Your activity: 171 p.v.
your limit has been reached. plz Donate us to allow your ip full access, Email: sshnevis@outlook.com

Benign (self-limited) focal epilepsies of childhood

Benign (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: Dec 2022. | This topic last updated: Dec 02, 2021.

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

Symptomatic 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 — Benign (self-limited) focal epilepsies of childhood are electroclinical syndromes of unknown or genetic cause that occur in developmentally and neurologically normal children and have a benign course, remitting prior to adulthood.

The best-described syndromes are:

Benign epilepsy with centrotemporal spikes (BECTS), also referred to as rolandic epilepsy

Early-onset childhood occipital epilepsy (Panayiotopoulos type), often referred to as Panayiotopoulos syndrome

Late-onset childhood occipital epilepsy (Gastaut type), also referred to as benign occipital epilepsy of childhood or Gastaut syndrome

These epilepsy syndromes are distinguished from symptomatic focal epilepsy, which refers to epilepsy that results from brain injury or other structural brain disease. Thus, the benign 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 [1,2]. (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 appropriate management. (See "Seizures and epilepsy in children: Clinical and laboratory diagnosis".)

BENIGN EPILEPSY WITH CENTROTEMPORAL SPIKES — Benign (childhood) epilepsy with centrotemporal spikes (BCECTS or BECTS), also known as rolandic epilepsy, is the most common type of focal epilepsy affecting children; it makes up approximately 10 to 20 percent of all childhood epilepsies [3-6]. In a proposed change to the terminology under review by the International League Against Epilepsy (ILAE), the term "benign" will be replaced by "self-limited," and the new terminology for BECTS will be "Self-limited epilepsy with centrotemporal spikes (SeLECTS)" [7].

Etiology — The presumed genetic basis of BECTS 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 BECTS [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 BECTS, as well as in patients with other clinical syndromes, 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 — BECTS 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 BECTS is a secondary generalized tonic-clonic seizure during sleep [3,19]. Overall, approximately half of children with BECTS have at least one secondary generalized seizure [4,9]. 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 BECTS 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 BECTS [27].

Cognitive and behavioral problems — A growing literature suggests that children with BECTS 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 BECTS 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 BECTS are characteristic, with centrotemporal sharp waves (70 to 100 milliseconds) that have several distinctive features (waveform 1) [3,4,18,50]:

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 [3,18,51].

The background EEG activity is normal.

Although this distinctive EEG appearance is highly associated with BECTS [52], these interictal centrotemporal spikes also occur in 1 to 2 percent of asymptomatic children without seizures as well as in other epilepsy syndromes [3].

Diagnosis — Other tests, including neurologic examination, neuropsychiatric examination, and imaging studies, are normal in BECTS. 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 symptomatic focal epilepsy 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

However, any one of these findings does not exclude BECTS, as they have been reported in individuals who otherwise have clinical features and course that is consistent with BECTS [25,55,56]. In some cases, abnormal neuroimaging findings are coincidental and not related to BECTS [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 BECTS (ie, rolandic epilepsy) who have greater seizure frequency (especially in the daytime) or severity, or those with secondary generalized seizures [58]. For patients with BECTS 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 generalized but not focal seizures.

Choice of antiseizure medication – If pharmacologic treatment is elected, 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 BECTS, causing increased seizures, worsened EEG appearance, and even neuropsychologic abnormalities, all of which clear with discontinuation of the medication [66,67]. This complication has been reported with carbamazepine, phenobarbital, and lamotrigine. However, these medications are generally safe and effective for treating patients with BECTS.

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 BECTS [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 BECTS 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 [4].

Seizure complications – Seizures are typically infrequent and nocturnal, and there is a very low incidence of status epilepticus [4,39]. 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 [4,69].

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 BECTS [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 BECTS. 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 BECTS 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 BECTS had bilateral putaminal hypertrophy, which appeared to be cognitively adaptive, as this finding was associated with improved cognitive performance [78].

