Self-limited epilepsy with centrotemporal spikes (SLECTS) …

Epilepsy & Seizures

Updated 03.17.2026
Released 10.18.1993
Expires For CME 03.17.2029

Self-limited epilepsy with centrotemporal spikes

Authors: K P Vinayan MD DM, Arushi Gahlot Saini MD DM MNAMS

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Editor: Solomon L Moshé MD

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Self-limited epilepsy with centrotemporal spikes

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Introduction

Overview

Self-limited epilepsy with centrotemporal spikes is the new name for the previous epilepsy syndrome of benign epilepsy with centrotemporal spikes. It was first reported in the 1950s and is now recognized as the most frequent epilepsy syndrome in children between the ages of 4 and 13 years. The term “benign” was applied to this syndrome to differentiate it from other sinister causes of focal epilepsies. However, cognitive dysfunction, language impairment, and atypical evolutions with adverse seizure and neurodevelopmental outcomes were rarely seen in this syndrome.

The ILAE Task Force report on the nosology and definitions of childhood-onset epilepsy syndromes proposes the terms “self-limited focal epilepsies” of childhood (SeLFE) for focal epilepsies with onset in childhood and “self-limited epilepsy with centrotemporal spikes” (SeLECTS) for Rolandic epilepsy (203). SeLECTS is the most frequent SeLFE and accounts for 6% to 7% of all childhood epilepsies (235). Its incidence is estimated to be 6.1 per 100,000 children below 16 years of age per year (232).

Key points

	• Presents with focal seizures with dysarthria, sialorrhea, dysphasia, and unilateral clonic or tonic–clonic movement of the mouth, or nocturnal focal to bilateral tonic-clonic seizures.
	• Seizures commonly occur within 1 hour of falling asleep or 1 to 2 hours prior to awakening.
	• There are high-amplitude, centrotemporal, biphasic epileptiform abnormalities on EEG.
	• There is no developmental regression.
	• Remission occurs by mid- to late adolescence.
	• Specific cognitive, language, memory, and attention-related disorders are increasingly being reported.
	• SeLECTS may atypically evolve into Landau-Kleffner syndrome and epileptic encephalopathy with continuous spike-and-wave activation in sleep (EE-SWAS) with potential for persistent neuropsychological impairments.

Historical note and terminology

The Rolandic (centrotemporal) region is named after Luigi Rolando (1773-1831), an Italian anatomist known for his pioneer research in the localization of function in the human brain. A particular EEG pattern with migratory spikes originating over the Rolandic region was first reported in the 1950s (77; 82). In 1958, the first clinical description associated with EEG features was published (158). The same EEG pattern was later correlated with a common form of focal childhood epilepsy, then called "midtemporal epilepsy," characterized by hemifacial or oropharyngeal ictal symptoms and a favorable prognosis (81). Because of the localization of the ictal events, Lombroso proposed the term "sylvian seizures" (144). In the same year, Loiseau and colleagues presented an electroclinical series of 122 children with what they called “a particular form of epilepsy in childhood,” stressing its benign character. Several long-term follow-up studies in the past had confirmed the relatively good prognosis (12; 126; 143). This form of epilepsy was called “benign childhood epilepsy with centrotemporal spikes” and was placed in the group of idiopathic localization-related (focal, local, partial) epilepsies in the International Classification of Epilepsies and Epileptic Syndromes (35; 55).

As new syndromes were recognized within the spectrum of benign childhood focal epilepsies (173), the term “benign” was considered initially acceptable for this syndrome (85). However, the 2017 ILAE Classification of the Epilepsies proposed the term “self-limited” for the “benign” course of epilepsies to reflect a “likely spontaneous resolution of a syndrome” (189). Thus, the new term “self-limited epilepsy with centrotemporal spikes” (SeLECTS) was proposed for this epilepsy syndrome. The word “benign” is sometimes completely omitted to name the syndrome as “childhood epilepsy with centrotemporal spikes” (CECTS) (214). However, the ILAE Task Force on Nosology and Definitions affirms the term SeLECTS for this epilepsy syndrome (203).

Clinical manifestations

Clinical features

Age, sex, and course of illness. The age at onset is typically between 4 and 10 years (range 3 to 14 years) in 90% of patients, with a peak at approximately 7 years. Both sexes are affected, with a slight male predominance (60%). Seizures usually resolve by puberty but can occasionally continue until 18 years of age (18). During the active phase of epilepsy, behavioral and neuropsychological deficits may emerge or worsen, particularly in language and executive functioning, and later improve or resolve. The prognosis for seizure remission is excellent, even for those whose seizures are initially difficult to control (21). The age at seizure onset has been proposed as the most important predictor of early remission, irrespective of the initial EEG findings, antiseizure medication treatment, or seizure frequency (124). Antecedent, birth, and neonatal history; development; cognition; neurologic examination; and head size prior to seizure onset are typically normal. A history of febrile seizures may be seen in 5% to 15% of cases. Preexisting attention-deficit/hyperactivity disorder and specific cognitive function deficits may occur (168). SeLECTS may be seen in children with a history of prior neurologic injury or intellectual disability, but these features are considered coincidental and not causal.

Ictal phenomenon. Focal seizures with characteristic frontoparietal opercular features or nocturnal bilateral tonic-clonic seizures are mandatory for diagnosis.

The characteristic semiology of the focal seizures includes the following (203):

(1) somatosensory symptoms, with unilateral numbness or paresthesia of the tongue, lips, gums, and inner cheek.

(2) orofacial motor signs, specifically tonic or clonic contraction of one side of the face, mouth, and tongue, then involving one side of the face. In nocturnal seizures, the initial focal component may often not be witnessed.

(3) speech arrest; children have difficulty or are unable to speak (dysarthria or anarthria) but can understand language.

(4) sialorrhea, a characteristic ictal symptom. It is unclear whether this is due to increased salivation, swallowing disturbance, or both.

(5) In seizures associated with SeLECTS, cognitive (eg, gustatory hallucinations), emotional (eg, fear), and autonomic features are not seen.

The initial event is often a nocturnal hemifacial convulsion, which may rapidly evolve to tonic-clonic activity of the ipsilateral upper limb, to an ipsilateral hemiclonic seizure, or to a focal to bilateral tonic-clonic seizure. Seizures are typically brief, lasting for 1 to 3 minutes. More than half of the patients retain consciousness and may recollect the sensations. Seizures occur in sleep in the great majority (80% to 90%) of patients, and only while awake in up to 20% of children (40). Todd paresis may occur postictally. Miscellaneous symptoms, such as abdominal pain, may rarely occur (12). Among 230 children with SeLECTS, six presented with sensory motor seizures in the leg as the main ictal manifestation (71). Generalized tonic-clonic seizures during wakefulness are exclusionary and should prompt one to review the diagnosis.

This is associated independently with bilateral, repetitive, broad, centrotemporal interictal EEG spikes displaying a characteristic tangential bipolar pattern (84). The ictal manifestations are not indicative of temporal lobe involvement, and the term “centro-temporal” refers only to the spike topography.

