A follow-up study of healthy children with epileptiform EEG discharges

Fifty-two healthy elementary schoolchildren with epileptiform EEGs who were not taking anticonvulsants were followed for an average period of 3 years and 3 months. Thirty-seven had centrotemporal spikes, three had occipital spikes, one had frontal spikes, nine had generalized spike-and-wave complexes, and two had a combination of multiple spike-and-wave complexes and focal spikes. Disappearance of epileptiform discharges was observed in 65% on waking EEGs and in 45% on sleep EEGs. After 5 years, the rate of disappearance was over 80% in waking and sleep EEGs. This study confirms that most epileptiform discharges in healthy children disappear spontaneously during childhood and that age-related development and disappearance occur in healthy children who would otherwise never be subjected to EEG examination. Epileptic seizures occurred in three cases (6%). This low percentage is not considered to be an indication for treating children with epileptiform discharges with anticonvulsants. However, the risk for seizure development may be higher than in the general population. According to the literature, children with multiple spike-wave complexes have a greater tendency to develop seizures. One girl with multiple spike-wave complexes and focal spikes developed generalized tonic-clonic convulsions. Two boys with centrotemporal spikes developed benign childhood epilepsy with centrotemporal spikes (BCECS). This study confirms that some (5%) of healthy children with centrotemporal spikes, the most common epileptiform pattern in healthy children, develop BCECS.     

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."     

Panayiotopoulos-type benign childhood occipital epilepsy: a prospective study

OBJECTIVE: To characterize the clinical and EEG features of the syndrome of benign childhood partial seizures with ictal vomiting and EEG occipital spikes (Panayiotopoulos syndrome [PS]). METHODS: Prospective study of children with normal general and neurologic examinations who had seizures with ictal vomiting and EEG with occipital spikes. RESULTS: From February 1990 to 1997, the authors found 66 patients with PS and 145 children with benign childhood epilepsy with centrotemporal spikes. Peak age at onset of PS was 5 years. Ictal deviation of the eyes and progression to generalized seizures were common. One-third had partial status epilepticus. During sleep, all had seizures. While awake, one-third also had seizures. Five children with PS had concurrent symptoms of rolandic epilepsy and another five developed rolandic seizures after remission of PS. Prognosis was excellent: one-third had a single seizure, one-half had two to five seizures, and only 4.5% had frequent seizures. CONCLUSIONS: Panayiotopoulos-type benign childhood occipital epilepsy is less common than benign childhood epilepsy with centrotemporal spikes but is well defined and recognizable by clinical and EEG features.     

Cognitive and behavioral problems in children with centrotemporal spikes

Atypical features in benign epilepsy of childhood with centrotemporal spikes (BECTS) are not uncommon. There are children with BECTS who do not have a benign outcome in terms of neuropsychologic functioning. BECTS have been linked with Landau-Kleffner syndrome (LKS) and continuous spikes and waves during slow sleep (CSWS). At the Medical College of Georgia from January 1988 to June 1999, 78 children, ages 2-16 years, were identified to have electroencephalogram evidence of centrotemporal spikes. Their medical records were reviewed for developmental history, behavioral problems, and school performance. Children with structural lesions/other epileptic syndromes were excluded. Fifty-six demonstrated a history of clinical seizures compatible with BECTS and 22 demonstrated centrotemporal spikes without clinical seizures. Among all children with centrotemporal spikes, 9% (n = 7) were diagnosed with mild intellectual disability (intelligence quotient < 70), 10% (n = 8) with borderline functioning, 31% (n = 24) with behavioral problems, and 17% (n = 13) with specific learning disabilities. Three children with BECTS experienced language delay and regression. Seizure control for BECTS usually is achieved without much difficulty, with excellent long-term prognosis. However, the data presented indicate that a large number of BECTS patients exhibit learning or behavior problems that require intervention. A small number may demonstrate language outcome similar to children with LKS and CSWS.     

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.     

