Benign childhood epilepsy with centrotemporal spikes
by Jerome Engel and Natalio Fejerman
October 1, 1993
Date of update: November 4, 1998
Date of update: January 2003
Date of update: March 2004
Medline SEARCH DATE: March 2004

ACKNOWLEDGEMENTS AND DISCLOSURES

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HISTORICAL NOTE AND NOMENCLATURE

A particular EEG pattern with migratory spikes originating over the rolandic (centrotemporal or midtemporal) region was first reported in the 1950s (Gastaut 1952; Gibbs et al 1954). In 1958 the first description of clinical features in association with the peculiarities of the EEG in those children was published (Nayrac and Beaussart 1958). The same EEG pattern was later correlated with a common form of focal childhood epilepsy, then called "midtemporal epilepsy," characterized by hemifacial and oropharyngeal ictal symptoms and a favorable prognosis (Gibbs and Gibbs 1960). Because of the localization of the ictal events, Lombroso proposed the term "sylvian seizures" (Lombroso 1967). In the same year, Loiseau and colleagues presented a series of 122 children with what they called “a particular form of epilepsy in childhood,” stressing its benign character and highlighting clinical and EEG features. Several long term follow-up studies confirmed the good prognosis (Beaussart 1972; Lerman and Kivity 1975; Beaussart and Faou 1978; Loiseau et al 1988). Atypical and not-so-benign evolutions have been reported in some patients with this form of epilepsy (Aicardi and Chevrie 1982; Fejerman and Di Blasi 1987; Fejerman 1996; Fejerman et al 2000; Hahn et al 2001; Fejerman 2002). This form of epilepsy is now called benign childhood epilepsy with centrotemporal spikes and is placed in the group of idiopathic localization-related (focal, local, partial) epilepsies in the International Classification of Epilepsies and Epileptic Syndromes (Commission on Classification and Terminology of the International League Against Epilepsy 1989; Engel 2001).

CLINICAL MANIFESTATIONS

Benign childhood epilepsy with centrotemporal spikes begins between 2 and 13 years of age. In 80% of patients, seizures appear between 5 and 10 years of age. Patients usually recover before age 16 (Beaussart 1972).
Most of the seizures reflect discharges in the precentral and postcentral gyri in the suprasylvian region with motor, sensory, and autonomic manifestations in the face, mouth, and throat (Loiseau and Beaussart 1972). This is associated with independently bilateral, repetitive, broad, centrotemporal interictal EEG spikes displaying a characteristic transverse dipolar pattern (Gregory and Wong 1984).
Loiseau and Duche provided five criteria for the diagnosis of benign childhood epilepsy with centrotemporal spikes: (1) onset between the ages of 2 and 13; (2) absence of neurologic or intellectual deficit before the onset; (3) partial seizures with motor signs, frequently associated with somatosensory symptoms or precipitated by sleep; (4) a spike focus located in the centrotemporal (rolandic) area with normal background activity on the interictal EEG; and (5) spontaneous remission during adolescence (Loiseau and Duche 1989).
The oropharyngeal and facial manifestations include drooling from hypersalivation and swallowing disturbance; guttural sounds; involuntary movements or tonic contractions of the tongue or jaw; unilateral numbness or paresthesia of the tongue, lips, gums, and cheek; speech arrest; and myoclonic contraction of one side of the face. Sensorimotor phenomena involving a leg, or half body, and miscellaneous symptoms such as abdominal pain, can also occur (Loiseau and Beaussart 1972).
Although partial seizures are characteristic of this disorder, generalized seizures are not infrequently observed, particularly in younger children (Luders et al 1987; Lerman 1998). The initial event is often a nocturnal hemifacial convulsion, which may spread to the arm and the leg or may become secondarily generalized. The ictal patterns vary from child to child; and, in some patients, from seizure to seizure. Each individual patient usually has a single type of seizure, but 20% to 25% of children experience more than one type (Loiseau and Duche 1989).
More than one-half of patients with benign childhood epilepsy with centrotemporal spikes have seizures only during sleep, whether during the day or the night. Seizures during waking hours are more likely to occur shortly after awakening (Luders et al 1987). Seizure frequency is usually low and around 10% of cases present only one seizure. However, in about 20% of children, seizures are frequent and may even occur several times per day (Dalla Bernardina et al 2002). It has been stated that the only predictor for a disease course in children with multiple seizures is an onset before three years of age (Kramer et al 2002).
Behavioral problems are less frequent than in other forms of childhood epilepsy (Heijbel and Bohman 1975). In a study of 40 children with centrotemporal spikes with and without seizures compared with 40 healthy controls, patients were significantly impaired in IQ, visual perception, short-term memory, and psychiatric status. The deficits in IQ were more correlated with frequency of spikes in the EEG than with the frequency of seizures (Weglage et al 1997). Similar findings were reported in 19 children with this syndrome (Deonna et al 2000). Another interesting finding is that in children with “attention deficit hyperactivity disorder,” an increased frequency of Rolandic spikes was found (Holtmann et al 2003). A longitudinal study of 1 boy with acquired epileptic dysgraphia was reported. Most probably, in this case, the acquired regression of graphomotor skills was associated with an increase in spike frequency as happens in the cases with atypical evolutions of this syndrome (Dubois et al 2003).
Atypical features in benign childhood epilepsy with centrotemporal spikes can be seen on clinical grounds (daytime-only seizures, post-ictal Todd paresis, prolonged seizures, or even status epilepticus), or in EEG features (atypical spike morphology, unusual location, or abnormal background). In a retrospective case series, atypical clinical features were seen in 50% of patients and atypical electrographic features in 31% (Wirrell et al 1995). More recently a follow-up study of 74 children with typical rolandic epilepsy and 14 with atypical features was reported and a significant higher percentage of learning and behavioral disabilities was found in the second group (Verrotti et al 2002).
Several cases of partial status epilepticus in this condition have been reported. The manifestations include hemifacial seizures, dysarthria or anarthria, and persistent drooling (Fejerman and Di Blasi 1987; Roulet et al 1989; Colamaria et al 1991; Fejerman et al 2000; Kramer et al 2001; Gregory et al 2002; Salas-Puig et al 2002).