Recurrence of seizures. Onset before 3 years of age has been stated as the single most important predictor for multiple seizures (118; 249). Neither clinical features, seizure characteristics, nor routine EEG findings were found to be useful in predicting the likelihood of a second seizure in SeLECTS (230). However, in a longer follow-up study of 52 children, the presence of a frontal focus and bilateral asynchrony was found to be correlated with the recurrence of seizures (211). The occurrence of atypical absence seizures, focal atonic seizures, and focal motor seizures with negative myoclonus with loss of balance and falls should suggest evolution to EE-SWAS, and evidence of cognitive impairment or regression should be sought.

Associated problems

Most children with SeLECTS have excellent seizure and neurodevelopmental outcomes. Nevertheless, numerous case series report associated developmental, cognitive, and behavioral morbidities. Many studies include small samples, often enriched for atypical cases and lacking detailed premorbid characterization. A critical appraisal of individual studies is beyond the scope of this article, and readers are referred to original publications for assessment of clinical validity.

Behavioral and psychiatric problems. Behavioral disorders may be present in approximately one third of patients with SeLECTS, including ADHD and oppositional defiant disorder (169). Early onset of seizures and the presence of bilateral interictal epileptiform discharges may suggest an increased risk for behavioral disorders in these children.

Cognitive and academic difficulties. Cognitive difficulties and low academic achievement are frequently reported (253). In a study of 40 children with centrotemporal spikes (with and without seizures), patients showed impairments in IQ, visual perception, short-term memory, and psychiatric status compared with controls; IQ deficits correlated more strongly with spike frequency than seizure frequency (231). In a cohort of 50 children, educational problems were identified in 54%, developmental learning disability in 38%, expressive language impairment in 18%, and attention disorders in 18%. Educational difficulties correlated with absence of a frontocentral dipole (p < 0.001), whereas language abnormalities correlated with atypical seizure semiology (p = 0.02) (225).

Additional studies demonstrated reduced academic performance, lower WISC digit span and similarities scores, and impaired auditory processing, likely reflecting executive dysfunction (156). Impaired social cognition, particularly theory-of-mind performance, was observed in 15 children (79).

Children and adolescents with SeLECTS had worse performance on social cognition compared to healthy children as evaluated by Faux-Pas Child Task, which evaluates the recognition and comprehension of other people’s mental state (136). Patients with SeLECTS also demonstrate processing speed dysfunction (133).

A large retrospective cohort study in Wales found that children with SeLECTS generally achieved similar educational attainment to peers without epilepsy in early school years but showed a trend toward slightly reduced attainment by age 16, whereas children with other epilepsy syndromes consistently had poorer outcomes across all ages (121). In a cohort of approximately 200 children with SeLECTS, academic and language outcomes assessed using standardized neuropsychological and educational measures showed a higher prevalence of reading and spelling difficulties than in controls, indicating domain-specific learning vulnerabilities that may serve as clinically relevant functional biomarkers in this syndrome (49).

Language and literacy impairments. A meta-analysis of 22 studies demonstrated reading and phonological processing deficits, supporting the need for early literacy and language assessment (198). Atypical seizure evolution and longer epilepsy duration may adversely affect language. A controlled study of 31 patients demonstrated significantly increased dyslexia rates (164). In an observational study of 108 probands and 159 siblings, reading disorder occurred in 42% of probands and 22% of siblings; seizure and treatment variables were not significant risk factors (223).

A higher spike-wave index during NREM sleep correlated with poorer nonverbal declarative memory consolidation, supporting disruption of sleep-dependent memory processes by interictal epileptic activity (72). A systematic review (2005 to 2016) found receptive and expressive language deficits across semantics, morphosyntax, imitation, verbal fluency, and verbal memory (212). Another systematic review of 43 studies showed impairments in inhibitory control, cognitive flexibility, and verbal fluency, although the quality of evidence was rated very low (178).

A detailed study of new word learning showed impaired phonological encoding and delayed recall despite preserved retention once learning was achieved, implicating deficits in phonological working memory and interference sensitivity within a fronto-temporo-parietal network (150). Neuropsychological and electrophysiologic studies further demonstrate auditory processing vulnerabilities, including central auditory processing disorder, phonological deficits, and altered auditory evoked potentials (N1, N2, MMN), which overlap with developmental dyslexia and correlate with seizure timing and neuropsychiatric features (215; 216).

Neuropsychological and electrophysiologic studies highlight language and auditory processing vulnerabilities in SeLECTS, including central auditory processing disorder, phonological deficits, and altered auditory evoked potentials (N1, N2, MMN). These abnormalities overlap with those seen in developmental dyslexia and correlate with seizure timing and neuropsychiatric features, supporting the view that language impairment may precede or evolve independently of seizures (215, 216).

Auditory, motor, and network-level abnormalities. Auditory spectral resolution, essential for speech perception in noise, is reduced in children with SeLECTS (205). Central auditory processing disorder has been reported in 46% of affected children without intellectual disability, dyslexia, or ADHD (149).

Functional connectivity studies reveal abnormal networks related to speech production involving the precuneus, cerebellum, thalamus, superior temporal gyrus, parietal regions, and frontal cortex (239). Oral dyspraxia, particularly affecting simple and sequential movements, was demonstrated in 74 children and attributed to genetically determined cortical immaturity affecting motor planning (13).

Children with active SeLECTS show impaired verbal working memory performance and altered functional network integrity on fMRI (32). Emotion discrimination deficits, particularly for sadness, fear, and disgust, were observed in newly diagnosed children and were more pronounced with earlier seizure onset but normalized after seizure remission (238). Persistent visual memory impairment has also been reported in children with long-term seizure control and normal-range IQ (70).

Atypical features and atypical evolution. Atypical features have been reported in up to 50% of cases (234; 43), including very early (younger than 3 years) or late (older than 14 years) onset, daytime-only seizures, daily seizures, focal or generalized status epilepticus, intellectual disability, poor neuropsychological outcomes, focal neurologic deficits, structural MRI lesions, lack of sleep activation, persistent unilateral EEG abnormalities, unusual spike morphology, diffuse slowing, and absence-like spike-wave discharges.

Early EEG features such as slow spike-wave focus, synchronous foci, or generalized 3-Hz spike-wave discharges predict lower full-scale and verbal IQ and impaired performance-scale tasks (151). In a multicenter study of 46 patients with atypical features, predominant manifestations included affective seizures (28.2%), opercular and oropharyngolaryngeal seizures with speech arrest and sialorrhea, opercular status epilepticus (15.3%), Todd paralysis (15.3%), negative myoclonus (13%), and rare focal tonic-dystonic seizures (73).

Atypical evolution accounts for approximately 5% of cases in tertiary centers. Predictors include early onset, neurocognitive regression with continuous spike-wave during sleep (suggestive of EE-SWAS), emergence of atypical absences or negative myoclonus, increased fronto-centro-temporal EEG activity, and more than five ripples on centrotemporal spikes (222; 174). Atypical SeLECTS is associated with more widespread fronto-temporo-parietal hypometabolism (130). Proposed risk factors include frontotemporal or temporoparietal epileptic foci, dysarthric seizure semiology, and early somatosensory auras (177).