BENIGN CHILDHOOD EPILEPSY WITH OCCIPITAL SPIKES — Benign childhood epilepsy with occipital spikes has two major variants: early-onset childhood occipital epilepsy (Panayiotopoulos type) and late-onset childhood occipital epilepsy (Gastaut type). However, one case series of 16 patients found that the syndromes were not discrete, with one-third of children showing mixed features of both syndromes [79]. Twin pairs in this case series were as likely to be discordant as concordant for benign occipital epilepsy, Panayiotopoulos, or mixed syndromes.

EARLY-ONSET CHILDHOOD OCCIPITAL EPILEPSY (PANAYIOTOPOULOS TYPE) — Early-onset benign childhood occipital epilepsy, often referred to as Panayiotopoulos syndrome, is characterized by a unique seizure type that has prominent autonomic features [80-90]. In a proposed change to the terminology under review by the International League Against Epilepsy (ILAE), the term "benign" will be replaced by "self-limited," and the new terminology for this syndrome will be "Self-limited epilepsy with autonomic seizures (SeLEAS)" [7].

Onset — Occurring in early childhood, Panayiotopoulos syndrome presents at a mean age of younger than 5 years (range 1 to 14 years) [83,84,88-90].

Clinical manifestations

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 [80-84,89-91]. 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 [92]. 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 [80-84,90]. 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 Panayiotopoulos syndrome also have or develop benign (childhood) epilepsy with centrotemporal spikes (BECTS), also known as rolandic epilepsy [80,87]. (See 'Benign epilepsy with centrotemporal spikes' 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 [80,91]. Cardiac disturbances are usually limited to tachycardia, but cardiopulmonary arrest has been described [80].

EEG findings — Interictal electroencephalography (EEG) in Panayiotopoulos syndrome shows occipital spikes in approximately 75 percent of cases; however, epileptiform activity arising from other or even multiple areas is not uncommon [82,86,89,90,93]. The morphology of the spikes is similar to that seen in BECTS (see 'Benign epilepsy with centrotemporal spikes' 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 BECTS, 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 [82,83]. The seizure discharge consists mainly of rhythmic theta or delta activity, intermixed with small spikes [91].

Diagnosis — The diagnosis of Panayiotopoulos syndrome 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 [87]. Magnetic resonance imaging is normal in Panayiotopoulos syndrome and is not required if the other findings are diagnostic [86].

Differential diagnosis — The unusual symptomatology of Panayiotopoulos syndrome 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 [81,87,89,94-96].

Panayiotopoulos syndrome can usually be distinguished from symptomatic 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 Panayiotopoulos syndrome 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 [80,81,87].

Instruction in the use of the benzodiazepine should be provided to parents and caregivers of children who have had a prolonged seizure [80,81,87].

However, there is no evidence that these prolonged seizures pose a risk of brain damage, and experts suggest avoiding over-treatment with multiple intravenous drugs that have the potential for cardiorespiratory complications [91]. (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 Panayiotopoulos syndrome, 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 [83]. 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 Panayiotopoulos syndrome is usually benign. Seizures are infrequent, and almost half of patients have just a single seizure [81,83,89,90]. Spontaneous remission usually occurs within two to three years from onset.

A few children develop drug-resistant seizures or neuropsychologic deficits [83,86]. Preexisting neurobehavioral disorders may be a risk factor for recurrent, more difficult to treat seizures [99].

LATE-ONSET CHILDHOOD OCCIPITAL EPILEPSY (GASTAUT TYPE) — Late-onset childhood occipital epilepsy is also referred to as benign occipital epilepsy of childhood or Gastaut syndrome. In a proposed change under review by the International League Against Epilepsy (ILAE), the new terminology for this syndrome will be "Childhood occipital visual epilepsy (COVE)" [7].

Onset — Late-onset childhood occipital epilepsy has a mean age of presentation of 8 to 9 years (range 3 to 16 years), which is somewhat later than in Panayiotopoulos syndrome [87,100].

