Robyn Whitney1, Saber Jan2, Maria Zak1, Bláthnaid McCoy3. 1. Division of Pediatric Neurology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada. 2. Division of Pediatric Neurology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada; Department of Pediatrics, Taibah University, Medina, Saudi Arabia. 3. Division of Pediatric Neurology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada; Department of Pediatrics, University of Toronto, Toronto, Ontario, Canada. Electronic address: blathnaid.mccoy@sickkids.ca.
Abstract
BACKGROUND: Seizures are a common early presentation in infants with tuberous sclerosis complex (TSC) and can be preceded by electrographic changes on electroencephalography (EEG) before clinical seizure onset. A limited number of studies have addressed the initial EEG findings in TSC and the outcome of early treatment with antiepileptic medication prior to clinical seizure onset. METHODS: We describe two infants with tuberous sclerosis complex whose surveillance EEG showed focal seizures that were not previously recognized by caregivers. We review previously reported patients with TSC with early EEG findings. Our patients were started on vigabatrin after the onset of focal seizures with the aim of preventing seizure recurrence, halting the possible progression to infantile spasms or focal seizures, and preventing neurodevelopmental decline. RESULTS: Both patients remain seizure free and have reached appropriate developmental milestones. CONCLUSIONS: We recommend early serial EEG monitoring once a diagnosis of TSC is suspected or confirmed in infants. Additional prospective studies are needed to assess the long-term outcome of early antiepileptic drug initiation as soon as electrographic seizure activity is detected.
BACKGROUND:Seizures are a common early presentation in infants with tuberous sclerosis complex (TSC) and can be preceded by electrographic changes on electroencephalography (EEG) before clinical seizure onset. A limited number of studies have addressed the initial EEG findings in TSC and the outcome of early treatment with antiepileptic medication prior to clinical seizure onset. METHODS: We describe two infants with tuberous sclerosis complex whose surveillance EEG showed focal seizures that were not previously recognized by caregivers. We review previously reported patients with TSC with early EEG findings. Our patients were started on vigabatrin after the onset of focal seizures with the aim of preventing seizure recurrence, halting the possible progression to infantile spasms or focal seizures, and preventing neurodevelopmental decline. RESULTS: Both patients remain seizure free and have reached appropriate developmental milestones. CONCLUSIONS: We recommend early serial EEG monitoring once a diagnosis of TSC is suspected or confirmed in infants. Additional prospective studies are needed to assess the long-term outcome of early antiepileptic drug initiation as soon as electrographic seizure activity is detected.
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