Self-limited focal epilepsies of childhood

Author:: Selim R Benbadis, MD
Section Editor:: Douglas R Nordli, Jr, MD
Deputy Editor:: John F Dashe, MD, PhD

Literature review current through: Jan 2024.

This topic last updated: Jan 05, 2024.

INTRODUCTION — Self-limited focal epilepsies of childhood occur in developmentally and neurologically normal children and have a favorable course, remitting prior to adulthood. These disorders will be reviewed here.

Localization-related epilepsies and other epileptic syndromes in childhood are discussed separately. (See "Focal epilepsy: Causes and clinical features" and "Epilepsy syndromes in children".)

CLASSIFICATION — Self-limited (formerly benign) focal epilepsies of childhood are electroclinical syndromes of suspected or proven genetic cause that occur in developmentally and neurologically normal children and have a favorable course, remitting prior to adulthood. In a change to the terminology by the International League Against Epilepsy (ILAE), the term "benign" was replaced by the term "self-limited," for these syndromes [1]. The term "benign" was abandoned because some individuals with these syndromes have associated comorbidities that affect quality of life.

The best-described syndromes are:

●Self-limited epilepsy with centrotemporal spikes (SeLECTS) (see 'Self-limited epilepsy with centrotemporal spikes (SeLECTS)' below)

●Self-limited epilepsy with autonomic seizures (SeLEAS) (see 'Self-limited epilepsy with autonomic seizures (SeLEAS)' below)

●Childhood occipital visual epilepsy (COVE) (see 'Childhood occipital visual epilepsy (COVE)' below)

These epilepsy syndromes are distinguished from structural focal epilepsy, which refers to epilepsy that results from brain injury or other structural brain disease. Thus, the self-limited focal epilepsies of childhood can be viewed as a spectrum of conditions with "functional" or "nonlesional" focal epileptogenicity, each characterized by location, seizure type(s), and electroencephalogram findings (figure 1). This is sometimes referred to as the benign childhood susceptibility syndrome [2,3]. (See "Seizures and epilepsy in children: Classification, etiology, and clinical features".)

Children with a first nonfebrile seizure or suspected epilepsy syndrome should be evaluated by a pediatrician or neurologist with experience in pediatric epilepsy to ensure an accurate diagnosis and proper management. (See "Seizures and epilepsy in children: Clinical and laboratory diagnosis".)

SELF-LIMITED EPILEPSY WITH CENTROTEMPORAL SPIKES (SeLECTS)

Terminology and epidemiology — SeLECTS was previously known as benign childhood epilepsy with centrotemporal spikes (BCECTS or BECTS), or as rolandic epilepsy [1].

SeLECTS is the most common type of focal epilepsy affecting children; it makes up approximately 10 to 20 percent of all childhood epilepsies [4-7].

Etiology — The presumed genetic basis of SeLECTS remains to be fully characterized [8]. Although more than one-fourth of patients have a family history of epilepsy, usually a focal epilepsy [8,9], a lack of concordance among twin pairs has argued against a major role for Mendelian-inherited factors [10,11]. However, the hallmark electroencephalographic (EEG) finding of centrotemporal spikes does appear to be inherited as an autosomal dominant trait [12,13]. In some families with this EEG trait, a linkage was found on chromosome 15q14 [12,14].

Pathogenic variants in the KCNQ2 gene have been identified in several families with SeLECTS [15]. Pathogenic variants in the GRIN2A gene that encodes for the alpha-2 subunit of the N-methyl-D-aspartate receptor have also been identified in rare sporadic and familial cases of SeLECTS, as well as in patients with more severe clinical syndromes on the same spectrum, including Landau-Kleffner and epileptic encephalopathy with continuous spikes and waves during sleep (CSWS) [16,17]. (See "Epilepsy syndromes in children", section on 'Developmental and epileptic encephalopathy with spike-wave activation in sleep (DEE-SWAS)'.)

