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Published on: September 6, 2017
Evolution of electroencephalogram in infants with tuberous sclerosis complex and neurodevelopmental outcome: a
Jessie De Ridder1, Katarzyna Kotulska2, Paolo Curatolo3
1Department of Development and Regeneration, Section Pediatric Neurology, Catholic University of Leuven (KU Leuven), Leuven, Belgium.
Insights
Infants with tuberous sclerosis complex (TSC) type 2 (TSC2) show more abnormal electroencephalogram (EEG) findings. These EEG changes predict neurodevelopmental outcomes in children with TSC.
Area of Science:
- Neurology
- Pediatrics
- Genetics
Background:
- Tuberous sclerosis complex (TSC) is a genetic disorder affecting multiple organs.
- Electroencephalogram (EEG) monitoring is crucial for assessing neurological status in infants with TSC.
- Understanding EEG evolution and its link to neurodevelopment is vital for early intervention.
Purpose of the Study:
- To analyze the progression of EEG characteristics in infants diagnosed with TSC.
- To determine the correlation between EEG patterns and neurodevelopmental outcomes at 24 months of age.
- To compare EEG findings between TSC type 1 (TSC1) and TSC type 2 (TSC2) variants.
Main Methods:
- Eighty-three infants from the EPISTOP trial underwent serial EEG monitoring up to 24 months.
- EEG maturation, background activity, and epileptiform discharges were analyzed.
- Comparisons were made between TSC1 and TSC2 variants, and between preventive and conventional treatment groups.
Main Results:
- Infants with TSC2 exhibited more frequent slower posterior dominant rhythms (PDR) and epileptiform foci compared to TSC1.
- A higher proportion of TSC2 cases showed abnormal EEGs at follow-up.
- Slower PDR correlated with lower cognitive and motor scores, while normal EEGs predicted better developmental outcomes and lower autism risk.
Conclusions:
- TSC2 is associated with more pronounced EEG abnormalities than TSC1.
- Specific EEG characteristics in infants with TSC are predictive of their neurodevelopmental trajectory.
- Serial EEG monitoring can aid in predicting neurodevelopmental outcomes in TSC patients.
Aim:
To describe the evolution of electroencephalogram (EEG) characteristics in infants with tuberous sclerosis complex (TSC) and the relationship with neurodevelopmental outcome at 24 months.
Method:
Eighty-three infants were enrolled in the EPISTOP trial and underwent serial EEG follow-up until the age of 24 months (males n=45, females n=37, median age at enrolment 28d, interquartile range 14-54d). Maturation of the EEG background and epileptiform discharges were compared between the TSC1 and TSC2 variants and between preventive and conventional groups respectively.
Results:
Children with TSC2 more frequently had a slower posterior dominant rhythm (PDR) at 24 months (51% vs 11%, p=0.002), a higher number of epileptiform foci (median=8 vs 4, p=0.003), and a lower fraction of EEGs without epileptiform discharges (18% vs 61%, p=0.001) at follow-up. A slower PDR at 24 months was significantly associated with lower cognitive (median=70 vs 80, p=0.028) and motor developmental quotients (median=70 vs 79, p=0.008). A higher fraction of EEGs without epileptiform discharges was associated with a lower probability of autism spectrum disorder symptoms (odds ratio=0.092, 95% confidence interval=0.009-0.912, p=0.042) and higher cognitive (p=0.004), language (p=0.002), and motor (p=0.001) developmental quotients at 24 months.
Interpretation:
TSC2 is associated with more abnormal EEG characteristics compared to TSC1, which are predictive for neurodevelopmental outcome.