22q11.2 Microduplication syndrome and epilepsy with continuous spikes and waves during sleep (CSWS). A case report and review of the literature

Chromosome 22q11.2 microduplication syndrome is characterized by a variable and usually mild phenotype and by incomplete penetrance. Neurological features of the syndrome may entail intellectual or learning disability, motor delay, and other neurodevelopmental disorders. However, seizures or abnormal EEG are reported in a few cases.We describe a 6-year-old girl with microduplication of chromosome 22q11.2 and epilepsy with continuous spikes and waves during sleep (CSWS). Her behavioral disorder, characterized by hyperactivity, impulsiveness, attention deficit, and aggressiveness, became progressively evident a few months after epilepsy onset, suggesting a link with the interictal epileptic activity characterizing CSWS.We hypothesize that, at least in some cases, the neurodevelopmental deficit seen in the 22q11.2 microduplication syndrome could be the consequence of a disorder of cerebral electrogenesis, suggesting the need for an EEG recording in affected individuals. Moreover, an array-CGH analysis should be performed in all individuals with cryptogenic epilepsy and CSWS.     

Phenobarbital-induced atypical absence seizure in benign childhood epilepsy with centrotemporal spikes.

This report describes atypical absence seizures induced by phenobarbital (PB) in a patient with benign childhood epilepsy with centrotemporal spikes. An 11-year-old girl had nocturnal motor seizures, beginning at the age of 7 years. Interictal EEG displayed centrotemporal sharp waves in the left and right sides alternatively, which increased significantly in frequency during sleep. She was given a diagnosis of benign childhood epilepsy with centrotemporal spike and was treated with valproic acid (VPA), which controlled her seizures for 3 years. She experienced nocturnal Rolandic seizures again, at the age of 10 years 2 months, 10 years 10 months in spite of the regular medication, and PB 60 mg daily was added to the VPA. Her parents noted de novo a few seconds of unresponsive fits 7 days after PB administration. Ictal EEG of the fits revealed diffuse irregular spike and wave bursts (1-4 seconds). VPA and PB in her blood was within the therapeutic ranges. After discontinuing PB, the atypical absence seizures immediately disappeared. Phenobarbital should be added to the list of drugs that potentially provoke de novo seizures.     

Somatosensory evoked spikes and epileptic seizures: a study of 385 cases.

We examined 385 children whose EEG showed high voltage potentials evoked by taps applied to one or both feet or hands (SES). The relationship between characteristics of SES and the occurrence of epileptic seizures and the characterization of epileptic syndromes were studied. Ninety-one children (23.6%) had epilepsy, 42 (10.9%) had only febrile convulsions and 252 children had other complaints. Epilepsy occurred in a higher proportion of cases when: SES by foot tapping were multiphasic, with high amplitude or SES were obtained by hand stimulation and there was spontaneous epileptiform activity in the EEG. The following epileptic syndromes were diagnosed: benign childhood epilepsy with centrotemporal spikes in 21 cases, benign epilepsy of childhood with occipital paroxysms in 2, benign psychomotor epilepsy in 1, "partial idiopathic others" in 43, generalized idiopathic in 8, symptomatic epilepsies in 13 and undetermined in 3 cases. In most cases SES were observed in children without evidence of cerebral organic lesion, suggesting the existence of an age-related, functional mechanism. Some characteristics of SES and the occurrence of spontaneous epileptiform activity showed a positive association with epileptic seizures. SES occurred in different types of partial and generalized epilepsies of childhood but in nearly 50% of the cases with epilepsy, there was a benign condition involving mainly the parietal lobe with versive, unilateral and sleep-generalized seizures.     

High-frequency oscillations in a spectrum of pediatric epilepsies characterized by sleep-activated spikes in scalp EEG

ObjectiveWe studied ripple-band (80–200 Hz) high-frequency oscillations in scalp electroencephalogram (EEG) in various pediatric epilepsies featuring sleep-activated spikes, such as epileptic encephalopathy with continuous spike-and-wave during sleep (CSWS) and investigated their characteristics.MethodsThe subjects were 94 children with epileptic disorders including idiopathic and non-idiopathic CSWS, benign epilepsy with centrotemporal spikes (BECTS), Panayiotopoulos syndrome, other types of focal epilepsies (oFE), and focal spikes without clinical seizures (Latent). We detected ripple oscillations using a semi-automatic detection tool based on localized power increase.ResultsIn the idiopathic CSWS Group, the median ratio of ripples per spike in the initial EEG was 5.73, which was significantly higher than those in the BECTS, Panayiotopoulos syndrome, oFE, and Latent Groups (0.39, 0.02, 0.35, 0, respectively, all with p < 0.01). Ripples were particularly frequent at younger ages.ConclusionsThis paper is the first to confirm a high ratio of ripples per spike in CSWS in the largest number of patients to date.SignificanceThe dense generation of ripples, which occurs through a combination of heavy loading of individual spikes with ripples and large numbers of spikes during sleep, characterizes CSWS and might be closely related to the pathophysiology of this epileptic encephalopathy.     