Clinical manifestations — Seizures often include visual symptoms, either blindness or elementary visual hallucinations [85,89,100-105]. Hemiclonic activity, automatisms, migraine-like headache, and versive movements can occur, and seizures may secondarily generalize. Compared with Panayiotopoulos syndrome, seizures are more frequent and of shorter duration, usually less than five minutes. Most seizures occur in the daytime.

EEG findings — The interictal EEG is similar to that in Panayiotopoulos syndrome, but with some differences: 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]. Background activity is normal.

Diagnosis — The diagnosis of late-onset childhood occipital epilepsy 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 symptomatic and benign occipital epilepsy overlap substantially, magnetic resonance imaging is generally recommended to exclude a symptomatic cause of late-onset childhood occipital epilepsy, such as a structural brain lesion [53,85,105,107]. Migraine with aura is also frequently considered in the differential diagnosis [94]. (See "Focal epilepsy: Causes and clinical features", section on 'Occipital lobe epilepsy'.)

Treatment and prognosis — The course of benign occipital epilepsy of childhood 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].

BENIGN FOCAL EPILEPSIES IN INFANCY — A number of benign 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-infantile epilepsy' and "Overview of neonatal epilepsy syndromes", section on 'Self-limited (familial) neonatal epilepsy'.)

INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5th to 6th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)

Basics topic (see "Patient education: Epilepsy in children (The Basics)")

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 – Benign 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 'Benign epilepsy with centrotemporal spikes' above.)

Benign epilepsy with centrotemporal spikes – Benign epilepsy with centrotemporal spikes (BECTS), also called 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 'Benign epilepsy with centrotemporal spikes' above.)

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

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

For patients with BECTS 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 BECTS 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 BECTS, seizures remit before or during adolescence. Mild cognitive and behavioral disturbances are common and may improve with age as well.

Early-onset childhood occipital epilepsy (Panayiotopoulos type) – Early-onset childhood occipital epilepsy, often referred to as Panayiotopoulos syndrome, describes a benign 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 Panayiotopoulos syndrome require treatment with antiseizure medication. However, approximately one-half of patients have only a single seizure, and prophylactic treatment may not be required. (See 'Early-onset childhood occipital epilepsy (Panayiotopoulos type)' above.)

Late-onset childhood occipital epilepsy (Gastaut type) – Late-onset childhood occipital epilepsy, also referred to as benign occipital epilepsy of childhood or Gastaut syndrome, 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 demonstrates occipital spikes that are activated by eye closure. Seizures often remit prior to adulthood. (See 'Late-onset childhood occipital epilepsy (Gastaut type)' above.)

