristics of the first ED-EEG were studied in relation to clinical outcome. Epilepsy-related outcomes were analyzed separately in a conventionally followed group (initiation of vigabatrin after seizure onset) and a preventive group (initiation of vigabatrin before seizures, but after appearance of interictal epileptiform discharges [IEDs]).
Results:
Eighty-three infants with TSC were enrolled at a median age of 28 days (interquartile range [IQR] 14-54). Seventy-nine of 83 patients (95%) developed epileptiform discharges at a median age of 77 days (IQR 23-111). Patients with a pathogenic TSC2 variant were significantly younger (P-value .009) at first ED-EEG and more frequently had multifocal IED (P-value .042) than patients with a pathogenic TSC1 variant. A younger age at first ED-EEG was significantly associated with lower cognitive (P-value .010), language (P-value .001), and motor (P-value .013) developmental quotients at 24 months. In the conventional group, 48 of 60 developed seizures. In this group, the presence of focal slowing on the first ED-EEG was predictive of earlier seizure onset (P-value .030). Earlier recording of epileptiform discharges (P-value .019), especially when multifocal (P-value .026) was associated with higher risk of drug-resistant epilepsy. In the preventive group, timing, distribution of IED, or focal slowing, was not associated with the epilepsy outcomes. However, when multifocal IEDs were present on the first ED-EEG, preventive treatment delayed the onset of seizures significantly (P-value <.001).
Significance:
Early EEG findings help to identify TSC infants at risk of severe epilepsy and neurodevelopmental delay and those who may benefit from preventive treatment with vigabatrin.
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