Onset — SeLECTS usually presents in early school-age children with normal development. The age of onset ranges from 3 to 13 years, with a peak incidence between 7 and 9 years [9].

Clinical manifestations

Seizure types — The most common seizure type is a focal seizure with motor symptoms involving the face and no impairment of consciousness [9,18,19]. The characteristic ictal symptoms correspond to the origin of seizures in the rolandic or perisylvian sensorimotor cortex, which represents the face and oropharynx, and include facial numbness or twitching, guttural vocalizations, hypersalivation, drooling, dysphasia, and speech arrest [19-23]. Motor activity in the upper, but not lower, extremity is also common. Three-quarters of seizures occur at night or on awakening [9,18,19].

Although these focal seizures are the most frequent seizure type, they can go unnoticed, especially because they occur mainly in sleep. As a result, a common presentation of SeLECTS is a secondary generalized tonic-clonic seizure during sleep [4,19]. Overall, approximately half of children with SeLECTS have at least one secondary generalized seizure [5,9]. Focal or generalized status epilepticus is unusual [24]. Approximately 10 percent of children have postictal paresis, often in association with comorbid migraine headaches [25,26].

A distinct clinical subset of patients with SeLECTS has been described in seven patients who had typical EEG findings, but predominantly atonic seizures that occurred several times a day and began at a somewhat earlier age (mean 2.4 years) than children with more typical SeLECTS [27].

Cognitive and behavioral problems — A growing literature suggests that children with SeLECTS can have mild cognitive deficits and behavioral problems, such as attention deficit disorder, mood disorders, and language and learning difficulties [28-46]. In addition to the disease itself, potential causes of cognitive dysfunction in these children include antiseizure medications, postictal decrements, and sleep disturbances [47].

In a systematic review that included 18 studies on language function in children with SeLECTS compared with controls, the most consistent impairments were in phonologic processing and single-word reading [48]. The mean effect size was highest for single-word reading (0.7 standard deviations below the control group). Deficits in both expressive and receptive language were also identified, although results were more heterogeneous across studies. In a separate systematic review, long-term memory storage and retrieval were the most affected domains on formal neuropsychologic testing [49].

EEG findings — EEG findings of SeLECTS are characteristic, with centrotemporal sharp waves (70 to 100 milliseconds) that have several distinctive features (waveform 1) [4,5,18,50]:

●The morphology is biphasic, with a negative sharp peak followed by a positive rounded component (amplitude 50 percent of the negativity).

●The sharp waves often have a "horizontal dipole," which typically reveals a maximum negativity in the centrotemporal region and a maximum positivity at the vertex or in the frontal region.

●The sharp waves often occur in repetitive bursts and can be bilateral and independent.

●Epileptiform activity is markedly activated by non-rapid eye movement sleep, and occasionally occurs only in sleep [4,18,51].

●The background EEG activity is normal.

Although this distinctive EEG appearance is highly associated with SeLECTS [52], these interictal centrotemporal spikes also occur in 1 to 2 percent of asymptomatic children without seizures as well as in other epilepsy syndromes [4].

Diagnosis — Other tests, including neurologic examination, neuropsychiatric examination, and imaging studies, are normal in SeLECTS. Thus, if the history and EEG findings are all consistent with this syndrome, further studies may not be necessary [19,53].

Atypical features that may indicate further testing (magnetic resonance imaging) to rule out structural focal epilepsy or alternative diagnoses include [9,54]:

●Prolonged or frequent seizures or status epilepticus

●Atypical seizure type

●Seizures only while awake

●Atypical EEG features

●Postictal paresis

●Older age of onset

●Abnormal neurologic examination

●Cognitive regression

However, none of these findings excludes SeLECTS, as they have been reported in individuals who otherwise have clinical features and course that is consistent with SeLECTS [25,55,56]. In some cases, abnormal neuroimaging findings are coincidental and not related to SeLECTS [57].

