Sleep Studies in Benign Epilepsy of Childhood with Rolandic Spikes. I. Sleep Pathology

Polygraphic all-night sleep records of 11 children with typical benign epilepsy of childhood with rolandic spikes (BERS) and those of eight nonepileptic controls were investigated with regard to sleep pathology. Basic properties of sleep organization and electromorphology were preserved in both groups. Seventeen quantitative sleep parameters were measured. Except for more waking in the BERS group, no sleep parameter showed significant differences between the BERS and the control group, but differences existed in comparison to normative values. We interpret our results as showing that in BERS epileptic neuronal malfunctioning does not affect sleep organization in a characteristic manner. The lack of detrimental interactions between epileptic neuronal behavior and pathological sleep alterations may be an additional cause of the benign course of BERS.     

Dipole Reversal: An Ictal Feature of Benign Partial Epilepsy with Centrotemporal Spikes

Benign epilepsy with centrotemporal spikes (BECTS) is characterized by brief stereotypical partial seizures with motor and/or sensory symptoms with frequent secondarily generalized seizures. The interictal EEG shows slow, disphasic, high-voltage spikes in the centrotemporal areas. The few published examples of ictal tracings depict focal rhythmic sharp waves and spikes without significant postictal slowing. We report an ictal event in BECTS that is unusual in the evolution and polarity of the ictal discharges. In this subclinical seizure, ictal multiple spike and wave discharges appear as a dipole: they are electropositive in T3-C3 and negative in F3. These surface positive epileptic discharges are unique and require explanation. We postulate that the seizure discharge arises in the depths of the sylvian fissure involving folded cortical areas. This occurs in such a way that the negative component of the discharges is concealed from the scalp electrodes. Thus, a relative positivity is recorded on the surface. This represents a dipole reversal relative to the interictal discharges (characteristic of BECTS). This geometrical explanation allows us to avoid postulating an unusual mechanism of generation of this seizure.     

Benign Childhood Epilepsy with Centrotemporal Spikes and Hippocampal Atrophy

A boy without significant family or personal history had three consecutive nocturnal seizures at 1-month intervals at age 10 years, all simple focal seizures with motor and sensory symptoms, the last with secondary generalization. Waking and napping EEG showed focal sharp changes typical of benign epilepsy with centrotemporal spikes (BECTS). A magnetic resonance imaging documented a marked right hippocampal atrophy (HA). After valproic acid (VPA) therapy, there were no more seizures, and there were fewer EEG changes. An EEG performed in the younger, fully asymptomatic 8-year-old sister documented unilateral right focal sharp waves. This case shows that HA, as well as other central nervous system lesions, can be found fortuitously in patients with BECTS.     

Benign Partial Epilepsy with Centrotemporal (or Rolandic) Spikes and Brain Lesion

We describe three patients with benign partial epilepsy with centrotemporal spikes (BECT) in association with proven brain lesion (agenesis of the corpus callosum, lipoma of the corpus callosum, and congenital toxoplasmosis, respectively). The age of onset, the ictal signs, the interictal electroencephalographic findings and the favorable outcome of epilepsy even after withdrawal of drug therapy led to the diagnosis of BECT although organic brain lesions were present. In such cases, the epilepsy should not be ascribed to the lesions but should be considered benign even though fortuitiously associated with brain lesions.     

Prolonged Intermittent Drooling and Oromotor Dyspraxia in Benign Childhood Epilepsy with Centrotemporal Spikes

Prolonged isolated sialorrhea of epileptic origin was described by Penfield and Jasper (1954) in a patient with a lesional epilepsy. A child with prolonged but intermittent drooling, lingual dyspraxia, and other clinical and electroencephalographic (EEG) features compatible with benign childhood epilepsy with centrotemporal spikes (BCECS) is described. The fluctuant course of the symptomatology and correlation with the intensity of the paroxysmal discharges on EEG are consistent with an epileptic dysfunction located in the lower rolandic fissure. No lesion was demonstrated by magnetic resonance imaging (MRI). Our case bears analogies with the recently reported status epilepticus of BCECS and the "acquired aphasia-epilepsy syndrome."     