Prognosis and complications

In general, SeLECTS is associated with an excellent prognosis.

Seizures. The majority of children have infrequent seizures, which are well controlled with the initial antiseizure medication. The prognosis is favorable even for those whose seizures are difficult to control, and seizures almost always remit spontaneously in late adolescence.

Cognitive functions. Most of the children have normal cognitive functions during the period of active epilepsy and after remission (95). Microstructural changes in cortex and white matter were described in some previous studies, the clinical significance of which is not clear (112; 75). A scoping review highlights that children with SeLECTS commonly exhibit deficits in executive functions—particularly inhibition, cognitive flexibility, and working memory—and that these impairments correlate with earlier age at onset, higher EEG abnormality burden, and psychiatric comorbidities such as ADHD, underscoring the need for routine neuropsychological assessment in clinical practice (67). In a cohort of children with Rolandic epilepsy and age-matched controls, overnight high-density EEG was used to quantify sleep spindle rate, and higher spindle rates were significantly associated with better overnight memory consolidation, suggesting that sleep spindle dynamics may serve as a functional biomarker of cognitive processing and memory integrity in this population (119).

Language impairment. Moderate to severe language impairment has been reported in a subset of children with SeLECTS, most prominently affecting expressive grammar, phonologic awareness, and literacy skills (161). Persistence of language deficits after seizure remission suggests potential long-term consequences (157). Abnormal neuropsychological development has been shown to correlate with higher frequency of NREM sleep discharges, epilepsy onset at school age, and exposure to a greater number of antiseizure medications (66).

Auditory processing and seizure timing. A study of 56 Turkish children with SeLECTS (aged 6 to 13 years) compared with 32 healthy controls examined the relationship between language impairment, central auditory processing, seizure timing, and neuropsychiatric function using standardized assessments (215). Inclusion criteria required typical SeLECTS per ILAE definition, EEG confirmation, normal MRI, seizure freedom for at least 1 year, and an IQ greater than 70. Patients were stratified by seizure timing: seizures shortly after sleep onset versus seizures just before awakening. Central auditory processing difficulties were highly prevalent irrespective of seizure timing. However, children with post-sleep seizures showed greater expressive language impairment, as well as increased difficulties in prosocial behavior and impulsivity.

Attention-deficit hyperactivity disorder (ADHD). The prevalence of ADHD may be up to 65%, and patients perform poorer on executive and attentional tasks (135; 42). This has been related to thinner superior-inferior frontal cortex, superior temporal cortex, left pericalcarine, and lingual and fusiform cortex compared to healthy controls (109). A study of 42 patients with newly diagnosed SeLECTS evaluated the long-term prognosis of attention deficit in children (over a period of 7 years) and showed an impaired attention network, mainly in the alerting and orienting domains (237).

Sleep problems. Children with SeLECTS may face a higher risk of experiencing sleep disorders (delayed sleep onset, sleep duration, night awakenings, parasomnias, daytime sleepiness, and sleep anxiety) relative to age-matched healthy children and correlate positively with impairment of attentional network function on standardized scales (106). This may have implications for interventions addressing sleep disorders to improve prognosis and quality of life in children with SeLECTS.

Atypical evolutions. Most of the children with atypical evolutions will also have a good long-term prognosis (Aicardi and Chevrie 1982; 64; 62; 20). However, Landau-Kleffner syndrome and EE-SWAS may portend permanent language dysfunction or neuropsychologic involvement (60; 62; 63). On EEG, interictal rolandic double/multiple spikes and slow-wave abnormalities may indicate a higher chance for SeLECTS with electrical status epilepticus in sleep and guide early intervention (145; 102). Using retrospective EEG review and multivariable regression modeling in children with SeLECTS, prolonged spike-and-wave clusters, high-amplitude spikes with secondary generalization, and younger seizure onset have been proposed as predictors of progression to EE-SWAS (96). Quantitative EEG-based prediction models show promise in identifying children with SeLECTS at risk of progression to EE-SWAS (92). Early alterations in spectral power, connectivity, and EEG complexity may enable preclinical identification of high-risk patients, supporting earlier intervention strategies (92).

Antiseizure medication therapy. Antiseizure medication therapy does not necessarily improve and may actually decrease health-related quality of life in SeLECTS children with low seizure frequency (04). In a multicentric study, the authors compared biochemical markers of bone metabolism in 1032 children with SeLECTS (divided into an untreated group [117] and monotherapy group [643]) with 272 healthy controls to identify the specific effects of epilepsy and antiseizure medications on bone metabolism (194). Bone metabolism was significantly lower in the untreated group than in the healthy control group (p < 0.05). When comparing the monotherapy and untreated groups, oxcarbazepine, levetiracetam, and topiramate did not affect bone metabolism, and lamotrigine corrected some of the abnormal markers of bone metabolism in patients with epilepsy.

Transition. SeLECTS almost always enters terminal remission before the general age of a planned transition of adolescents (21).

Biological basis

Etiology and pathogenesis

Genetic architecture and inheritance. Early studies suggested that the characteristic centrotemporal EEG pattern in SeLECTS followed an autosomal dominant inheritance with variable penetrance, based largely on observations of monozygotic twins (93; 54; 10). Subsequent work supported a multifactorial model for epilepsies with focal epileptiform sharp waves (50). In a multicenter twin collaboration study of 18 twin pairs, no concordant twin pairs with classic SeLECTS were identified, indicating that noninherited factors play a major etiologic role (220). Atypical evolution of SeLECTS appears to involve more complex inheritance patterns rather than a simple monogenic mechanism (218).

Candidate genes and chromosomal abnormalities. SeLECTS has been associated with multiple chromosomal loci, including 15q14, terminal deletions of 1q, 11p13, deletions in 16p13, and polymorphisms involving ELP4 and BDNF, as well as pathogenic variants in GRIN2A, PRRT2, KCNQ2/3, DEPDC5, NR4A2, CHRNA4, ZMYND11, RBFOX1/3, and GABRG2 (125; 48; 83; 182; 52; 179; 159; 53; 97; 162). GRIN2A has emerged as a major gene in epileptic encephalopathies and is particularly implicated in severe SeLECTS phenotypes.

Genome-wide studies have identified rare deletions across chromosomes 1 to 20, overlapping with autism- and epilepsy-associated candidate genes (104), as well as multiple single-nucleotide polymorphisms on chromosomes 3, 10, and 15 near or within KALRN, PTCHD3/RAB18, and CHRNB4, respectively (195). Environmental risk factors, including maternal smoking around birth, have also been implicated (195). A trio-based whole-exome sequencing study in 28 Chinese patients with typical and atypical SeLECTS identified variants in ADGRV1, GRIN2B, and RyR2, suggesting a role for calcium homeostasis in disease mechanisms (141).

Perisylvian network dysfunction and epileptic spectrum. The focal semiology and centrotemporal EEG discharges in SeLECTS point to dysfunction in the Sylvian and Rolandic regions. SeLECTS, Landau-Kleffner syndrome, and continuous spike-and-waves during slow sleep are often conceptualized as a spectrum of age-dependent, nonlesional, genetically influenced epilepsies involving a perisylvian epileptic network, in which epileptiform activity interferes with cognitive development (91; 88).