Management

●Sleep hygiene – Sleep deprivation and other sleep disturbances may worsen seizure frequency. Healthy sleep practices for children are outlined in the table (table 1). Getting sufficient sleep along with keeping relatively constant bedtime and wake-up times on weekdays and weekends are particularly important. Identification of sleep problems in children is discussed in detail separately. (See "Assessment of sleep disorders in children".)

●Who should be treated with antiseizure medication? – Pharmacologic treatment is generally reserved for patients with SeLECTS who have greater seizure frequency (especially in the daytime) or severity, or those with secondarily generalized seizures [58]. For patients with SeLECTS who have focal seizures without impairment of consciousness, we suggest against treatment with antiseizure medication, if the child and family are comfortable with this approach [9,59]. In an observational case series, almost half of 79 patients were not treated [59]. Treatment was associated with a reduction in secondarily generalized but not focal seizures.

●Choice of antiseizure medication – If pharmacologic treatment is chosen, moderate doses of a single antiseizure medication are usually sufficient. Options include levetiracetam (the most common first choice due to its good safety profile, tolerability, and lack of drug-drug interactions) and other antiseizure medications (eg, oxcarbazepine, sulthiame, gabapentin) with demonstrated effectiveness in focal epilepsies [23,60-63]. Sulthiame (not available in the United States) demonstrated efficacy in a small placebo-controlled trial [64,65].

Rarely, antiseizure medications appear to aggravate SeLECTS, causing increased seizures, worsened EEG appearance, and even neuropsychologic abnormalities, all of which clear with discontinuation of the medication [66,67]. This rare complication has been reported with carbamazepine, phenobarbital, and lamotrigine. However, these medications are generally safe and effective for treating patients with SeLECTS and may be useful additional treatment options.

●Duration of antiseizure medication treatment – As in most epilepsies, an attempt at weaning antiseizure medications is warranted after a certain period of seizure freedom. One study suggested that a one-year seizure-free duration is sufficient before weaning treatment in patients with SeLECTS [68]. An initial trial of weaning is associated with relapse in approximately 14 percent of patients, but there is little information regarding any factors that might be associated with seizure relapse [9]. Nonetheless, patients and families should be aware of this possibility and participate in the decision to wean off treatment.

Prognosis

●Favorable natural history – The natural history of SeLECTS is favorable. Treated or not, this syndrome has an excellent prognosis, with spontaneous remission in the majority of patients before age 12 to 13 years [9,59,69]. In a case series of 87 patients, the mean duration of epilepsy was two years [5].

●Seizure complications – Seizures are typically infrequent and nocturnal, and there is a very low incidence of status epilepticus [5,39]. In a meta-analysis of 794 reported cases, 16 percent had only one seizure, and only 5 percent had 15 or more [9]. More frequent seizures and a longer duration of epilepsy appear to occur in those whose onset is prior to three years of age [5,69].

Although the risk of premature mortality is extremely low [70-72], rare cases of sudden unexpected death in epilepsy have been reported in children with a diagnosis of SeLECTS [73]. (See "Sudden unexpected death in epilepsy".)

Some children who go on to develop Landau-Kleffner syndrome or other epileptic encephalopathy (eg, epilepsy with CSWS, atypical benign focal epilepsy of childhood) may have a presentation in early childhood that is typical for SeLECTS. In reported cases, the more severe diagnosis was apparent within 6 to 24 months after presentation [39,74]. (See "Epilepsy syndromes in children", section on 'Developmental and epileptic encephalopathy with spike-wave activation in sleep (DEE-SWAS)'.)

