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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.
- age-dependent epilepsy, with a typical onset at 5–8 years
- incidence is approximately 6.1 per 100,000 children aged <16 years per year[1]
- 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
- Rarely Children with SeLECTS evolve to Developmental and/or Epileptic Encephalopathy with spike-wave activation in sleep (D/EE-SWAS)which is distinguished by cognitive and language regression
- D/EE-SWAS incorporates the several syndromes previously named Landau-Kleffner syndrome, Epileptic Encephalopathy with Continuous Spike-Wave in Sleep and Atypical benign partial epilepsy (pseudo-Lennox syndrome). All these conditions share some EEG features.
Seizure Semiology
- Seizures are brief, typically less than 2-3 minutes
- usually few in number (most children have less than 10 lifetime seizures) and may occur sporadically, with frequent seizures seen over a few days or weeks and then several months until
the next seizure
- Seizures occur during sleep in 80-90% of patients and only while awake in fewer than 20% of children
- Seizures 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 seizure1)
- cognitive (e.g. gustatory hallucinations), emotional(e.g. fear), and autonomic features are not seen2)
EEG
- 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.
- 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-and slow-wave activity 42–44
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
1
If a child has atypical absence seizures, focal atonic seizures, or focal motor seizures with negative myoclonus, loses their balance, and falls, this could mean they have progressed to D/EE-SWAS. It is important to look for evidence of cognitive difficulties or regression.
2
if prolonged focal non-motor seizures with prominent autonomic
features, especially ictal vomiting is present then Self-Limited Epilepsy with Autonomic Seizures (SeLEAS) (formerly called Panayiotopoulos syndrome or early-onset benign occipital epilepsy) should be considered
1.
a
International League Against Epilepsy classification and definition of epilepsy syndromes with onset in childhood: Position paper by the ILAE Task Force on Nosology and Definitions. Epilepsia. 2022 Jun;63(6):1398-1442. doi: 10.1111/epi.17241. Epub 2022 May 3.
[PMID: 35503717] [DOI: 10.1111/epi.17241] .
[PMID: 35503717] [DOI: 10.1111/epi.17241] .
2.
a
Mutations in GRIN2A cause idiopathic focal epilepsy with rolandic spikes. Nat Genet. 2013 Sep;45(9):1067-72. doi: 10.1038/ng.2728. Epub 2013 Aug 11.
[PMID: 23933819] [DOI: 10.1038/ng.2728] .
[PMID: 23933819] [DOI: 10.1038/ng.2728] .
3.
a
Update on the genetics of the epilepsy-aphasia spectrum and role of GRIN2A mutations. Epileptic Disord. 2019 Jun 1;21(S1):41-47. doi: 10.1684/epd.2019.1056.
[PMID: 31149903] [DOI: 10.1684/epd.2019.1056] .
[PMID: 31149903] [DOI: 10.1684/epd.2019.1056] .
Discussion