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Published on: September 6, 2017
Early Subclinical Status Epilepticus May Contribute to Developmental Delays in Infants With Tuberous Sclerosis
Alexandria E Melendez-Zaidi1, Kayla L Pence2, Rohini Coorg1
1Division of Neurology, Department of Pediatrics, Texas Children's Hospital & Baylor College of Medicine, Houston, Texas.
Insights
Early diagnosis of tuberous sclerosis complex (TSC) in a newborn, prompted by cardiac rhabdomyoma, led to early detection of subclinical seizures. Prompt treatment with antiseizure medications, including vigabatrin, may improve neurodevelopmental outcomes.
Area of Science:
- Neonatal Neurology
- Genetics
- Pediatric Cardiology
Background:
- Tuberous Sclerosis Complex (TSC) is a genetic disorder that can cause benign tumors to grow in various organs, including the heart and brain.
- Cardiac rhabdomyomas are a common prenatal finding in TSC, often leading to early diagnosis.
- Neonatal seizures are a known complication of TSC, but continuous EEG monitoring is not always standard practice.
Observation:
- A newborn with a prenatally diagnosed cardiac rhabdomyoma received an early genetic diagnosis of TSC.
- Presymptomatic electroencephalography (EEG) revealed subclinical seizures, meeting the criteria for status epilepticus on day 1 of life.
- The infant was treated with antiseizure medications (ASMs), including vigabatrin, based on evidence from the EPISTOP and PREVeNT trials.
Findings:
- Early genetic diagnosis of TSC facilitated timely intervention for neonatal seizures.
- Vigabatrin initiation in the neonatal period may help mitigate refractory epilepsy and epileptic spasms in TSC.
- Continuous neonatal EEG monitoring identified subclinical seizures not apparent through clinical examination.
Implications:
- This case highlights the importance of considering neonatal EEG monitoring in newborns with TSC, especially those with cardiac rhabdomyomas.
- Early identification and treatment of neonatal seizures in TSC may reduce the risk of developing infantile spasms.
- Proactive management, including EEG and ASMs, holds potential for improving long-term neurodevelopmental outcomes in infants with TSC.
Abstract:
We present a case of a newborn with a prenatally discovered cardiac rhabdomyoma leading to early genetic diagnosis of tuberous sclerosis complex (TSC). This early diagnosis prompted a presymptomatic electroencephalography (EEG) that revealed subclinical seizures meeting the definition for status epilepticus on day 1 of life. Antiseizure medications (ASMs), including vigabatrin, were started. The EPISTOP and PREVeNT trials demonstrated that early life initiation of vigabatrin may reduce the degree of refractory epilepsy and epileptic spasms (ES) in this population (TSC). Although neonatal seizures are a known entity in TSC, continuous neonatal EEG monitoring is not standard at birth. This case supports early consideration for neonatal EEG monitoring to identify and treat neonatal seizures, reduce risk for infantile spasms, and potentially improve neurodevelopmental outcomes.
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