CLINICAL VIGNETTE

No information was provided by the author.

ETIOLOGY

The characteristic centrotemporal EEG spike pattern in benign childhood epilepsy is inherited as an autosomal-dominant trait with variable penetrance (Heijbel et al 1975). This type of inheritance was also suggested by studies of monozygotic twins with rolandic discharges (Kajitani et al 1980), and HLA antigens and their haplotypes (Eeg-Olofsson 1992). However, in another study of clinical and genetic aspects in children with benign focal sharp waves, including 134 probands with seizures (24% of which had typical rolandic seizures), the findings were in agreement with a multifactorial pathogenesis of epilepsies with “benign” focal epileptiform sharp waves (Doose et al 1997). Epileptic seizures appear in only 25% or less of individuals with this EEG trait (Luders et al 1987). Expression of the gene may be influenced by other genetic and environmental factors (Loiseau and Duche 1989).
A family with nine affected individuals in three generations was reported showing the features of rolandic epilepsy associated with oral and speech dyspraxia and cognitive impairment (Scheffer et al 1995).
Linkage to chromosome 15q14 was found in 54 patients of 22 families with benign childhood epilepsy with centrotemporal spikes (Neubauer et al 1998). However, in a study of 70 families with the same syndrome in Italy, the mentioned linkage could not be found (Pruna et al 2000). A similar seizures and EEG phenotype of benign childhood epilepsy with centrotemporal spikes was found in 3 children with “de novo” terminal deletions of the long arm of chromosome 1q and the authors suggested that it could be a potential site for a candidate gene (Vaughn et al 1996).