  1. Panayiotopoulos CP. Benign childhood partial epilepsies: benign childhood seizure susceptibility syndromes. J Neurol Neurosurg Psychiatry 1993; 56:2.
  2. Panayiotopoulos CP. Benign childhood focal seizures and related epileptic syndromes. In: A Clinical Guide to Epileptic Syndromes and Their Treatment, 2nd ed, Panayiotopoulos CP (Ed), Springer, London 2007. p.285.
  3. Lüders H, Lesser RP, Dinner DS, et al. Benign focal epilepsy of childhood. In: Epilepsy: Electroclinical Syndromes, Lüders H, Lesser RP (Eds), Springer-Verlag, London 1987. p.303.
  4. Kramer U, Zelnik N, Lerman-Sagie T, Shahar E. Benign childhood epilepsy with centrotemporal spikes: clinical characteristics and identification of patients at risk for multiple seizures. J Child Neurol 2002; 17:17.
  5. Kramer U, Nevo Y, Neufeld MY, et al. Epidemiology of epilepsy in childhood: a cohort of 440 consecutive patients. Pediatr Neurol 1998; 18:46.
  6. Cavazzuti GB. Epidemiology of different types of epilepsy in school age children of Modena, Italy. Epilepsia 1980; 21:57.
  7. International League Against Epilepsy. Proposed classification: Syndromes in children. Available at: https://www.ilae.org/guidelines/definition-and-classification/proposed-classification-and-definition-of-epilepsy-syndromes/proposed-classification-syndromes-in-children (Accessed on November 19, 2021).
  8. Vears DF, Tsai MH, Sadleir LG, et al. Clinical genetic studies in benign childhood epilepsy with centrotemporal spikes. Epilepsia 2012; 53:319.
  9. Bouma PA, Bovenkerk AC, Westendorp RG, Brouwer OF. The course of benign partial epilepsy of childhood with centrotemporal spikes: a meta-analysis. Neurology 1997; 48:430.
  10. Vadlamudi L, Harvey AS, Connellan MM, et al. Is benign rolandic epilepsy genetically determined? Ann Neurol 2004; 56:129.
  11. Vadlamudi L, Kjeldsen MJ, Corey LA, et al. Analyzing the etiology of benign rolandic epilepsy: a multicenter twin collaboration. Epilepsia 2006; 47:550.
  12. Neubauer BA. The genetics of rolandic epilepsy. Epileptic Disord 2000; 2 Suppl 1:S67.
  13. Bali B, Kull LL, Strug LJ, et al. Autosomal dominant inheritance of centrotemporal sharp waves in rolandic epilepsy families. Epilepsia 2007; 48:2266.
  14. Pruna D, Persico I, Serra D. Lack of association with the 15q14 candidate region for benign epilepsy of childhood with centro-temporal spikes in a Sardinian population. Epilepsia 2000; 41:164.
  15. Neubauer BA, Waldegger S, Heinzinger J, et al. KCNQ2 and KCNQ3 mutations contribute to different idiopathic epilepsy syndromes. Neurology 2008; 71:177.
  16. Lemke JR, Lal D, Reinthaler EM, et al. Mutations in GRIN2A cause idiopathic focal epilepsy with rolandic spikes. Nat Genet 2013; 45:1067.
  17. Lesca G, Rudolf G, Bruneau N, et al. GRIN2A mutations in acquired epileptic aphasia and related childhood focal epilepsies and encephalopathies with speech and language dysfunction. Nat Genet 2013; 45:1061.
  18. Gregory DL, Wong PK. Topographical analysis of the centrotemporal discharges in benign rolandic epilepsy of childhood. Epilepsia 1984; 25:705.
  19. Loiseau P, Beaussart M. The seizures of benign childhood epilepsy with Rolandic paroxysmal discharges. Epilepsia 1973; 14:381.
  20. Uematsu S, Lesser R, Fisher RS, et al. Motor and sensory cortex in humans: topography studied with chronic subdural stimulation. Neurosurgery 1992; 31:59.
  21. Penfield W, Jasper H. Epilepsy and the Functional Anatomy of the Brain, Little, Brown and Co, Boston 1954.
  22. Lombroso CT. Sylvian seizures and midtemporal spike foci in children. Arch Neurol 1967; 17:52.
  23. Ma CK, Chan KY. Benign childhood epilepsy with centrotemporal spikes: a study of 50 Chinese children. Brain Dev 2003; 25:390.