●Cognitive and behavioral outcomes – As discussed above (see 'Cognitive and behavioral problems' above), there is some evidence that SeLECTS may be associated with mild cognitive impairment, particularly with respect to reading and language. Most longitudinal follow-up studies suggest that these cognitive impairments may resolve, with seizures, by adolescence [28,29,45]; however, at least one study found that there was evidence of enduring language deficits into early adulthood [75]. In this study as well as others, there was evidence of atypical cortical lateralization of language [36,75-77]. One case series found that, compared with controls, children with SeLECTS had bilateral putaminal hypertrophy, which appeared to be cognitively adaptive, as this finding was associated with improved cognitive performance [78].

SELF-LIMITED EPILEPSY WITH AUTONOMIC SEIZURES (SeLEAS)

Terminology — SeLEAS was previously known as early-onset childhood occipital epilepsy, often referred to as Panayiotopoulos syndrome [1].

Onset — Occurring in early childhood, SeLEAS presents at a mean age of younger than 5 years (range 1 to 14 years) [79-83].

Clinical manifestations — SeLEAS is characterized by a unique seizure type that has prominent autonomic features [79-89].

●Seizures – The seizures in this syndrome have a distinctive yet variable phenomenology compared with other focal epilepsy syndromes. Vomiting is the most characteristic and frequent ictal sign, occurring in 70 to 85 percent of patients [79,80,82-86,90]. Syncope-like seizures, with sudden loss of muscle tone and unresponsiveness with or without associated autonomic signs, have been described in up to half of patients [91]. Visual symptoms, usually a seizure aura, are variably present. Many seizures are characterized by progressive impairment of consciousness along with staring or head and eye deviation. In some seizures, focal or bilateral clonic activity occurs.

The seizures are usually nocturnal and last more than five minutes [79,80,83-86]. One-third to one-half of episodes last more than 30 minutes, representing a nonconvulsive status epilepticus. Generalized convulsive status epilepticus is uncommon.

A small number of patients (10 to 13 percent) with SeLEAS also have or develop (childhood) epilepsy with centrotemporal spikes (SeLECTS), formerly known as rolandic epilepsy [84,89]. (See 'Self-limited epilepsy with centrotemporal spikes (SeLECTS)' above.)

●Autonomic features – Along with vomiting, other common autonomic signs, which are typically at seizure onset, are pallor (or less often flushing, cyanosis), mydriasis, miosis, incontinence, coughing, and hypersalivation [84,90]. Cardiac disturbances are usually limited to tachycardia, but cardiopulmonary arrest has been described [84].

EEG findings — Interictal electroencephalography (EEG) in SeLEAS shows occipital spikes in approximately 75 percent of cases; however, epileptiform activity arising from other or even multiple areas is not uncommon [82,83,86,88,92]. The morphology of the spikes is similar to that seen in SeLECTS (see 'Self-limited epilepsy with centrotemporal spikes (SeLECTS)' above). The occipital spikes may also show fixation-off sensitivity (ie, abnormalities on routine EEG that consistently occur with the eyes closed but not with eyes open), as seen in late-onset childhood occipital epilepsy (Gastaut type). As with SeLECTS, the frequency of epileptiform activity increases during sleep, and background activity is normal.

Ictal recordings have demonstrated variable loci of seizure onset, including frontal, frontotemporal, and occipital origins [79,86]. The seizure discharge consists mainly of rhythmic theta or delta activity, intermixed with small spikes [90].

Diagnosis — The diagnosis of SeLEAS is made in the setting of a suggestive clinical history in a child of the typical age with the characteristic EEG findings. A sleep recording may be required if the routine EEG is normal [89]. Magnetic resonance imaging is normal in SeLEAS and is not required if the other findings are diagnostic [88].

Differential diagnosis — The unusual symptomatology of SeLEAS requires a high level of suspicion for epilepsy. Because of the prominent dysautonomic features, these seizures can be mistaken for nonepileptic syndromes, including syncope, gastroenteritis, encephalitis, and migraine [82,85,89,93-95].

SeLEAS can usually be distinguished from structural temporal lobe epilepsy (TLE). In TLE, autonomic symptoms are usually much less pronounced and occur near the end rather than at the beginning of the seizure. (See "Focal epilepsy: Causes and clinical features".)