PATHOGENESIS AND PATHOPHYSIOLOGY

Although the pathophysiology of benign childhood epilepsy with centrotemporal spikes is unknown, and there is no associated structural lesion, the typical focal ictal clinical behavior and EEG discharge indicate a disturbance in the sylvian and rolandic areas. Electrophysiologic studies, however, fail to demonstrate a discrete generator, and a large, shifting area of dysfunction may be present. In some patients with benign childhood epilepsy with centrotemporal spikes, the occurrence of generalized spike-wave EEG discharges, as well as focal spikes in other areas, suggests a relationship between this disorder and the idiopathic generalized epilepsies, as well as with other idiopathic localization-related partial epilepsies (Luders et al 1987; Lerman and Kivity 1991). Ten percent to 20% of patients with centrotemporal spikes may also have sharp slow wave complexes in other cortical locations (Panayiotopoulos 1999).
The cornerstone of the diagnosis of benign childhood epilepsy with centrotemporal spikes lies in the characteristic interictal EEG pattern: centrotemporal spikes on normal background activity. The centrotemporal spikes are typically seen independently on both sides of the head. They are broad, diphasic, high-voltage (100-microvolts to 300-microvolts) spikes, with a transverse dipole, and they are often followed by a slow wave. The spikes may occur isolated or in clusters, with a rhythm of about 1.5 Hz to 3 Hz (Loiseau and Duche 1989). Focal rhythmic slow activity is occasionally observed in the region where the spikes are seen (Mitsudome et al 1997b). Generalized 3 Hz spike-and-waves and focal spikes in other brain areas can also be seen in a minority of children (Beydoun et al 1992).
The centrotemporal spikes are not enhanced by eye opening or closure, by hyperventilation, or by photic stimulation. Even more, it has been reported that hyperventilation reduces the frequency of Rolandic spikes (Watanabe 2004). The discharge rate is increased in drowsiness and in all stages of sleep, and in about one third of children, the spikes appear only in sleep (Lombroso 1967). The sleep EEG organization is preserved (Dalla Bernardina and Beghini 1976). There is no correlation between intensity of spike discharges in the EEG and frequency, length, or duration of clinical seizures (Lerman and Kivity 1975).
The ictal EEG discharge begins focally in the centrotemporal area and then spreads to adjacent areas or generalizes. Ictal onset may shift from side to side (Gibbs et al 1954; Dalla Bernardina 1975).
Several authors emphasized the characteristic dipolar pattern in the EEG (Gregory and Wong 1984; Lischka and Graf 1992; Tsai and Hung 1998). Two groups of patients have been disclosed according to EEG findings (maximal negativity was registered in high- and low-central regions, but never in midtemporal regions), a high-central region group with more frequent hand involvement and the low-central group with common orofacial symptoms.
Combined recording of interictal spikes and somatosensory-evoked potentials concluded that in some patients multiple simultaneous neuronal populations are active within the central region (Baumgartner et al 1996).
Magnetoencephalographic analysis of generator and propagation of rolandic discharges in benign childhood epilepsy with centrotemporal spikes with neuromagnetic three-dimensional dipole localization suggested that rolandic discharges are generated through a mechanism similar to that of somatosensory-evoked responses (Minami et al 1996). A localization analysis of spontaneous magnetic brain activities also suggested the value of magnetoencephalography for pathophysiological elucidation (Kamada et al 1998). Six children with bilateral centrotemporal synchronous discharges were studied using magnetoencephalography and EEG with equivalent current dipole modelling. Results implied cortical epileptogenicity in bilateral perirolandic areas (Lin et al 2003). Interictal spikes were recorded during fMRI acquisition in a MR-compatible digital EEG system in 7 children, and the spike-related activation in the perisylvian central region was found in 3 of them (Boor et al 2003).

EPIDEMIOLOGY

Benign childhood epilepsy with centrotemporal spikes accounts for about 24% of all epileptic seizures in children between ages 5 and 14 (Cavazzuti 1980). Its annual incidence has been reported to be between 7.1 and 21 per 100,000 in children under age 15 (Heijbel et al 1975). 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 (Luders et al 1987).
The prevalence of epilepsy is much higher among close relatives of children with benign childhood epilepsy with centrotemporal spikes than in a matched control group (Bray and Wiser 1964). In 1 study, 15% of siblings had seizures and rolandic spikes, 19% of siblings had rolandic spikes without attacks, and 11% of the parents had childhood seizures that had disappeared by adulthood (Heijbel et al 1975).
In an epidemiological study of epilepsy in childhood with a cohort of 440 consecutive patients, excluding only neonatal seizures from the analysis, benign rolandic epilepsy of childhood accounted for 8% of patients (Kramer et al 1998).

PREVENTION

No information is available.

DIFFERENTIAL DIAGNOSIS

The presence of the characteristic centrotemporal spikes alone is not diagnostic of epilepsy. Within the susceptible age range, more children will exhibit the characteristic EEG spike pattern without seizures than with seizures. Furthermore, centrotemporal spikes with other morphologic features can be seen in nonepileptic children with diffuse brain disturbances such as cerebral palsy (Perlstein et al 1947) and Rett syndrome (Robertson et al 1988). Distinction between benign childhood epilepsy with centrotemporal spikes and more serious nonidiopathic epileptic conditions, 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 benign childhood epilepsy with centrotemporal spikes when nocturnal convulsions are the presenting complaint, and a generalized epileptic syndrome is initially suspected.
Because of their prevalence, fortuitous associations may be found between benign childhood epilepsy with centrotemporal spikes and nonevolutive brain lesions (Santanelli et al 1989). Isolated cases of children with this epileptic syndrome and unilateral opercular neuronal migration disorders have been published (Ambrosetto 1992; Fejerman 1996; Sheth et al 1997). Cerebral tumors presenting as pseudo-benign partial epilepsy in childhood with centrotemporal spikes were reported in 5 patients (Shevell et al 1996). More recently, 5 children with a so-called “malignant rolandic-sylvian epilepsy” secondary to neuronal migration disorders and gliosis were reported as presenting similar clinical and EEG features of benign childhood epilepsy with centrotemporal spikes. The authors emphasized the role of magnetoencephalography in the differential diagnosis (Otsubo et al 2001).
The pathophysiologic relationships that may exist between benign childhood epilepsy with centrotemporal spikes and other benign partial nonrolandic epilepsies can make differential diagnosis difficult. The coexistence of 2 types of benign partial epilepsies in children has been reported, either presenting in sequence one after the other or at the same time (Panayiotopoulos 1993; Caraballo et al 1998; Covanis et al 2003), although treatment and prognosis are the same (Lerman and Kivity 1991).