  24. Gregory DL, Farrell K, Wong PK. Partial status epilepticus in benign childhood epilepsy with centrotemporal spikes: are independent right and left seizures a risk factor? Epilepsia 2002; 43:936.
  25. Dai AI, Weinstock A. Postictal paresis in children with benign rolandic epilepsy. J Child Neurol 2005; 20:834.
  26. Dai AI, Demiryürek S. The Clinical Implications of Todd Paralysis in Children With Benign Rolandic Epilepsy. J Child Neurol 2016; 31:289.
  27. Cherian A, Baheti NN, Menon RN, et al. Atonic variant of benign childhood epilepsy with centrotemporal spikes (atonic-BECTS): a distinct electro-clinical syndrome. Brain Dev 2012; 34:511.
  28. Baglietto MG, Battaglia FM, Nobili L, et al. Neuropsychological disorders related to interictal epileptic discharges during sleep in benign epilepsy of childhood with centrotemporal or Rolandic spikes. Dev Med Child Neurol 2001; 43:407.
  29. Lindgren S, Kihlgren M, Melin L, et al. Development of cognitive functions in children with rolandic epilepsy. Epilepsy Behav 2004; 5:903.
  30. Northcott E, Connolly AM, Berroya A, et al. The neuropsychological and language profile of children with benign rolandic epilepsy. Epilepsia 2005; 46:924.
  31. Clarke T, Strug LJ, Murphy PL, et al. High risk of reading disability and speech sound disorder in rolandic epilepsy families: case-control study. Epilepsia 2007; 48:2258.
  32. Perkins FF Jr, Breier J, McManis MH, et al. Benign rolandic epilepsy -- perhaps not so benign: use of magnetic source imaging as a predictor of outcome. J Child Neurol 2008; 23:389.
  33. Boatman DF, Trescher WH, Smith C, et al. Cortical auditory dysfunction in benign rolandic epilepsy. Epilepsia 2008; 49:1018.
  34. Kavros PM, Clarke T, Strug LJ, et al. Attention impairment in rolandic epilepsy: systematic review. Epilepsia 2008; 49:1570.
  35. Vago C, Bulgheroni S, Franceschetti S, et al. Memory performance on the California Verbal Learning Test of children with benign childhood epilepsy with centrotemporal spikes. Epilepsy Behav 2008; 13:600.
  36. Lillywhite LM, Saling MM, Harvey AS, et al. Neuropsychological and functional MRI studies provide converging evidence of anterior language dysfunction in BECTS. Epilepsia 2009; 50:2276.
  37. Danielsson J, Petermann F. Cognitive deficits in children with benign rolandic epilepsy of childhood or rolandic discharges: a study of children between 4 and 7 years of age with and without seizures compared with healthy controls. Epilepsy Behav 2009; 16:646.
  38. Cerminara C, D'Agati E, Lange KW, et al. Benign childhood epilepsy with centrotemporal spikes and the multicomponent model of attention: a matched control study. Epilepsy Behav 2010; 19:69.
  39. Tovia E, Goldberg-Stern H, Ben Zeev B, et al. The prevalence of atypical presentations and comorbidities of benign childhood epilepsy with centrotemporal spikes. Epilepsia 2011; 52:1483.
  40. Smith AB, Kavros PM, Clarke T, et al. A neurocognitive endophenotype associated with rolandic epilepsy. Epilepsia 2012; 53:705.
  41. Vannest J, Tenney JR, Gelineau-Morel R, et al. Cognitive and behavioral outcomes in benign childhood epilepsy with centrotemporal spikes. Epilepsy Behav 2015; 45:85.
  42. Danhofer P, Pejčochová J, Dušek L, et al. The influence of EEG-detected nocturnal centrotemporal discharges on the expression of core symptoms of ADHD in children with benign childhood epilepsy with centrotemporal spikes (BCECTS): A prospective study in a tertiary referral center. Epilepsy Behav 2018; 79:75.
  43. Lima EM, Rzezak P, Dos Santos B, et al. The relevance of attention deficit hyperactivity disorder in self-limited childhood epilepsy with centrotemporal spikes. Epilepsy Behav 2018; 82:164.