Distributed network involvement beyond the rolandic cortex. Electrophysiologic studies do not identify a single discrete epileptogenic generator, suggesting instead a large, shifting zone of dysfunction that may include deep brain structures such as the cingulate gyrus, basal ganglia, and thalamus, supported by hemodynamic responses associated with centrotemporal spikes (103). The occurrence of generalized spike-wave discharges and multifocal spikes in some patients suggests overlap with idiopathic generalized and other focal epilepsies (127). Approximately 20% of patients exhibit sharp slow-wave complexes outside the centrotemporal region (172).

MEG studies comparing drug-naïve children with SeLECTS, childhood absence epilepsy, and controls demonstrate shared low-frequency slowing but distinct network signatures in SeLECTS, characterized by focal abnormalities, widespread low-to-mid-frequency hyperconnectivity, and reduced high-frequency power, particularly in temporal regions (228). Functional neuroimaging further demonstrates altered dynamic connectivity within thalamocortical, attentional, and visual networks, indicating system-level reorganization beyond a focal epileptogenic zone (59; 27).

Thalamocortical circuitry and sleep-related mechanisms. Because SeLECTS emerges during a period of active white matter maturation and is markedly activated during non-REM sleep, aberrant thalamocortical circuitry has been strongly implicated (123). Epileptiform spikes may be facilitated by reduced NMDA or h-type currents, with altered inhibitory transmission in the thalamic reticular nucleus mediating antagonistic interactions between spikes and sleep spindles (128). Median nerve somatosensory evoked field conduction times have been proposed as adjunctive markers of residual thalamocortical dysfunction in resolved cases (31).

Children with atypical evolution may demonstrate symmetric thalamic hypoperfusion on interictal SPECT without MRI abnormalities, correlating with increased cognitive risk (06). Sleep EEG studies show significantly reduced spindle amplitude, duration, and density in SeLECTS, with epilepsy risk increasing by 1.9% for each 1 mV decrease in spindle amplitude (186). Given that spindles are generated in the thalamus and propagated via thalamocortical circuits, regional spindle deficits may predict cognitive outcome (204). Tractography studies demonstrate aberrant thalamocortical connectivity to the rolandic cortex, absence of expected age-related strengthening, and persistence of abnormalities longitudinally (214).

Arterial spin labeling MRI has shown increased thalamic blood flow in SeLECTS, with perfusion patterns varying by spike lateralization and antiseizure medication exposure, suggesting intrinsic thalamic involvement and medication-specific neurobiological effects (99).

Neurodevelopmental delay and brain maturation. Multiple neuroimaging studies support a neurodevelopmental origin of SeLECTS, with widespread abnormalities reflecting delayed maturation rather than seizure-induced injury (199). Structural MRI studies demonstrate altered gray and white matter volumes in rolandic regions and a mean delay of approximately 0.45 years in brain-predicted age (255; 185). Quantitative EEG studies show delayed cortical maturation in centrotemporal regions even after seizure remission (197).

Surface-based morphometry reveals aberrant cortical thickness, gyrification, and sulcal depth in regions associated with language, attention, and memory, with negative correlations between age at seizure onset and cortical metrics, and between verbal IQ and gyrification (134). Longitudinal educational data indicate that reading difficulties may precede seizure onset, suggesting preexisting neurodevelopmental vulnerability (250). Normative morphometric modeling further demonstrates individualized cortical and subcortical deviations in language-, attention-, and sensorimotor-related regions, even in children with normal conventional MRI (245). Connectome-based predictive modeling using machine-learning approaches is being investigated to characterize brain-behavior relationships in SeLECTS (226).

Glymphatic system dysfunction. Emerging evidence implicates glymphatic dysfunction in SeLECTS pathophysiology. Increased Virchow-Robin space counts and volumes have been observed and correlate with reductions in full-scale, verbal, and performance IQ, even in children with normal conventional MRI (74). Diffusion MRI modeling demonstrates impaired perivascular water diffusion associated with epileptiform burden and disease duration, supporting glymphatic measures as candidate biomarkers of network dysfunction (247). Although Virchow-Robin space measures decline over time and correlate with seizure duration, longitudinal studies are needed to clarify causality and prognostic significance.

Immune-mediated mechanisms. Immune dysregulation has been proposed as a contributor to SeLECTS and related phenotypes. Cerebrospinal fluid studies in children with electrical status epilepticus in sleep demonstrate elevated proinflammatory cytokines, including IL-6 and TNF-alpha, suggesting a role for neuroinflammation in disease expression and cognitive sequelae (246). A prospective cohort study identified an autoimmune-associated SeLECTS subgroup with distinct clinical features and favorable seizure and EEG responses to immunotherapy, indicating potentially treatable immune-mediated mechanisms in a subset of patients (257).

Epidemiology

SeLECTS accounts for about 24% of all epileptic seizures in children between 5 and 14 years of age (25). Its annual incidence has been reported to be between 7.1 and 21 per 100,000 in children under 15 years of age, with a male preponderance (93). In an epidemiological study of epilepsy in childhood in a cohort of 440 consecutive patients, SeLECTS accounted for 8% of patients (117). In an epidemiological study of 205 cases of childhood epilepsy in a Swedish county, SeLECTS was the most common epilepsy syndrome (122). Because nocturnal seizures can be easily missed in diagnosis, this disorder may be even more common than generally suspected. There is a slight male predominance. The prevalence of epilepsy is much higher among close relatives of children with SeLECTS than in a matched control group (19). In a study, 15% of siblings had seizures and centrotemporal spikes, 19% of siblings had centrotemporal spikes without clinical events, and 11% of the parents had childhood seizures that had disappeared by adulthood (93). A retrospective cohort study of 379 children aged 0 to 16 years born between 1994 and 2012 in the UK and followed from birth until September 2017 reported that the contemporary UK incidence of this epilepsy is 5/100,000/year and has remained virtually unchanged between 1997 and 2014; males and children aged 6 to 11 years have the highest incidence. Comorbidities, particularly pervasive developmental disorders, were noted in 12% of children (206).

A population study from the UK identified SeLECTS in 3% of 6732 children (aged 0 to 16 years) with epilepsy based on their primary care records between 2004 and 2017 (120). The authors concluded that there was no change in the incidence of SeLECTS over time, whilst the incidence of childhood epilepsy overall was decreasing. The prevalence of epilepsy and SeLECTS was 0.55% and 0.02%, respectively, in the year 2017, with a corresponding crude incidence of 51.2 in 100,000 per year and 1.1 in 100,000 per year, probably due to under-recording of SeLECTS.

Differential diagnosis

Confusing conditions

The presence of the characteristic centrotemporal spikes alone is not diagnostic of SeLECTS. These may be seen in children without seizures as well as in nonepileptic children with diffuse brain disturbances, such as cerebral palsy (176) and Rett syndrome (183).

SeLECTS is the most common epilepsy syndrome, seen in patients with fragile X syndrome, and centrotemporal spikes may be present as an asymptomatic finding (111).