DIAGNOSTIC WORKUP

When the clinical and EEG features are typical, the diagnosis is certain (Lerman 1998). But because of the few cases that have presented the clinical and EEG phenotype of benign partial epilepsy with centrotemporal spikes in whom cortical dysplasia (Ambrosetto 1992; Fejerman 1996; Sheth et al 1997) and even brain tumors (Shevell et al 1996) were found, one might question whether an MRI study is indicated. In 10 of 71 consecutive patients with this syndrome studied with CT or MRI, neuroimaging abnormalities were found (Gelisse et al 2003).
Positron emission tomography might be helpful to distinguish benign partial epilepsy with centrotemporal spikes from symptomatic cases of partial epilepsy in children because regional glucose metabolism is normal in patients with the former (Van Bogaert et al 1998).

PROGNOSIS AND COMPLICATIONS

In general, benign childhood epilepsy with centrotemporal spikes is associated with excellent prognosis. Seizures are difficult to control in only a small number of cases (Beaussart and Faou 1978; Blom and Heijbel 1982). The prognosis is favorable even for those whose seizures are difficult to control, and seizures almost always remit spontaneously in late adolescence. In their investigation of 168 patients 7 to 30 years after cessation of epilepsy with centrotemporal spikes, Loiseau and colleagues reported that seizures occurred in only three cases after adulthood (Loiseau et al 1988). The seizure types were all generalized tonic-clonic seizures. Two of the three had obviously isolated incidences. This incidence of generalized seizures in adults with a history of epilepsy with centrotemporal spikes in childhood is nevertheless higher than that of seizures in the general population (Loiseau et al 1988). Cognitive functions were evaluated in 23 adolescents and young adults in complete remission from benign childhood epilepsy with centrotemporal spikes showing no significant differences with controls. However, qualitative analysis suggested a different organizational pattern for cerebral language in adolescents and young adults in remission from this syndrome (Hommet et al 2001).
The presence of atypical interictal epileptiform EEG patterns does not appear to alter prognosis (Beydoun et al 1992). However, atypical evolutions may cause doubt about prognosis. For example, in the cases of benign atypical partial epilepsy described by Aicardi and Chevrie (Aicardi and Chevrie 1982), the children showed partial or generalized atonic fits leading to multiple daily falls. These inhibitory attacks appear in clusters that last for weeks, and the EEG shows continuous spikes-and-waves during slow sleep. Status lasting days or weeks including motor facial seizures and anarthria with persistent drooling constitute another complication of this syndrome (Fejerman and Di Blasi 1987; Fejerman et al 2000). Both complications have shown an ultimate good prognosis. However, acquired epileptic aphasia and the syndrome of continuous spikes-and-waves during slow sleep have also been associated with the syndrome of benign partial epilepsy with centrotemporal spikes, and in this cases the risk of permanent language dysfunction or neuropsychologic involvement is clearly present (Fejerman 1996; Fejerman et al 2000). EEG activity in this atypical evolution seems to be a kind of bilateral secondary synchrony, but the reasons why some children develop this EEG pattern are yet not understood. In some cases, certain antiepileptic drugs seemed to be responsible (Shields and Saslow 1983; Caraballo et al 1998; Prats et al 1998). A meta-analysis of the course of patients with benign partial epilepsy with centrotemporal spikes, based on 794 patients in 13 cohorts, concluded that the early prediction of seizure outcome in the new patient cannot be given with certainty (Bouma et al 1997). In a small group of patients with status epilepticus in benign childhood epilepsy with centrotemporal spikes the finding of independent right and left seizures was considered a risk factor (Gregory et al 2002).