  44. Teixeira J, Santos ME. Language skills in children with benign childhood epilepsy with centrotemporal spikes: A systematic review. Epilepsy Behav 2018; 84:15.
  45. Ross EE, Stoyell SM, Kramer MA, et al. The natural history of seizures and neuropsychiatric symptoms in childhood epilepsy with centrotemporal spikes (CECTS). Epilepsy Behav 2020; 103:106437.
  46. Zanaboni MP, Varesio C, Pasca L, et al. Systematic review of executive functions in children with self-limited epilepsy with centrotemporal spikes. Epilepsy Behav 2021; 123:108254.
  47. Tang SS, Clarke T, Owens J, Pal DK. Sleep behavior disturbances in rolandic epilepsy. J Child Neurol 2011; 26:239.
  48. Smith AB, Bajomo O, Pal DK. A meta-analysis of literacy and language in children with rolandic epilepsy. Dev Med Child Neurol 2015; 57:1019.
  49. Wickens S, Bowden SC, D'Souza W. Cognitive functioning in children with self-limited epilepsy with centrotemporal spikes: A systematic review and meta-analysis. Epilepsia 2017; 58:1673.
  50. Berroya AM, Bleasel AF, Stevermuer TL, et al. Spike morphology, location, and frequency in benign epilepsy with centrotemporal spikes. J Child Neurol 2005; 20:188.
  51. Blom S, Heijbel J. Benign epilepsy of children with centro-temporal EEG foci. Discharge rate during sleep. Epilepsia 1975; 16:133.
  52. Frost JD Jr, Hrachovy RA, Glaze DG. Spike morphology in childhood focal epilepsy: relationship to syndromic classification. Epilepsia 1992; 33:531.
  53. Camfield P, Camfield C. Epileptic syndromes in childhood: clinical features, outcomes, and treatment. Epilepsia 2002; 43 Suppl 3:27.
  54. Shevell MI, Rosenblatt B, Watters GV, et al. "Pseudo-BECRS": intracranial focal lesions suggestive of a primary partial epilepsy syndrome. Pediatr Neurol 1996; 14:31.
  55. Wirrell EC, Camfield PR, Gordon KE, et al. Benign rolandic epilepsy: atypical features are very common. J Child Neurol 1995; 10:455.
  56. Datta A, Sinclair DB. Benign epilepsy of childhood with rolandic spikes: typical and atypical variants. Pediatr Neurol 2007; 36:141.
  57. Gelisse P, Corda D, Raybaud C, et al. Abnormal neuroimaging in patients with benign epilepsy with centrotemporal spikes. Epilepsia 2003; 44:372.
  58. Pearl PL. Epilepsy Syndromes in Childhood. Continuum (Minneap Minn) 2018; 24:186.
  59. Peters JM, Camfield CS, Camfield PR. Population study of benign rolandic epilepsy: is treatment needed? Neurology 2001; 57:537.
  60. Tzitiridou M, Panou T, Ramantani G, et al. Oxcarbazepine monotherapy in benign childhood epilepsy with centrotemporal spikes: a clinical and cognitive evaluation. Epilepsy Behav 2005; 7:458.
  61. Bello-Espinosa LE, Roberts SL. Levetiracetam for benign epilepsy of childhood with centrotemporal spikes-three cases. Seizure 2003; 12:157.
  62. Coppola G, Franzoni E, Verrotti A, et al. Levetiracetam or oxcarbazepine as monotherapy in newly diagnosed benign epilepsy of childhood with centrotemporal spikes (BECTS): an open-label, parallel group trial. Brain Dev 2007; 29:281.
  63. Hughes JR. Benign epilepsy of childhood with centrotemporal spikes (BECTS): to treat or not to treat, that is the question. Epilepsy Behav 2010; 19:197.
  64. Rating D, Wolf C, Bast T. Sulthiame as monotherapy in children with benign childhood epilepsy with centrotemporal spikes: a 6-month randomized, double-blind, placebo-controlled study. Sulthiame Study Group. Epilepsia 2000; 41:1284.
  65. Tan HJ, Singh J, Gupta R, de Goede C. Comparison of antiepileptic drugs, no treatment, or placebo for children with benign epilepsy with centro temporal spikes. Cochrane Database Syst Rev 2014; :CD006779.
