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)

Self-Limited Epilepsy with Centrotemporal Spikes (SeLECTS), 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 Developmental and/or Epileptic Encephalopathy with spike-wave activation in sleep (D/EE-SWAS). D/EE-SWAS now incorporates several well-known syndromes previously named Landau-Kleffner syndrome, Epileptic Encephalopathy with Continuous Spike-Wave in Sleep and Atypical benign partial epilepsy (pseudo-Lennox syndrome).

• incidence is approximately 6.1 per 100,000 children aged <16 years per year
• age-dependent epilepsy, with a typical onset at 5–8 years
• Both sexes are affected, with a slight male predominance (60%)
• Most of the time it is a self-limiting epileptic syndrome, with seizure remission within adolescence
• Seizures are overall relatively infrequent, with 60–70% of patients experiencing two to ten seizures lifetime and 10–20% only one

• Seizures predominantly occur during sleep, are usually stereotyped, self-limiting focal (hemifacial) motor with associated sensory features
• somatosensory symptoms, with unilateral numbness or paresthesia of the tongue, lips, gums, and inner cheek
• orofacial motor signs, specifically tonic or clonic contraction of one side of the face, mouth and tongue, then involving one side of the face;
• speech arrest – children have difficulty or are unable to speak (dysarthria or anarthria) but can understand language;
• sialorrhea, a characteristic ictal symptom (unclear whether it is due to increased salivation, swallowing disturbance, or both.)
• seizures may evolve to a focal to bilateral tonic-clonic seizure
• In seizures associated with SeLECTS, cognitive (e.g. gustatory hallucinations), emotional(e.g. fear), and autonomic features are not seen

• normal background activity
• High amplitude centrotemporal sharp-and-slow wave complexes that activate in drowsiness and sleep are mandatory for diagnosis.
• Triphasic, high-voltage (100-microvolts to 300microvolts) sharp waves (initial low-amplitude positivity, then high amplitude negativity followed again by low amplitude positivity), with a transverse dipole (frontal positivity, temporo-parietal negativity), often followed by a high voltage slow wave FIXME37,38.
• The discharges may be isolated or occur in trains of doublets and triplets, and focal, rhythmic, slow activity is occasionally observed in the same region as the spikes.
• The discharges may be unilateral or bilateral and independent (Figure 2A)
• There may be discharges seen outside the centrotemporal region (midline, parietal,frontal, occipital).
• A marked increase in the frequency of epileptiform activity in drowsiness and sleep always occurs.
• The EEG pattern may also change such that sharp- or spike-and-slow waves have a broader field and become bilaterally synchronous (Figure 2B).
• In 10-20% of children, centrotemporal sharp- or spike-and-slow wave may be activated by sensory stimulation of the fingers or toes 40
• Seizures are typically infrequent - it is rare to obtain an ictal recording and there are few published reports in the literatureFIXME.
• Seizures may be accompanied by a brief decrease in amplitude of the background EEG, followed by diffuse sharp wave discharges of increasing amplitude, predominantly in one centrotemporal region41, followed by high amplitude slowing and then a return to the usual interictal EEG (Figure 2C).
• With focal to bilateral tonic-clonic seizures, ictal rhythms may become bilaterally synchronous (as opposed to generalized) sharp- or spike-andslow-wave activity 42–44

If a continuous spike-and-slow-wave pattern is present in sleep, the child should be evaluated for progressive language or cognitive impairment or regression. This EEG pattern should only lead to a diagnosis of D/EE-SWAS if developmental plateauing or regression is also present 21,39. Patients with SeLECTS 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.

• 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.
• Mutations in GRIN2A have been implicated in various types of Epilepsy-Aphasia-Spectrum (EAS) disordersS^[1][2]