Girl with a PRRT2 mutation and infantile focal epilepsy with bilateral spikes

This paper documents the case of a female Japanese patient with infantile focal epilepsy, which was different from benign infantile seizures, and a family history of infantile convulsion and paroxysmal choreoathetosis. The patient developed partial seizures (e.g., psychomotor arrest) at age 14 months. At the time of onset, interictal electroencephalography (EEG) showed bilateral parietotemporal spikes, but the results of neurologic examination and brain magnetic resonance imaging were normal. Her seizures were well controlled with carbamazepine, and she had a normal developmental outcome. EEG abnormalities, however, persisted for more than 6 years, and the spikes moved transiently to the occipital area and began to resemble the rolandic spikes recognized in benign childhood epilepsy. Her father had paroxysmal kinesigenic dyskinesia, with an onset age of 6 years, and her youngest sister had typical benign infantile seizures. Genetic analysis demonstrated that all affected members had a heterozygous mutation of c.649_650insC in the proline-rich transmembrane protein-2 (PRRT2) gene. This case indicates that the phenotypic spectrum of infantile seizures or epilepsy with PRRT2-related pathology may be larger than previously expected, and that genetic investigation of the effect of PRRT2 mutations on idiopathic seizures or epilepsy in childhood may help elucidate the pathological backgrounds of benign childhood epilepsy.     

Hypnic Tonic Postural Seizures in Healthy Children Provide Evidence for a Partial Epileptic Syndrome of Frontal Lobe Origin

Summary: In the pediatric age period, three idiopathic partial epileptic syndromes are recognized: benign childhood epilepsy with centrotemporal spikes, childhood epilepsy with occipital paroxysms, and primary reading epilepsy. All other partial epilepsies are considered cryptogenic, and no other idiopathic partial epilepsies have been recognized. We observed 10 children with tonic partial postural seizures, mainly hypnic, coinciding with a recognizable ictal epileptiform EEG pattern. The children all had normal neuropsychological development before and after seizure onset. The seizures were tractable in all. Onset was in the early pediatric age period; no other type of seizure was recognized. The occurrence of a family history of epilepsy was high. The seizure pattern was typical of supplementary motor area origin. Because of the normal neuropsychological status, high familial incidence of epilepsy, and benign course of this partial epilepsy, we believe it should be considered idiopathic, using the criteria of the International Classification of Epilepsies and Epileptic Syndromes. We therefore suggest the existence of a partial idiopathic epileptic syndrome having onset with seizures of frontal lobe origin.     

Epilepsy in fragile X syndrome

Epilepsy is reported to occur in 10 to 20% of individuals with fragile X syndrome (FXS). A frequent seizure/EEG pattern in FXS appears to resemble that of benign focal epilepsy of childhood (BFEC, benign rolandic epilepsy). To evaluate seizure frequency and type in a Chicago FXS cohort, data regarding potential seizure history were reviewed for 136 individuals with FXS (age range 2 to 51 years: 113 males and 23 females). Seizures occurred in 15 males (13.3%) and one female (4.8%): of these, 12 had partial seizures. EEG findings were available for 35 individuals (13 of 16 with seizures and 22 of 120 without seizures) and showed an epileptiform abnormality in 10 (77%) individuals with seizures and five (23%) individuals without seizures--the most common epileptiform pattern being centrotemporal spikes. Seizures were easily controlled in 14 of the 16 individuals with seizures. Many individuals, including all with centrotemporal spikes, had remission of seizures in childhood. The most common seizure syndrome resembled BFEC and this pattern had the best prognosis for epilepsy remission. Deficiency of FMRP (fragile X mental retardation protein) appears to lead to increased neuronal excitability and susceptibility to epilepsy, but particularly seems to facilitate mechanisms leading to the BFEC pattern.     