  66. Corda D, Gelisse P, Genton P, et al. Incidence of drug-induced aggravation in benign epilepsy with centrotemporal spikes. Epilepsia 2001; 42:754.
  67. Catania S, Cross H, de Sousa C, Boyd S. Paradoxic reaction to lamotrigine in a child with benign focal epilepsy of childhood with centrotemporal spikes. Epilepsia 1999; 40:1657.
  68. Braathen G, Andersson T, Gylje H, et al. Comparison between one and three years of treatment in uncomplicated childhood epilepsy: a prospective study. I. Outcome in different seizure types. Epilepsia 1996; 37:822.
  69. Loiseau P, Duché B, Cordova S, et al. Prognosis of benign childhood epilepsy with centrotemporal spikes: a follow-up study of 168 patients. Epilepsia 1988; 29:229.
  70. Callenbach PM, Bouma PA, Geerts AT, et al. Long term outcome of benign childhood epilepsy with centrotemporal spikes: Dutch Study of Epilepsy in Childhood. Seizure 2010; 19:501.
  71. Camfield CS, Camfield PR. Rolandic epilepsy has little effect on adult life 30 years later: a population-based study. Neurology 2014; 82:1162.
  72. Berg AT, Nickels K, Wirrell EC, et al. Mortality risks in new-onset childhood epilepsy. Pediatrics 2013; 132:124.
  73. Doumlele K, Friedman D, Buchhalter J, et al. Sudden Unexpected Death in Epilepsy Among Patients With Benign Childhood Epilepsy With Centrotemporal Spikes. JAMA Neurol 2017; 74:645.
  74. Fejerman N, Caraballo R, Tenembaum SN. Atypical evolutions of benign localization-related epilepsies in children: are they predictable? Epilepsia 2000; 41:380.
  75. Monjauze C, Broadbent H, Boyd SG, et al. Language deficits and altered hemispheric lateralization in young people in remission from BECTS. Epilepsia 2011; 52:e79.
  76. Datta AN, Oser N, Bauder F, et al. Cognitive impairment and cortical reorganization in children with benign epilepsy with centrotemporal spikes. Epilepsia 2013; 54:487.
  77. Bulgheroni S, Franceschetti S, Vago C, et al. Verbal dichotic listening performance and its relationship with EEG features in benign childhood epilepsy with centrotemporal spikes. Epilepsy Res 2008; 79:31.
  78. Lin JJ, Riley JD, Hsu DA, et al. Striatal hypertrophy and its cognitive effects in new-onset benign epilepsy with centrotemporal spikes. Epilepsia 2012; 53:677.
  79. Taylor I, Berkovic SF, Kivity S, Scheffer IE. Benign occipital epilepsies of childhood: clinical features and genetics. Brain 2008; 131:2287.
  80. Covanis A. Panayiotopoulos syndrome: a benign childhood autonomic epilepsy frequently imitating encephalitis, syncope, migraine, sleep disorder, or gastroenteritis. Pediatrics 2006; 118:e1237.
  81. Lada C, Skiadas K, Theodorou V, et al. A study of 43 patients with panayiotopoulos syndrome, a common and benign childhood seizure susceptibility. Epilepsia 2003; 44:81.
  82. Koutroumanidis M. Panayiotopoulos syndrome: an important electroclinical example of benign childhood system epilepsy. Epilepsia 2007; 48:1044.
  83. Caraballo R, Cersósimo R, Fejerman N. Panayiotopoulos syndrome: a prospective study of 192 patients. Epilepsia 2007; 48:1054.
  84. Panayiotopoulos CP. Benign childhood epilepsy with occipital paroxysms: a 15-year prospective study. Ann Neurol 1989; 26:51.
  85. Du JC, Chien YH, Weng WC, et al. Clinical analysis of childhood occipital lobe epilepsy in 43 Taiwanese patients. Pediatr Neurol 2007; 36:387.
  86. Oguni H, Hayashi K, Funatsuka M, Osawa M. Study on early-onset benign occipital seizure susceptibility syndrome. Pediatr Neurol 2001; 25:312.
  87. Ferrie C, Caraballo R, Covanis A, et al. Panayiotopoulos syndrome: a consensus view. Dev Med Child Neurol 2006; 48:236.