MANAGEMENT

Benign childhood epilepsy with centrotemporal spikes is an age-related syndrome that almost always disappears by adulthood regardless of age at onset; therefore, excessive restriction of activities or overprotection of affected children is not advised (Loiseau and Duche 1989).
Drug therapy is necessary only in about 30% of patients. Monotherapy should be used whenever possible. Carbamazepine and valproate were always the drugs of choice. However the use of benzodiazepines at night may be considered in those children who only have seizures during sleep (Fejerman 2002). The start of antiepileptic treatment can be deferred until a second seizure occurs. Partial seizures, a short interval between the first and second attack, and an early onset, usually indicate the necessity of treatment. A subset of patients requiring more than 1 medication for seizure control showed more seizures prior to initiation of treatment and tended to have a higher frequency of tics, attention deficit hyper activity disorder, and learning disabilities (Al-Twajri and Shevell 2002). Because the seizures will almost invariably disappear in adolescence, therapy beyond that period is of little value. Relapse of seizures, however, may occur after premature withdrawal (Loiseau and Duche 1989).
In a prospective study of treatment in childhood epilepsy, it was concluded that 1 year of treatment can be recommended in children with benign childhood epilepsy with centrotemporal spikes (Braathen et al 1996). Benzodiazepine treatment for several weeks was also recommended (De Negri et al 1997). In comparison with valproate and carbamazepine, clonazepam showed to be more efficient in making rolandic discharges disappear after four weeks of treatment (Mitsudome et al 1997a). Sulthiame was recommended in several reports (Doose et al 1988; Lerman and Lerman-Sagie 1995; Lerman 1998). In a double blind, placebo controlled study of 66 children with benign childhood epilepsy with centrotemporal spikes, Sulthiame was found to be remarkably effective in preventing seizures and well tolerated (Rating et al 2000). A more recent report also shows the benefits of Sulthiame (Engler et al 2003). In fact, it seems to us to be the drug of choice in patients presenting atypical evolutions associated to secondary bilateral synchronies in the EEG (Fejerman et al 2000; Fejerman 2002).
Curiously enough, through an ictal clinical and EEG study in 1 child, it was suggested that voluntary protrusion of the tongue could stop seizures and EEG discharges (Veggiotti et al 1999).

PREGNANCY

No information is available.

ANESTHESIA

No information is available.

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ILAE.

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Abbreviations

EEG:electroencephalogram
HLA:human leukocyte antigen

ICD Code

345.5

SYNONYMS

Sylvian epilepsy
Midtemporal epilepsy

ASSOCIATED DISORDERS

Idiopathic generalized seizures

MAJOR KEYWORD DESCRIPTORS

anarthria
centrotemporal interictal EEG spikes
chromosome 15q14
dysarthria
dysphasia
hemifacial symptoms
idiopathic localization-related epilepsies
involuntary movements
midtemporal spikes
migratory spikes
mixed seizures
motor seizures
myoclonus
nocturnal seizures
oropharyngeal ictal symptoms
partial seizures
persistent drooling
rolandic discharges
rolandic seizures
sharp slow wave complexes
status epilepticus
sylvian seizures

MINOR KEYWORD DESCRIPTORS

convulsions
epilepsy
seizures
sleep
spikes

AGE OF PRESENTATION

02-05 years
06-12 years
13-18 years

AGE OF TYPICAL PRESENTATION

06-12 years

POPULATION GROUP(S) PREFERENTIALLY AFFECTED

none selectively affected

OCCUPATION GROUP(S) PREFERENTIALLY AFFECTED

none selectively affected

SEX

male>female, >1:1

FAMILY HISTORY

family history may be obtained
family history typical

HEREDITY

heredity may be a factor
autosomal dominant

GLOSSARY

Benign childhood epilepsy with centrotemporal spikes: an idiopathic localization-related epilepsy characterized by childhood onset and centrotemporal spikes on EEG.

PERMUTED TOPIC, SYNONYMS, SUBTOPICS

Benign childhood epilepsy with centrotemporal spikes
childhood epilepsy with centrotemporal spikes, Benign
epilepsy with centrotemporal spikes, Benign childhood
centrotemporal spikes, Benign childhood epilepsy with
spikes, Benign childhood epilepsy with centrotemporal
epilepsy, Midtemporal
epilepsy, Sylvian

RELATED TOPICS

Benign epilepsy of infancy with partial seizures
Early onset benign childhood seizures with occipital spikes
Epilepsy
Epilepsy with grand mal seizures on awakening
Parasomnias
Sleep disorders
Sleep disorders associated with epilepsy

DIFFERENTIAL DIAGNOSIS

cerebral palsy
Rett syndrome
nonidiopathic epileptic conditions
mesial temporal lobe epilepsy
nonevolutive brain lesions
cerebral tumors
malignant rolandic-sylvian epilepsy
neuronal migration disorders
gliosis
benign partial nonrolandic epilepsies

 

 

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