Benign childhood epilepsy with centrotemporal spikes: clinical characteristics and identification of patients at risk for multiple seizures

This study aimed to characterize patients diagnosed as having benign childhood epilepsy with centrotemporal spikes and few seizures and compare them with patients with benign childhood epilepsy with centrotemporal spikes with multiple seizures. The medical files of 87 consecutive patients with benign childhood epilepsy with centrotemporal spikes were reviewed and data on gender, age at disease onset, duration of the disease, number of seizures, seizure semiology, and electroencephalographic and neuroimaging findings were analyzed. The mean age at disease onset was 8 years. The mean duration of the disease was 2 years. Eighteen percent of the patients had more than 20 seizures each, whereas 24% had 1 to 3 seizures each. The only predictor for a disease course with multiple seizures was an onset prior to 3 years of age.     

Atypical BECTS and homocystinuria

Reported is an association of atypical benign childhood epilepsy with centrotemporal spikes (BECTS) and homocystinuria in three apparently healthy children with borderline intelligence, two of whom had difficult-to-control seizures. In all three, EEG were suggestive of BECTS, although the clinical features were not. Homocystinuria could not be diagnosed for several years, pending metabolic evaluation.     

中央-颞部棘波的临床价值

目的 探讨脑电图出现的阵发性中央-颞部棘波的临床价值。方法 观察38例脑电图表现为中央-颞部局限性棘波且并非仅表现为中央沟发作患儿的临床发生经过及脑电图表现的变化。其中28例以局限性运动性发作为主,4例表现为不固定的单侧或双侧发作性头痛;2例于5岁前因发热而出现双侧肢体抽搐;余4例无神经系统病症。患儿首次接受脑电图检查的年龄为4～9岁,随访4～7年。结果38例患儿中,癫痫发作者的临床类型呈多种形式;而无癫痫发作病史者中,既有头痛、头晕主诉者,亦有无神经系统疾病病史者。所有患儿的脑电图均表现为不恒定,多次复查脑电图可出现不同部位的局限性慢波或尖波等内容。结论 中央-颞部局限性棘波并非伴中央-颞部棘波的儿童期良性癫痫的特异性脑电图表现。     

A case of parietal lobe epilepsy with ictal laughter

We report here about an 8-year-old boy with parietal lobe epilepsy (PLE) and ictal laughter. At the age of 6, he began to experience drop seizures, followed by sensory fits. Interictal EEG showed frequent spikes at C3, C4, P3 and Cz. Despite treatment with antiepileptic drugs, he often fell down in seizures after feeling abnormal sensations in the right shoulder. On ictal video EEG at the age of 7 years, (1) he became motionless and complained of fear and pain in the right hand, (2) he had clonic seizures of the right upper limb and fell down to his left, (3) he laughed though he did not feel funny. Ictal EEG showed spikes which originated in Pz and then were generalized. In many of the previously reported cases, ictal laughter is associated with hypothalamic hamartomas, infantile spasms,. complex partial seizures of frontal, temporal, or parietal origin. We diagnosed the present case as having PLE. However, other localization could not be roled out because the spikes were generalized quickly. To date, there are two reported cases of ictal laughter with PLE, but ictal EEG is lacking in these patients. Ictal laughter is rare in non-lesional cryptogenic PLE, but it may imply PLE's pathogenesis.     

Treatment of benign focal epilepsies in children: when and how should be treated?

Benign focal epilepsies represent almost one-fourth of all childhood epilepsies and are a frequent occurrence in clinical practice. They include benign infantile seizures (BIS), Panayiotopoulos syndrome (PS), and benign childhood epilepsy with centrotemporal spikes (BCECTS) in this order of the onset age. Because the prognosis is always excellent in patients with benign focal epilepsies, we must consider the risks and benefits of chronic antiepileptic drug (AED) administration. AED treatment is usually not recommended for the patients with a first attack, but should be considered for those with a second or third attack. A choice of AED has been based on the expert opinion. Carbamazepine (CBZ) is recommended for both acute and chronic treatment of seizure clusters in patients with BIS. Valproic acid (VPA), CBZ or clobazam (CLB) appears to be a first option of AED for patients with PS. A common first choice for BCECTS is CBZ in the USA and Japan, and VPA in the EU. The treatment period should be as short as possible without waiting for EEG normalization, possibly within 2 years after the initiation of AED. We must remember that some patients with BCECTS may have an “atypical evolution”. In conclusion, when and how to treat this benign condition should be determined in an individual manner based on the length and frequency of seizures, circadian rhythm of the attacks, interictal EEG findings, cognitive and behavioral functions in daily life and the attitude of the parents toward seizure recurrences and AED side effects.     