儿童良性癫痫伴中央颞区棘波放电患者的GRIN2A突变筛查及遗传特征分析

目的对儿童良性癫痫伴中央颞区棘波放电(BECTS)患者进行GRIN2A基因突变筛查,分析其在BECTS患者中的致病性和遗传特征。方法收集2012年6月~2014年12月期间我院诊断的BECTS患者,采用PCR扩增和一代测序方法筛查GRIN2A基因突变。结果在收集的53例BECTS患者中,发现4例存在GRIN2A杂合突变,其中1例患者同时具有2个GRIN2A基因突变位点。突变包括4个错义突变(c.2627TC,p.I876T;c.1341TA,p.N447K;c.4322CA,p.T1441N;c.1271CT,p.P424L)和1个剪切位点突变(c.415-38CT),且均为未见报道的新突变位点。除c.1271CT为新生突变、c.1341TA未能验证突变来源外,其余3个突变均为遗传性突变。突变率为7.5%,外显率为75%。经保守性分析,发生错义突变的氨基酸变化位点均为高度保守。结论GRIN2A可能是BECTS的致病基因。GRIN2A突变存在不完全外显,临床进行遗传咨询时应引起高度重视。     

Incidence of Drug-Induced Aggravation in Benign Epilepsy with Centrotemporal Spikes

PURPOSE: Benign epilepsy with centrotemporal spikes (BECTS) is characterized by an excellent prognosis. Drug therapy is necessary in only a minority of patients. Carbamazepine (CBZ) and phenobarbital (PB) have been reported to cause electroclinical aggravation in some cases. The incidence of drug-induced aggravation in BECTS has never been established. METHODS: We retrospectively studied 98 consecutive cases of BECTS, examined at the Centre Saint Paul between 1984 and 1999; 82 patients had received one or more treatments, often successively and in association. RESULTS: We found only one case of electroclinical aggravation with CBZ among 40 patients exposed to CBZ (35 in monotherapy, five in polytherapy). An additional case showed a marked EEG aggravation on CBZ + PB among 14 patients taking PB (nine with monotherapy and five with polytherapy), and PB was apparently responsible. No patient treated with valproate or benzodiazepines showed aggravation. CONCLUSIONS: Aggravation of BECTS caused by antiepileptic drugs happens only rarely. There is a minor risk of aggravation with CBZ and also probably with PB. Drug-induced aggravation may occur only during certain periods coinciding with spontaneous worsening of BECTS.     

Sleep Studies in Benign Epilepsy of Childhood with Rolandic Spikes. II. Analysis of Discharge Frequency and Its Relation to Sleep Dynamics

Polygraphic all-night sleep recordings of 11 children with benign epilepsy of childhood with rolandic spikes (BERS) were investigated from the viewpoint of spike distribution. Spike/min ratios were calculated with regard to different sleep stages and waking, identical stages of the consecutive cycles, and stages 1-2 taking place on the descending versus the ascending slopes of sleep cycles. Maximum spike/min ratios were related to slow sleep stages, especially delta sleep, and in general to the first cycle. Stages 1-2 on the descending (DESC) slopes of consecutive cycles showed a decrease in spike density during the night, as opposed to the increasing rate of activation of the same stages on the ascending (ASC) slopes. Based on our results, we suggest that the actual drive of spiking as well as seizures in BERS is the functional overweight of sleep-inducing mechanisms.     

Benign Rolandic Epilepsy: High Central and Low Central Subgroups

Summary: The spikes in benign rolandic epilepsy (BRE) typically involve both the central and midtemporal regions as recorded on standard EEG montages, but the seizures are characterized by sensorimotor manifestations that are rarely referable to the temporal lobe. To study this apparent disparity, we evaluated the field distribution of interictal spikes in 33 BRE patients using closely spaced electrodes (AEEGS 1990) arranged over perisylvian cortex. None of the 33 patients showed maximum negativity in the midtemporal regions (T3/T4). Instead, maximum negativity was evident in the high central region (C3/C4) in 10 children (30.3%) and in the low central region (C5/C6) in 23 (69.7%). Hand involvement was significantly frequent (50%) in the high central group, and drooling with oromotor involvement was a distinctive symptom (65.2%) in the low central group. Our findings indicate that the spikes in patients with BRE are exclusively suprasylvian in origin and correlate with two electroclinical subgroups.     

Benign Familial Neonatal Convulsions Followed by Benign Epilepsy with Centrotemporal Spikes in Two Siblings

Summary: Purpose: To report on sibling cases with benign familial neonatal convulsions (BFNC) followed by benign epilepsy with centrotemporal spikes (BECT).
Methods: Case histories and EEGs were obtained for the two siblings with neonatal and subsequent epileptic seizures in one pedigree with BFNC.
Results: The family included six affected cases of BFNC in two generations: the proband, the proband's mother and two sisters, and the proband's maternal uncle and his daughter. The proband developed a generalized tonic convulsion 2 days after birth with no apparent cause and normal interictal EEG, and experienced a total of 18 episodes of tonic or clonic seizures or both by age 9 months. In the follow-up course, an EEG recording showed rolandic discharges at 2 years, and a sylvian seizure occurred at 4 years during sleep. On carbamazepine therapy, the last seizure was recorded at 9 years after a total of 11 episodes of sylvian seizures, with normal EEGs after 12 years. The proband's sister experienced nine episodes of brief tonic seizures between 7 and 9 days after birth, and also developed eight episodes of sylvian seizures from 4 to 7 years, with rolandic discharges on EEG until age 9 years. All of the family members had normal psychomotor development, with no neurologic sequelae.
Conclusions: This report of BFNC followed by BECT in sibling cases is significant in view of the genetic analysis and the classification of epilepsies and epileptic syndromes.     