DEE-SWAS or EE-SWAS. Patients with DEE-SWAS may present with similar seizures but can be distinguished by cognitive and language regression. Children with SeLECTS may rarely evolve to this syndrome.

Focal seizures due to a structural brain abnormality. Distinction between SeLECTS and structural focal epilepsies, such as mesial temporal lobe epilepsy, can usually be made easily on the basis of history and the unique dipole pattern of the centrotemporal spike. The EEG alone is sufficient to make the diagnosis of SeLECTS when nocturnal convulsions are the presenting complaint, and a generalized epileptic syndrome is initially suspected.

Other self-limited focal epilepsies. The morphology of the EEG abnormalities in the various self-limited focal epilepsies may overlap, and their seizure localization may change with age. If patients present with prolonged focal nonmotor seizures with prominent autonomic features, especially ictal vomiting, self-limited epilepsies with autonomic seizures should be considered.

Associated or underlying disorders

Fortuitous associations may be found between SeLECTS and static brain lesions with epilepsy (187). Reported examples are unilateral opercular neuronal migration disorders (03; 60; 192), cerebral tumors (193), neuronal migration disorders and gliosis (167), and hippocampal sclerosis (170).

Pathophysiologic relationships may exist between SeLECTS and SeLFEs, which can make differential diagnosis difficult. The coexistence of two types of SeLFEs in children has been reported, either presenting in sequence one after the other or at the same time (171; 23; 36; 24; 26). The coexistence of childhood absence epilepsy and SeLECTS was detected in 11 patients through a systematic record review from eight epilepsy centers (224). A comparison of 17 patients with absence epilepsy and centrotemporal spikes with age-matched 90 children with absence epilepsy showed significantly more global developmental (five [29%] vs. five [6%], P < .009) and expressive language (four [24%] vs. five [6%], P < .034) delay, and more difficulties with school performance (11 [65%] vs. 32 [36%], P < .025), especially with language-related tasks (six [35%] vs. five [6%], P < .001) in children with absence epilepsy and centrotemporal spikes (44).

An interesting association with migraine has been proposed in patients with SeLECTS (16). In a comparative cohort of children with SeLECTS, with cryptogenic/symptomatic partial epilepsy and with no history of seizures (n = 53 each), a higher rate of migraine was identified in focal epilepsy, regardless of the etiology (236). Another study of 72 children with SeLECTS and their siblings showed that the prevalence of migraine was 15% in epilepsy probands versus 7% in nonepileptic controls (33). The prevalence of migraine was 14% in siblings of SeLECTS and 4% in siblings of controls.

Diagnostic workup

EEG

Characteristics of EEG spikes in SeLECTS
	• Background activity is typically normal, with normal sleep architecture. If sustained focal slowing without centrotemporal spikes or diffuse slowing is recorded, another epilepsy syndrome or a structural lesion should be considered, and brain imaging is recommended. A change in morphology, particularly the appearance of focal fast spikes or polyspikes, or a marked increase in the slow component, or a brief depression of voltage, may suggest the possibility of a focal lesion (41).
	• High-amplitude (greater than 200 μV, peak to trough) centrotemporal sharp-and-slow-wave complexes that activate in drowsiness and sleep are mandatory for diagnosis (94). They are triphasic, high-voltage (100 to 300 µV) sharp waves (initial low-amplitude positivity, then high-amplitude negativity, followed again by low-amplitude positivity), with a transverse dipole (frontal positivity, temporoparietal negativity), often followed by a high-voltage slow wave.
	• The abnormalities 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 (155).
	• Spikes may be unilateral or bilateral and independent or synchronous and may be seen outside the centrotemporal region (midline, parietal, frontal, occipital). Generalized 3 Hz spike-and-waves and focal spikes in other brain areas may also be seen in a minority of children (15).
	• The centrotemporal spikes are not enhanced by eye opening or closure, by hyperventilation, or by photic stimulation. Hyperventilation may even reduce the frequency of rolandic spikes (229). They may be activated by sensory stimulation of the fingers or toes in 10% to 20% cases (45).
	• Ictal recordings are rare and may be accompanied by a brief decrease in amplitude of the background EEG, followed by diffuse sharp wave abnormalities of increasing amplitude, predominantly in one centrotemporal region, followed by high-amplitude slowing and then a return to the usual interictal EEG (190).
	• With focal to bilateral tonic-clonic seizures, ictal rhythms may become bilaterally synchronous (as opposed to generalized) sharp wave or spike-and-wave activity (02).

There is no correlation between the burden of spike discharges on EEG and frequency, length, or duration of clinical seizures (126). In fact, extreme discrepancies between the rarity of seizures and the activity of the EEG foci are not uncommon, and clinical experience indicates that the EEG is often relatively unchanged, even with effective treatment (08). Neuropsychological examination of children aged between 4 and 10 years with newly diagnosed active typical SeLECTS (n=12) or typical rolandic discharges in waking and sleep EEG recordings (n=26), prior to antiseizure medication initiation and with normal MRI and normal psychomotor and psycholinguistic development before diagnosis, showed higher cognitive and working memory impairment compared to healthy controls (160). A small case-control study of 52 children showed that 14 Hz and 6 Hz positive spikes were present in more than 80% of children with SeLECTS but absent in controls, suggesting these EEG variants are highly prevalent in this syndrome and may reflect developmental maturation effects in thalamocortical circuits and heightened cortical excitability, offering insight into underlying network dysregulation beyond classical centrotemporal spikes (180).

Dipolar pattern in SeLECTS. Several authors emphasized the characteristic dipolar pattern in EEG (84; 137; 219). According to EEG findings, two groups of patients have been noted: (1) a high-central region group with more frequent hand involvement and (2) a low-central group with common orofacial symptoms. Electroencephalographic spike source dipoles of centrotemporal spikes were analyzed in 37 patients (113). Differences in spike-source dipole were found in patients showing atypical outcomes.

Newer techniques for EEG analysis. An algorithm for automatic classification of centrotemporal spikes in the interictal EEG according to the epilepsy type has been proposed and may distinguish benign from symptomatic causes (153). A novel machine-learning model that efficiently distinguished rolandic seizures from normal EEG signals has been proposed, with an accuracy exceeding 97.6% (148). This proposed machine-learning model processes the identification procedure in the following order: (1) creating preliminary EEG features using signal empirical mode decomposition, (2) applying a weighted overlook graph, and (3) classifying the results through a 2-dimensional convolutional neural network. A novel SeLECTS spike detection algorithm based on time domain EEG sequence features and the long short-term memory neural network has been proposed to aid in the automated detection of rolandic spikes, with a sensitivity of 92.04% and precision of 85.75% (243).