Management

●Prolonged seizures – Prolonged seizures in patients with SeLEAS should be treated as nonconvulsive status epilepticus. Benzodiazepines (eg, rectal diazepam, 0.5 mg/kg, maximum 20 mg) are generally advocated as the initial treatment [84,85,89].

Instruction in the use of the benzodiazepine should be provided to parents and caregivers of children who have had a prolonged seizure [84,85,89].

However, there is no evidence that these prolonged seizures pose a risk of brain damage, and experts suggest avoiding over-treatment with multiple drugs that have the potential for cardiorespiratory complications [90,96]. (See "Nonconvulsive status epilepticus: Treatment and prognosis".)

●Seizure prevention – Prophylactic pharmacologic treatment is reserved for patients with frequent seizures or those with severe symptoms. Although any of the antiseizure medications used for focal epilepsy may be used, levetiracetam is generally considered the first choice because of its good safety profile, tolerability, and lack of drug-drug interactions [97,98]. Historically, there is the most experience with carbamazepine. In a report of 172 children with SeLEAS, antiseizure medication treatment as monotherapy with carbamazepine (112 patients), phenobarbital (20), valproate (20), oxcarbazepine (10), or clobazam (10) was associated with seizure freedom in 88 percent [79]. However, 71 patients (42 percent) had experienced only a single seizure before treatment.

●Duration of antiseizure medication treatment – Withdrawal of antiseizure medication treatment is recommended within one to two years if seizures are not ongoing.

Prognosis — The clinical course of SeLEAS is usually favorable. Seizures are infrequent, and almost half of patients have just a single seizure [79,82,83,85]. Spontaneous remission usually occurs within two to three years from onset.

A few children develop drug-resistant seizures or neuropsychologic deficits [79,88]. Preexisting neurobehavioral disorders may be a risk factor for recurrent, more difficult to treat seizures [99].

CHILDHOOD OCCIPITAL VISUAL EPILEPSY (COVE)

Terminology — COVE was previously known as late-onset childhood occipital epilepsy, benign occipital epilepsy of childhood, or Gastaut syndrome [1].

Onset — COVE has a mean age of presentation of 8 to 9 years (range 3 to 16 years), which is somewhat later than in SeLEAS [89,100].

Clinical manifestations — Seizures often include visual symptoms, either blindness or elementary visual hallucinations [82,87,100-105]. Hemiclonic activity, automatisms, migraine-like headache, and versive movements can occur, and seizures may secondarily generalize. Compared with SeLEAS, seizures are more frequent and of shorter duration, usually less than five minutes. Most seizures occur in the daytime.

EEG findings — The interictal EEG of COVE is similar to that in SeLEAS, but with some differences: Background activity is normal, but epileptiform activity is more predominantly occipital, often occurs in long bursts of spike-wave complexes, and is markedly activated by eye closure (ie, fixation-off sensitivity) [101,105,106].

Diagnosis — The diagnosis of COVE is made in the setting of a suggestive clinical history in a child of the typical age with the characteristic EEG findings.

Because clinical and EEG features of subtypes of occipital epilepsy overlap substantially, magnetic resonance imaging is generally obtained to exclude an underlying brain lesion [53,87,105,107]. Migraine with aura is also frequently considered in the differential diagnosis [93]. (See "Focal epilepsy: Causes and clinical features", section on 'Occipital lobe epilepsy'.)

Treatment and prognosis — The course of COVE is less consistently benign than the previously described syndromes. Antiseizure medication treatment is more often prescribed since the duration of epilepsy is longer and seizures can be frequent [100,105]. Any medication used in focal epilepsies is likely to be efficacious (see "Seizures and epilepsy in children: Initial treatment and monitoring", section on 'Seizure-related considerations'). As with other self-limited epilepsies, levetiracetam is generally considered the first choice because of its good safety profile, tolerability, and lack of drug-drug interactions [98,108]. Historically, carbamazepine was the most commonly prescribed in published cases [104].