Generalized Spike-Waves, Multiple Loci, and Clinical Course in Children with EEG Features of Benign Epilepsy of Childhood with Centrotemporal Spikes

In 41 patients with EEG features of benign epilepsy of childhood with centrotemporal spikes (BECCT), we noted associated generalized spike-wave discharges (GSWD) in 14.6% and multiple independent sharp wave foci in 9.8%. The presence or absence of these EEG features was not predictive of the clinical course. The high incidence of GSWD in children with BECCT suggests a possible relation in the expression of these two EEG traits.     

Benign Partial Epilepsy of Childhood with Monomorphic Sharp Waves in Centrotemporal and Other Locations

We reviewed EEGs from children whose history and clinical course was compatible with benign partial epilepsy of childhood with centrotemporal spikes. In 21% of patients with a single EEG focus, the discharge was outside the centrotemporal area. In 37.5% of patients with more than one focus, one was in the centrotemporal area while the other was not. We suggest that the typical EEG features of this syndrome are the normal background, the stereotypic morphology of the sharp waves, and their activation by drowsiness and sleep, not their exclusive location in the centrotemporal regions. Insistence on a centrotemporal location for the EEG discharges in this syndrome may lead to a misclassification of the type of epilepsy in some children with implications for therapeutic decisions and prognostic statements.     

Prognosis of Benign Childhood Epilepsy with Centrotemporal Spikes: A Foliow-Up Study of 168 Patients

A spontaneous and complete recovery of benign childhood epilepsy with centrotemporal or rolandic spikes (BECT) is taken for granted. However, some authors have reported the occurrence of generalized tonic-clonic seizures in a few adult patients and in some children who have seizures after a long period without problems. The aims of this study were (a) to search for early predictors of outcome and (b) to ascertain the long-term prognosis of BECT in a large group of patients. An attempt to relocate 268 patients born between 1941 and 1967 and consecutively seen as outpatients was undertaken. The outcome after age 20 is known for only 168. Being adults and cured, the others are no longer in touch with their clinics or have moved. Only one indicator of short-term prognosis was found: The earlier the onset of BECT, the longer the period with seizures. Of the 168 patients, 165 are seizure-free with follow-up ranging from 7 to 30 years. Three patients experienced generalized tonic-clonic seizures at age 18, 22-24, and 35. Two apparently had an isolated seizure. The occurrence of such seizures after recovery from BECT is a rare event (approximately 2% of cases) and a relapse with partial seizures is quite uncommon. These patients do not differ from patients remaining seizure-free.     

Antiepileptic Drug Treatment of Benign Childhood Epilepsy with Rolandic Spikes: Is It Necessary?

Ten untreated and 20 treated patients with benign childhood epilepsy with rolandic (centrotemporal) spikes (BERS) were studied retrospectively after recovery. Seizure frequency, seizure recurrence, and duration of active epilepsy were similar in both groups. No differences were found in social adjustment. Antiepileptic treatment may be unnecessary in most cases of BERS.     

Benign epilepsy with centrotemporal spikes

Benign epilepsy with centrotemporal spikes (BECTS) is a common disorder in childhood. After a brief overview of BECTS, a review of the data in favor of treatment with anticonvulsant medications is followed by the data indicating that treatment is not indicated. Some children appear to have cognitive consequences from BECTS. The parents and children with BECTS require a full discussion of the pros and cons of treatment, but based on data available at this time, it is concluded that treatment is generally not indicated for most patients. Future research may lead to changes in the recommendations.     

Electrophysiologic and Neuropsychologic Evaluation of Patients with Centrotemporal Spikes

The present study was designed to evaluate neurocognitive functions with endogenous potentials and neurophysiologic tests in patients with centrotemporal spikes who were not on any medication. Of the patients, 85.7% had seizures, 9.5% had pavor nocturnes, and 4.8% had atypical headache. The patients, especially who had atypical seizures or left-sided epileptic activity, were found to have significant visuomotor function impairment (p <.05). In P300 test, N2P3 amplitude was lower in the patients, particularly who had left sided epileptic activity (p <.05). MMN and LDN results were normal. Serial evaluations of such patients with endogenous potentials and neuropsychological tests may be helpful to show development of neurocognitive impairment.