Spike ripples have been identified as a promising new biomarker of epilepsy in noninvasive EEG studies (68). Ripples on centrotemporal spikes are considered to indicate epilepsy severity. The absence of ripples predicts a relatively benign clinical entity (222). A study proposed that spike ripples (ripples cooccurring with epileptiform discharges) have a comparable sensitivity and negative predictive value, but greater specificity and positive predictive value, as compared to spikes alone in predicting seizure risk in children with SeLECTS (116). A comparative study of seven patients with atypical SeLECTS and 18 patients with typical SeLECTS in the secondary bilateral synchrony and non-secondary bilateral synchrony periods found that ripples were enhanced when interictal epileptiform discharges were bilaterally synchronized in patients with atypical SeLECTS (100). The study highlighted the distinction between atypical and typical SeLECTS with ripple distribution in the non-secondary bilateral synchrony period.

Intrahemispheric cortico-cortical EEG functional connectivity (EEGfC) was explored in 17 nonmedicated children with SeLECTS and 19 controls, and the areas of increased EEGfC corresponded to cortical areas related to speech and attention deficits (34).

A nonlinear analysis, including measures such as multiscale entropy and recurrence quantitative analysis, of visually normal EEGs has been used to differentiate between patients with and without SeLECTS (188). Similarly, partial directed coherence, classical machine learning, and deep learning based on EEG data are being used to assist in identifying relevant biological features of SeLECTS within EEG data and to explore brain connectivity (30; 140).

EEG studies demonstrate that SeLECTS is characterized by abnormal electrophysiologic signatures beyond centrotemporal spikes, including increased wakeful EEG power and functional connectivity, altered quantitative EEG metrics, and interictal high-frequency oscillations (HFOs). Notably, scalp high-frequency oscillation burden during non-REM sleep has been associated with ADHD comorbidity, whereas quantitative EEG features can predict evolution to EE-SWAS, supporting EEG-derived markers as potential risk-stratification and prognostic biomarkers in SeLECTS (76; 92; 110).

Spike clearance velocity. Spike clearance velocity (defined as a decrease in the spike-wave rate over time in 4-year sequential follow-up EEGs) has also been suggested as an EEG marker to guide antiseizure medications in children with SeLECTS (213). The study cohort consisted of 127 children with SeLECTS divided into three groups based on antiseizure medication responsiveness: group I was seizure-free with monotherapy (n: 61, 48%); group II was seizure-controlled with monotherapy (n: 52, 41%); and group III was seizure-controlled with dual therapy (n: 14, 11%). The authors found no statistical significance across the study groups with respect to initial EEG spike characteristics (spike-wave rate, spike localization, and spike topography). However, the spike clearance velocity was significantly slower in group III than in group I in 4-year sequential follow-up EEGs. Further, the spike clearance velocity was not different across the antiseizure medication groups (oxcarbazepine, valproic acid, and levetiracetam).

Magnetoencephalographic analysis. MEG-based studies in patients with centrotemporal spikes in SeLECTS suggest that these discharges are generated through a mechanism similar to that of somatosensory evoked responses (152). An MEG-based study of 33 children with SeLECTS and 18 healthy children showed magnetic source inactivation in the medial frontal cortex and posterior cingulate cortex regions during the interictal period, possibly accounting for the cognitive decline in early untreated children with SeLECTS (131). Magnetic source localization in the 4 to 8 Hz frequency band may be a new imaging marker for the diagnosis of new SeLECTS and cognitive impairment by identifying disordered networks (129). A combination of band-dependent MEG alterations and clinical features has been used in SeLECTS patients who evolve into atypical SeLECTS by using a triple-network model and then constructing a prediction model with high predictive efficiency, an accuracy of 0.80, and an AUC of 0.84 (132). MEG and resting-state fMRI studies reveal that children with SeLECTS exhibit widespread alterations in functional brain networks, including disrupted neuromagnetic connectivity, altered functional gradients, and abnormal dynamic functional connectivity. These network changes correlate with cognitive impairment, while compensatory reorganization is also observed, suggesting that SeLECTS involves a balance between network disruption and adaptive plasticity rather than a purely focal disorder (146; 201; 202).

Brain MRI

Structural abnormality. Typically, MRI is normal and is not required for the electroclinical diagnosis of the syndrome. Nonspecific MRI findings, such as hippocampal asymmetry, white matter abnormalities, and enlargement of the lateral ventricles, should not exclude a diagnosis of SeLECTS. Focal cortical dysplasia and tumors have presented with the clinical and EEG phenotype of SeLECTS (03; 60; 193; 192). Patients with focal epilepsy due to structural abnormalities, such as focal cortical dysplasia, heterotopia, or low-grade brain tumors, may mimic SeLECTS but usually show atypical features, such as unilateral EEG abnormality or drug resistance (203). In 171 consecutive patients with this syndrome studied with CT or MRI, 10 children were noted to have neuroimaging abnormalities (78).

Volumetric changes. Frontal and prefrontal volumes revealed growth disturbances in patients with cognitive impairments (227). Thickness of the corpus callosum has been suggested as a new radiologic biomarker for predicting first antiseizure medication response and seizure recurrence in SeLECTS patients (46). Thicknesses of genu and isthmus were significantly reduced in the SeLECTS group compared to healthy controls, and the total number of seizures negatively correlated with the thickness of the body, isthmus, and splenium (p < 0.001), thus working as a protective mechanism to prevent seizure spread to other brain regions. In multimodal neuroimaging studies of children with SeLECTS compared to age-matched controls, combined structural MRI, voxel-based morphometry, and diffusion tensor imaging revealed reduced gray matter volume in the bilateral frontal and temporal lobes and altered white matter microstructure in language and motor pathways, with these changes correlating with language performance deficits, suggesting that neuroanatomical alterations may underlie specific cognitive impairments in SeLECTS and hold promise as imaging biomarkers of functional outcome (201; 202).

Positron emission tomography

Positron emission tomography is not routinely performed in these children. It may be helpful to distinguish SeLECTS from symptomatic cases of focal epilepsy in children (221). Focal cortical hypermetabolism in the central cortex was noted in three children with atypical SeLECTS during the seizure-free period (37), suggesting a localized, increased cortical activity, likely due to either subclinical seizure activity or “active” inhibitory (GABAergic) processes. An interictal ¹⁸F-FDG positron emission tomography study with voxel-based and region-of-interest analyses demonstrated reduced glucose metabolism and structural volume in the bilateral putamen and pallidum in children with SeLECTS, and these lenticular nucleus abnormalities correlated significantly with cognitive impairment, particularly in atypical phenotypes (251).

Functional MRI (fMRI)

Several fMRI studies have been reported in SeLECTS. It is increasingly being used to study the dynamic alternations in networks in SeLECTS (254). Aberrant functional connectivity between motor and language networks, abnormal intrahemispheric segregation, and interhemispheric integration have been seen in these children (14; 248). Another fMRI study of 26 children with SeLECTS showed aberrant dynamic regional interplay in sensorimotor, linguistic, and lateral temporal regions, which may affect cognitive performance in these children (105). Resting state fMRI studies in a smaller number of patients with SeLECTS (n=12) show normal cortical-subcortical functional connectivity with compensatory hyperconnectivity between cortical networks caused by widespread cortical abnormal discharges and might account for the self-limited clinical outcome in SeLECTS (69). A “granger causality density” method has been proposed as an effective and reliable neuroimaging biomarker to localize the interictal focus of SeLECTS and make an early diagnosis (39). Resting-state fMRI is being used to assess local brain activity by studying the static and dynamic changes in the amplitude of low-frequency fluctuations in patients with SeLECTS (58). The influence of wakefulness fluctuations on brain networks involved in centrotemporal spike occurrence and their connectivity to the key regions of the language circuitry is also being explored by fMRI studies (209). This may have implications in understanding the complexities of epilepsy syndromes. Using resting-state functional MRI and dynamic functional network connectivity analysis, it has been shown that children with SeLECTS spent more time in a highly integrated brain connectivity state with greater variability over time compared to childhood absence epilepsy, suggesting distinct dynamic network dysfunction patterns in SeLECTS that may reflect differences in underlying neurobiology and functional network reorganization between childhood epilepsy subtypes (200).