Magnetoencephalography localizing spike sources of atypical benign partial epilepsy

Rationale: Atypical benign partial epilepsy (ABPE) is characterized by centro-temporal electroencephalography (EEG) spikes, continuous spike and waves during sleep (CSWS), and multiple seizure types including epileptic negative myoclonus (ENM), but not tonic seizures. This study evaluated the localization of magnetoencephalography (MEG) spike sources (MEGSSs) to investigate the clinical features and mechanism underlying ABPE. Methods: We retrospectively analyzed seizure profiles, scalp video EEG (VEEG) and MEG in ABPE patients. Results: Eighteen ABPE patients were identified (nine girls and nine boys). Seizure onset ranged from 1.3 to 8.8 years (median, 2.9 years). Initial seizures consisted of focal motor seizures (15 patients) and absences/atypical absences (3). Seventeen patients had multiple seizure types including drop attacks (16), focal motor seizures (16), ENM (14), absences/atypical absences (11) and focal myoclonic seizures (10). VEEG showed centro-temporal spikes and CSWS in all patients. Magnetic resonance imaging (MRI) was reported as normal in all patients. MEGSSs were localized over the following regions: both Rolandic and sylvian (8), peri-sylvian (5), peri-Rolandic (4), parieto-occipital (1), bilateral (10) and unilateral (8). All patients were on more than two antiepileptic medications. ENM and absences/atypical absences were controlled in 14 patients treated with adjunctive ethosuximide. Conclusion: MEG localized the source of centro-temporal spikes and CSWS in the Rolandic-sylvian regions. Centro-temporal spikes, Rolandic-sylvian spike sources and focal motor seizures are evidence that ABPE presents with Rolandic-sylvian onset seizures. ABPE is therefore a unique, age-related and localization-related epilepsy with a Rolandic-sylvian epileptic focus plus possible thalamo-cortical epileptic networks in the developing brain of children.     

A case of temporal lobe epilepsy with improvement of clinical symptoms and single photon emission computed tomography findings after treatment with clonazepam

A 26-year-old female presented psychomotor seizures, deja vu and amnestic syndrome after meningitis at the age of 14 years. Repeated electroencephalograms (EEG) demonstrated occasional spikes localized in the right temporal region in addition to a considerable amount of theta waves mainly in the right fronto-temporal region. Single photon emission computed tomography (SPECT) showed a marked hypoperfusion corresponding to the region in which the EEG showed abnormal findings, although magnetic resonance imaging (MRI) demonstrated no abnormal findings associated with the clinical features. Treatment with clonazepam in addition to sodium valproate resulted in a remarkable improvement of clinical symptoms (i.e. psychomotor seizures and deja vu), as well as of the EEG and SPECT findings. The present study suggests that SPECT is a useful method not only to determine the localization of regions associated with temporal lobe epilepsy but also to evaluate the effect of treatment in temporal lobe epilepsy.     

Epileptic encephalopathy of late childhood: Landau-Kleffner syndrome and the syndrome of continuous spikes and waves during slow-wave sleep.

Landau-Kleffner syndrome (LKS) and the syndrome of continuous spikes and waves during slow wave sleep (CSWS) are two points on the spectrum of functional childhood epileptic encephalopathies. They are characterized by a severe paroxysmal EEG disturbance that may permanently alter the critical synaptogenesis by strengthening synaptic contacts that should have been naturally "pruned." The much more common benign epilepsy with centrotemporal spikes is also related to LKS and CSWS by a common pathophysiology. Although prognosis in LKS and CSWS for seizure control is good, cognitive function declines and permanent neuropsychologic dysfunction is seen in many cases. This permanent damage is most evident in those patients who had early-onset EEG abnormality and a prolonged active phase of continuous spike-and-wave discharges during sleep. If the active phase of paroxysmal activity persists for over 2 to 3 years, even successful treatment does not resolve neuropsychologic sequelae. In LKS, the paroxysmal activity permanently affects the posterior temporal area and results in auditory agnosia and language deficits; in CSWS, the frontal lobes are more involved and other cognitive disturbances predominate. Aggressive treatment should include high-dose antiepileptic drugs, corticosteroids, and surgery in specific cases.