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Childhood Epilepsy with centrotemporal spikes
Self-Limited Epilepsy with Centrotemporal Spikes (SeLECTS) (formerly called childhood epilepsy with centrotemporal spikes, benign epilepsy of childhood with centrotemporal spikes or benign rolandic epilepsy)
Childhood Epilepsy with centrotemporal spikes (CECTS), historically referred to as Benign epilepsy with centrotemporal spikes (BECTS) or rolandic epilepsy, is the most common focal epilepsy syndrome of childhood, accounting for 15–20% of all childhood epilepsies. Childhood epilepsy with centrotemporal spikes, atypical childhood epilepsy with centrotemporal spikes, epileptic encephalopathy with continuous spike-and-wave during sleep, and Landau Kleffner syndrome are all conditions that share some EEG features. The focal seizures and cognitive impairment can be mild to severe. They are thought to comprise a phenotypic spectrum from rolandic epilepsy (also benign epilepsy with centrotemporal spikes, BECTS) to atypical benign partial epilepsy (ABPE), Landau-Kleffner syndrome (LKS) and epileptic encephalopathy with continuous spike and waves during slow-wave sleep (CSWS).
Characteristic features
- age-dependent epilepsy, with a typical onset at 5–8 years
- Most of the time it is a self-limiting epileptic syndrome, with seizure remission within adolescence
- Seizures predominantly occur during sleep, are usually stereotyped, self-limiting focal (hemifacial) motor with associated sensory features
- seizures may evolve to a focal to bilateral tonic-clonic seizure
- Seizures are overall relatively infrequent, with 60–70% of patients experiencing two to ten seizures lifetime and 10–20% only one
EEG
- normal background activity
- with stereotyped centrotemporal, rolandic spikes followed by slow waves, unilaterally or bilaterally, which can be synchronous or asynchronous on both sides, or migrate from side to side and even extend to adjacent brain regions.
Patients with CECTS may show “atypical” symptoms, such as starting their seizures early (<4 years old), having side effects from AEDs, having different types of seizures, having seizures that can't be controlled or a history of status epilepticus, having seizures during the day, having atypical EEG abnormalities or peculiar EEG abnormalities activation during sleep, and having developmental delay or neurologic deficits before the seizures start. It is important carry out cognitive and neuropsychological assessments at the time of diagnosis and during the follow-up to trace the trajectories of neuropsychological development of these patients more deeply.
Genetics
- Centrotemporal spikes can be a trait that is passed down through siblings in a way that depends on their age and is autosomally dominant, even if they don't have seizures.
References
[PMID: 23933819] [DOI: 10.1038/ng.2728] .
[PMID: 31149903] [DOI: 10.1684/epd.2019.1056] .
Discussion