Transcranial magnetic stimulation

A pilot study proposed transcranial magnetic stimulation with EMG and EEG to assess cortical excitability and motor cortex plasticity in children with SeLECTS and their relationship to learning ability (11). Resting-state functional MRI has shown that centrotemporal spikes are associated with early hemodynamic activation in epileptogenic regions, an effect counteracted by levetiracetam treatment (242). A small open pilot study of four children with frequent seizures reported that low-frequency repetitive transcranial magnetic stimulation reduced seizure frequency after magnetoencephalography-guided targeting of epileptiform sources (107).

Subsequent studies suggest that low-frequency rTMS may exert broader neuromodulatory effects in SeLECTS, including reduction of interictal epileptiform activity and spike-wave index, modulation of abnormal functional connectivity, enhancement of sleep spindle activity, and associated cognitive improvement, supporting its potential role as an investigational therapeutic approach (90; 191).

Other investigational techniques

An excitatory and inhibitory imbalance in neural networks has been suggested as a useful diagnostic biomarker for SeLECTS using a resting-state dynamic causal modeling-based support vector machine (rs-DCM-SVM) algorithm, with an accuracy of 81% to 88% (38).

Although MRI is conventionally considered normal in classical presentations of SeLECTS, white matter abnormality is increasingly being reported by the combination of advanced MRI techniques. Comparison of white matter microstructural characteristics (axial, radial, and mean diffusivity and fractional anisotropy) between children with active SeLECTS and typical EEGs (n = 13), resolved SeLECTS (n = 12), and controls (n = 17), and their relationships with language scores on neuropsychological testing show impairments in regions constituting language networks in SeLECTS (166). Both diffusion tensor imaging and quantitative susceptibility mapping techniques revealed extensive white matter alterations in the frontal, parietal, and temporal lobes in 27 children with SeLECTS and typical EEGs as compared to 23 age- and gender-matched healthy controls and can be employed as a novel approach for characterizing these changes and looking at their associations with various clinical parameters (241).

Studies highlight that elevated IL-6, TNF-alpha, and neuron-specific enolase levels, along with EEG spike characteristics and younger age of onset, are significantly associated with cognitive impairment in children with SeLECTS, suggesting potential biomarkers for early identification and intervention (244).

In a multimodal MRI study of 20 drug-naïve children with SeLCTS and 33 controls, combined structural and resting-state functional analyses using parallel independent component analysis revealed co-altered gray matter volume and low-frequency neural activity in frontal, temporal, thalamic, and cerebellar regions, with these structure-function components significantly correlated with serotonergic, noradrenergic, and glutamatergic neurotransmitter systems, suggesting linked neurochemical and network alterations in BECTS (28).

Management

SeLECTS is an age-related epilepsy syndrome that almost invariably remits by adolescence, regardless of age at onset; therefore, excessive activity restriction and overprotection are not recommended. Antiseizure medication is generally reserved for children with very frequent seizures, focal motor seizures, a short interval between the first and second seizure, or an early age at onset. Monotherapy is preferred.

A Cochrane systematic review reported seizure-free rates of 64% with carbamazepine, 67% to 70% with clobazam, 67% to 89% with oxcarbazepine, 86% to 100% with levetiracetam, 69% with topiramate, and 68% to 90% with sulthiame (210). Later systematic reviews suggested sulthiame, levetiracetam, or clobazam as reasonable first-line options but emphasized heterogeneity in efficacy and tolerability and the need for larger randomized controlled trials (80). More randomized controlled trials comparing antiseizure medications with larger populations are required to determine the optimal antiepileptic drug treatment to guide clinicians.

Antiseizure medication treatment

Carbamazepine and valproic acid. Carbamazepine and valproic acid were traditionally first-line treatments. Carbamazepine achieved seizure freedom in up to 64% of patients during the first year, according to a Cochrane meta-analysis (210). In a comparative study of 89 patients, response rates were 11.2% with carbamazepine and 56% with valproic acid (108). In a trial of 56 patients, seizure-freedom rates were similar between valproic acid and levetiracetam, but EEG normalization was more frequent with valproic acid (95% vs. 72% at 18 months) (240). Use of valproic acid in adolescent females is limited by concerns regarding hormonal abnormalities, obesity, and polycystic ovarian syndrome.

Levetiracetam. Levetiracetam monotherapy has gained attention as an alternative first-line agent (47). It has shown greater suppression of spike-wave index than carbamazepine and oxcarbazepine, with equivalence to valproic acid (87). In a comparative study of 89 patients, levetiracetam achieved the highest response rate (71%) (108). A meta-analysis of 49 studies (2000 to 2018) suggested levetiracetam was superior to carbamazepine for cognitive protection (09). Functional imaging studies demonstrated modulation of centrotemporal spike-related activation and higher-cognitive network deactivation (256), with mild but significant improvements in multiple cognitive domains at 2-year follow-up (165). However, pooled analysis of 19 randomized studies (1224 patients) found no higher probability of seizure freedom with levetiracetam compared with carbamazepine (RR 1.25, 95% CI 0.52–2.99) or with sulthiame, oxcarbazepine, or valproic acid (29). Once-daily nocturnal dosing was noninferior to twice-daily dosing for seizure control and EEG normalization, with better adherence and behavioral tolerability (56).

Oxcarbazepine. Oxcarbazepine monotherapy has demonstrated seizure-free rates of 67% to 89% (210). Some studies report greater efficacy and cognitive improvement compared with levetiracetam (207). In a multicenter cohort of 1817 children, oxcarbazepine and levetiracetam were more effective than valproic acid (139), whereas other retrospective and meta-analytic studies reported similar seizure-freedom rates among oxcarbazepine, levetiracetam, and valproic acid (86; 29).

Benzodiazepines (clobazam, clonazepam). Short-term benzodiazepine therapy has been used in SeLECTS. Clonazepam was more effective than carbamazepine or valproate in suppressing rolandic discharges after 4 weeks (154). Nocturnal clobazam may be considered in children with seizures occurring exclusively during sleep (61). In a prospective controlled study, clobazam monotherapy was as effective as carbamazepine and better tolerated, although the quality of evidence was low (05).

Sulthiame. Sulthiame has shown high efficacy (68% to 90% seizure freedom) and good tolerability (210). In a double-blind, placebo-controlled trial of 66 children, sulthiame was highly effective in preventing seizures (181) and has been favored in atypical evolutions with secondary bilateral synchrony (62; 61). In a noninferiority study, seizure-free rates were high with both sulthiame and levetiracetam, but treatment discontinuation was more frequent with levetiracetam (17). Both drugs produced similar reductions in spike-wave index (208).