Seizures may persist into adulthood but remit beforehand in two-thirds of patients [105,106,109].

SELF-LIMITED FOCAL EPILEPSIES IN INFANCY — Several self-limited focal epilepsy syndromes, both familial and sporadic, have been described that begin in the neonatal period or during infancy. These are reviewed separately. (See "Overview of neonatal epilepsy syndromes", section on 'Self-limited (familial) neonatal syndromes' and "Overview of infantile epilepsy syndromes", section on 'Self-limited infantile epilepsy syndromes'.)

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●Beyond the Basics topics (see "Patient education: Seizures in children (Beyond the Basics)" and "Patient education: Treatment of seizures in children (Beyond the Basics)")

SUMMARY AND RECOMMENDATIONS

●Description – Self-limited focal epilepsies of childhood share as common features a normal neurologic and developmental history, age-dependent onset, specific clinical and electroencephalographic (EEG) features, and a favorable long-term prognosis with spontaneous seizure remission prior to adulthood. (See 'Self-limited epilepsy with centrotemporal spikes (SeLECTS)' above.)

●Self-limited epilepsy with centrotemporal spikes (SeLECTS) – SeLECTS, previously known as benign childhood epilepsy with centrotemporal spikes (BECTS) or rolandic epilepsy, is the most common type of focal epilepsy affecting children, presenting at a mean age of eight years, with focal motor or secondarily generalized seizures that occur mostly at night. (See 'Self-limited epilepsy with centrotemporal spikes (SeLECTS)' above.)

•Centrotemporal spikes are seen on EEG in patients with SeLECTS (waveform 1). These have a distinctive morphology and are increased with sleep. Although this EEG pattern is highly correlated with SeLECTS, it can also occur in asymptomatic children.

•A typical history and EEG are sufficient to establish the diagnosis of SeLECTS. The possibility of symptomatic epilepsy should be evaluated with magnetic resonance imaging when there are atypical features.

•For patients with SeLECTS who have focal seizures without impairment of consciousness, we suggest against using antiseizure medications (Grade 2B). Antiseizure medications with efficacy in focal seizures can reduce the frequency of secondary generalized seizures in SeLECTS and can be considered in those patients with a high frequency or severity of seizures. (See 'Management' above and "Seizures and epilepsy in children: Initial treatment and monitoring", section on 'Seizure-related considerations'.)

•In most cases of SeLECTS, seizures remit before or during adolescence. Mild cognitive and behavioral disturbances are common and may improve with age as well.

●Self-limited epilepsy with autonomic seizures (SeLEAS) – SeLEAS, previously known as early-onset childhood occipital epilepsy, or as Panayiotopoulos syndrome, describes a self-limited focal epilepsy associated with interictal occipital spikes that begins in early childhood (mean age five years) with rare seizures that have prominent autonomic features (eg, vomiting, pallor) and are often prolonged. Prolonged seizures in patients with SeLEAS require treatment with antiseizure medication. However, approximately one-half of patients have only a single seizure, and prophylactic treatment may not be needed. (See 'Self-limited epilepsy with autonomic seizures (SeLEAS)' above.)

●Childhood occipital visual epilepsy (COVE) – COVE, previously known as late-onset childhood occipital epilepsy (Gastaut type), or as benign occipital epilepsy of childhood, presents at a mean age of nine years with seizures that have prominent visual symptoms including blindness and hallucinations. Seizures usually occur during the daytime and are brief but can be frequent, and thus require antiseizure medication treatment in most individuals. EEG shows occipital spikes that are activated by eye closure. Seizures often remit prior to adulthood. (See 'Childhood occipital visual epilepsy (COVE)' above.)

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Topic 6158 Version 41.0

خرید پکیج

Self-limited focal epilepsies of childhood

References