  88. Panayiotopoulos CP. Benign childhood epileptic syndromes with occipital spikes: new classification proposed by the International League Against Epilepsy. J Child Neurol 2000; 15:548.
  89. Michael M, Tsatsou K, Ferrie CD. Panayiotopoulos syndrome: an important childhood autonomic epilepsy to be differentiated from occipital epilepsy and acute non-epileptic disorders. Brain Dev 2010; 32:4.
  90. Specchio N, Trivisano M, Di Ciommo V, et al. Panayiotopoulos syndrome: a clinical, EEG, and neuropsychological study of 93 consecutive patients. Epilepsia 2010; 51:2098.
  91. Ferrie CD, Caraballo R, Covanis A, et al. Autonomic status epilepticus in Panayiotopoulos syndrome and other childhood and adult epilepsies: a consensus view. Epilepsia 2007; 48:1165.
  92. Koutroumanidis M, Ferrie CD, Valeta T, et al. Syncope-like epileptic seizures in Panayiotopoulos syndrome. Neurology 2012; 79:463.
  93. Specchio N, Trivisano M, Claps D, et al. Documentation of autonomic seizures and autonomic status epilepticus with ictal EEG in Panayiotopoulos syndrome. Epilepsy Behav 2010; 19:383.
  94. Panayiotopoulos CP, Michael M, Sanders S, et al. Benign childhood focal epilepsies: assessment of established and newly recognized syndromes. Brain 2008; 131:2264.
  95. Ozkara C, Benbir G, Celik AF. Misdiagnosis due to gastrointestinal symptoms in an adolescent with probable autonomic status epilepticus and Panayiotopoulos syndrome. Epilepsy Behav 2009; 14:703.
  96. Graziosi A, Pellegrino N, Di Stefano V, et al. Misdiagnosis and pitfalls in Panayiotopoulos syndrome. Epilepsy Behav 2019; 98:124.
  97. García C, Rubio G. Efficacy and safety of levetiracetam in the treatment of Panayiotopoulos syndrome. Epilepsy Res 2009; 85:318.
  98. Yıldırım M, Bektaş Ö, Akıncı Göktaş Ö, et al. Levetiracetam monotherapy in children with epilepsy: Experience from a tertiary pediatric neurology center. Epilepsy Behav 2021; 116:107745.
  99. Hirano Y, Oguni H, Funatsuka M, et al. Neurobehavioral abnormalities may correlate with increased seizure burden in children with Panayiotopoulos syndrome. Pediatr Neurol 2009; 40:443.
  100. Caraballo RH, Cersósimo RO, Fejerman N. Childhood occipital epilepsy of Gastaut: a study of 33 patients. Epilepsia 2008; 49:288.
  101. Caraballo RH, Cersósimo RO, Fejerman N. Late-onset, "Gastaut type", childhood occipital epilepsy: an unusual evolution. Epileptic Disord 2005; 7:341.
  102. Kivity S, Ephraim T, Weitz R, Tamir A. Childhood epilepsy with occipital paroxysms: clinical variants in 134 patients. Epilepsia 2000; 41:1522.
  103. Tsai ML, Lo HY, Chaou WT. Clinical and electroencephalographic findings in early and late onset benign childhood epilepsy with occipital paroxysms. Brain Dev 2001; 23:401.
  104. Shahar E, Barak S. Favorable outcome of epileptic blindness in children. J Child Neurol 2003; 18:12.
  105. Caraballo R, Koutroumanidis M, Panayiotopoulos CP, Fejerman N. Idiopathic childhood occipital epilepsy of Gastaut: a review and differentiation from migraine and other epilepsies. J Child Neurol 2009; 24:1536.
  106. Gastaut H. A new type of epilepsy: benign partial epilepsy of childhood with occipital spike-waves. Clin Electroencephalogr 1982; 13:13.
  107. Martinović Z. Clinical correlations of electroencephalographic occipital epileptiform paroxysms in children. Seizure 2001; 10:379.
  108. Verrotti A, Parisi P, Loiacono G, et al. Levetiracetam monotherapy for childhood occipital epilepsy of gastaut. Acta Neurol Scand 2009; 120:342.
  109. Panayiotopoulos CP. Elementary visual hallucinations, blindness, and headache in idiopathic occipital epilepsy: differentiation from migraine. J Neurol Neurosurg Psychiatry 1999; 66:536.
Topic 6158 Version 39.0

References