Topiramate and lamotrigine. Topiramate monotherapy (2 to 4 mg/kg/day) showed comparable efficacy and EEG effects with once-nightly and twice-daily dosing in 85 patients, with fewer adverse effects using nocturnal dosing (138). In a retrospective comparison, seizure recurrence rates were 19.4% with lamotrigine, 21.7% with topiramate, and 11.4% with oxcarbazepine; improvements in language and problem-solving were greater with lamotrigine and oxcarbazepine (89).

Lacosamide. Lacosamide monotherapy achieved seizure freedom in 39%, 67%, and 72% of children at 0 to 3, 4 to 6, and 7 to 12 months, respectively (163). In a larger cohort, total effectiveness was 94%, cumulative seizure control 85% at 18 months, retention 91%, and EEG spike-index response 73%, with no significant IQ change (65). Younger age at onset and higher baseline seizure frequency were associated with reduced EEG response.

Duration of therapy and relapse

Because seizures usually remit by adolescence, prolonged therapy beyond this period is of limited value. Seizure relapse occurs in 14% to 28% of cases after premature withdrawal or poor response (101; 175). Antiseizure medications control seizures but do not significantly shorten spike persistence or normalize interictal EEG (114). Younger age at onset predicts longer EEG abnormalities and poorer initial response (175; 124).

Nonpharmacological treatment

Auditory stimulation during non-REM sleep suppresses spike activity and has been proposed as a nonpharmacological treatment of SeLECTS (115). Further studies are needed before this technique can be implemented in clinical practice.

Because sleep and epilepsy have an established bidirectional relationship, especially in SeLECTS, a UK-based, multicenter, open-label, randomized trial is underway to assess the effectiveness of behavioral sleep intervention in children with SeLECTS (01). The Changing Agendas on Sleep, Treatment and Learning in Epilepsy (CASTLE) Sleep-E trial compares clinical and cost effectiveness between standard care and standard care augmented with a novel, tailored, parent-led CASTLE Online Sleep Intervention. The latter consists of a self-paced, e-learning module for parents that supports parents in implementing general prevention techniques (eg, good sleep hygiene) and specific behavioral change techniques (eg, bedtime fading) relevant to their child’s sleep problems.

Outcomes

Seizure outcome. Long-term seizure outcome in SeLECTS is excellent, with fewer than 2% of patients developing absence seizures or generalized tonic-clonic seizures in adulthood. Adults with resolved SeLECTS show no adverse outcomes in development, education, employment, or social adaptation (22), although small long-term studies have reported selective deficits in verbal perception and oromotor performance in young adults followed for 10 years (217).

Cognitive and behavioral outcome. During the active phase of epilepsy in childhood, outcomes are variable and frequently include cognitive and behavioral impairment. A systematic review and meta-analysis of 42 studies published before 2016 found significantly lower neuropsychological test scores across all cognitive domains in children with SeLECTS compared with healthy controls, although publication bias could not be excluded (233).

In a large prospective longitudinal cohort, ADHD symptoms (18.3%) and learning difficulties (21.7%) were present before diagnosis; after diagnosis, new or persistent ADHD (20%), mood disorders (23.6%), learning difficulties (14.5%), and behavioral disorders (7.3%) were common (184). At 9-year follow-up, formal neuropsychological performance was comparable to population norms and sibling controls (184). A systematic review of nine case-control and cohort studies reported ADHD prevalence of approximately 60% in SeLECTS (07). Predictors of ADHD included early seizure onset, longer disease duration, and lower intelligence scores (98), and comorbid ADHD was associated with greater impairment in selected intellectual domains (57). An eye-tracking study demonstrated prolonged antisaccade reaction times and impaired cognitive flexibility, particularly in children with earlier seizure onset, indicating domain-specific executive dysfunction (196).

Mortality. Sudden unexpected death in epilepsy is exceedingly rare in SeLECTS. Three children among 189 decedents in the North American Sudden Unexpected Death in Epilepsy Registry were reported to have SeLECTS. This observation requires further validation (51).

Quality of life. Reduced health-related quality of life in children with SeLECTS has been associated with executive dysfunction, antiseizure medication use, longer treatment duration, and higher seizure frequency (252).

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Contributors

All contributors' financial relationships have been reviewed and mitigated to ensure that this and every other article is free from commercial bias.

Authors

K P Vinayan MD DM

Dr. Vinayan of the Amrita Institute of Medical Sciences has no relevant financial relationships to disclose.
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Arushi Gahlot Saini MD DM MNAMS

Dr. Saini of Postgraduate Institute of Medical Education and Research, Chandigarh, India, has no relevant financial relationships to disclose.
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Editor

Solomon L Moshé MD

Dr. Moshé of Albert Einstein College of Medicine has no relevant financial relationships to disclose.
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Former Authors

Natalio Fejerman MD

Patient Profile

Age range of presentation: 2 to 18 years
Sex preponderance: male>female, >1:1
Heredity: heredity may be a factor

autosomal dominant
Population groups selectively affected: none selectively affected
Occupation groups selectively affected: none selectively affected

ICD & OMIM codes

ICD-10: Localization-related (focal) (partial) symptomatic epilepsy and epileptic syndromes with simple partial seizures: G40.1
ICD-11: Benign childhood epilepsy with centro-temporal spikes: 8A61.20

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Self-limited epilepsy with centrotemporal spikes

Associated Disorders

Idiopathic generalized seizures

Differential Diagnosis

cerebral palsy
Rett syndrome
DEE-SWAS
EE-SWAS
structural focal epilepsies
- SeLFEs

Also Relevant

Benign infantile seizures
Epilepsy
Epilepsy with myoclonic-atonic seizures
Landau-Kleffner syndrome
Lennox-Gastaut syndrome
- Panayiotopoulos syndrome
- Parasomnias
- Sleep and epilepsy

Clinical Categories

Epilepsy & Seizures

Self-limited epilepsy with centrotemporal spikes

Cite this article

Introduction

Overview

Key points

Historical note and terminology

Clinical manifestations

Clinical features

Associated problems

Prognosis and complications

Biological basis

Etiology and pathogenesis

Epidemiology

Differential diagnosis

Confusing conditions

Associated or underlying disorders

Diagnostic workup

EEG

Brain MRI

Positron emission tomography

Functional MRI (fMRI)

Transcranial magnetic stimulation

Other investigational techniques

Management

Antiseizure medication treatment

Duration of therapy and relapse

Nonpharmacological treatment

Outcomes

Special considerations

Anesthesia

References

Contributors

Authors

Editor

Former Authors

Patient Profile

ICD & OMIM codes

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Questions or Comment?

Also in This Category

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Self-limited (familial) infantile epilepsy

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Hippocampal and parahippocampal seizures

Sleep hyperkinetic (hypermotor) epilepsy

Alcohol withdrawal seizures

Typical absences

Visual-sensitive epilepsies

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