Oxcarbazepine and atypical evolution of benign idiopathic focal epilepsy of childhood

Patients that have benign epilepsy with centrotemporal spikes (BECTS) may occasionally experience an atypical development in their course when treated with drugs such as carbamazepine. Three patients with electroclinical patterns consistent with BECTS showed seizure exacerbation during oxacarbazepine (OXC) therapy. Two manifested atypical absences, neuropsychological disturbances, and generalized spike-and-wave discharges in their electroencephalograms (EEGs) that became continuous during sleep. The third patient showed, during OXC therapy, more frequent partial motor seizures which ended with ictal vomiting and post-ictal obnubilation. EEGs recorded during sleep showed discontinuous paroxysmal activity in the right centrotemporal area. Symptoms were reversed following discontinuation of the OXC therapy. Although electroclinical findings were consistent with a BECTS diagnosis, all patients had some atypical features. Our observations show that BECTS patients, in particular those presenting with atypical findings, might be at risk for developing paradoxical reactions to OXC therapy. We suggest that OXC should be included in the list of drugs that may cause electroclinical deterioration in these patients.     

Paradoxic reaction to lamotrigine in a child with benign focal epilepsy of childhood with centrotemporal spikes

A girl aged 5 years developed nocturnal seizures (mouth twitching, salivation, anarthria, with right arm jerking and occasional secondary generalisation), with frequent focal sharp waves over the left centrotemporal region in her EEG, suggesting benign childhood epilepsy with centrotemporal spikes (BECTS). Seizures became diurnal and frequent, not modified by carbamazepine (CBZ) or valproate (VPA) but responding to VPA and lamotrigine (LTG) with recommended dosage schedules for this combination. Her school performance then deteriorated insidiously, with poor memory and concentration, clumsiness, stuttering, and emotional lability. After 4 months, new episodes, < or =10 per day, occurred. These lasted a few seconds; she stared into space, her jaw dropped, her head dropped to the right, and her eyelids flickered. She usually maintained awareness. Attacks were often provoked by blowing or sneezing. Ictal EEG showed anterior-predominant 3/s sharp-slow wave complexes lasting < or =8 s, with bilateral rolandic discharges interictally. Withdrawal of LTG resulted in rapid improvement in cognitive function and gradual remission of the new attacks. CONCLUSIONS: This appears to be a paradoxic reaction to LTG in the setting of BECTS.     

Correlation between scalp high-frequency oscillations and prognosis in patients with benign epilepsy of childhood with centrotemporal spikes

Aims

The study aimed to explore whether high-frequency oscillations (HFOs) can predict seizure risk and atypical manifestations of benign epilepsy of childhood with centrotemporal spikes (BECTS).

Methods

We recruited 60 patients and divided them into three groups: (1) seizure-free BECTS, (2) active typical BECTS, and (3) active atypical forms of BECTS. Electroencephalogram was used to record the number, location, average amplitude, and duration of spikes, and spike ripples were analyzed using time-frequency technology. Multivariable logistic regression analysis was used to investigate independent predictive factors for prognosis.

Results

The number of sleep spike ripples, rather than spikes, was an independent risk factor for the active period of the disease (odds ratio [OR] = 4.714, p = 0.003) and atypical forms of BECTS (OR = 1.455, p = 0.049); the optimal thresholds for the spike ripple rate were >0 (area under the curve [AUC] = 0.885, sensitivity = 96.15%, specificity = 73.33%) and >0.6/min (AUC = 0.936, sensitivity = 84.21%, specificity = 96.15%), respectively. Furthermore, in typical BECTS, the spike ripple rate showed significant negative correlations with time since the last seizure (ρ = −0.409, p = 0.009) and age (ρ = −0.379, p = 0.016), while the spike rate did not.

Conclusion

Spike ripple was a marker for distinguishing typical and atypical forms of BECTS and reflected the risk of seizure recurrence better than the spike alone. The present findings might assist clinicians in BECTS treatment.     

Benign epilepsy of childhood with centrotemporal spikes and unilateral developmental opercular dysplasia

The case of a 15-year-old right-handed girl with developmental delay, mild retardation, astereognosis, and tactile discrimination impairment in the left arm, is reported. During sleep, right partial simple motor seizures with sialorrhea and saliva pooling were present, sometimes followed by secondary generalization. Absences and drop attacks were also observed. Electroencephalography revealed left centrotemporal spike waves spreading to the homologous contralateral region, generalized 3-Hz spike-and-wave complexes enhanced by hyperventilation, and generalized polyspike-and-wave discharges during drowsiness. Magnetic resonance imaging showed right perisylvian (opercular) malformation. Benign epilepsy of childhood with centrotemporal spikes (BECCTS) is a focal idiopathic epilepsy presumed to be of genetic origin. Although brain damage is not expected, structural lesions including opercular macrogyria have been reported. This coexistence has been considered mainly casual and only exceptionally causal. The Foix-Chavany-Marie syndrome or operculum syndrome is the result of bilateral opercular damage. In our child the right structural and the left functional lesions appeared mutually activated, yielding a transient opercular syndrome. The concomitance of BECCTS and developmental opercular dysplasia is suggestive of a common genetic substratum.     

儿童良性癫痫伴中央颞区棘波患者的认知损害研究

目的探讨儿童良性癫痫伴中央颞区棘波(Benign epilepsy of childhood with centrotemporal spikes,BECTS)患者的认知损害特点。方法纳入2010年9月-2019年12月于首都医科大学宣武医院神经内科就诊的BECTS患者61例(BECTS组)。选取60名年龄、性别和教育程度相匹配的健康儿童和青少年作为对照组。对BECTS组及对照组进行神经心理学任务测试以评估其注意力、记忆力、计算力、语言功能、执行能力、视空间功能、视知觉及反应能力等多项认知功能。应用SPSS 20.0统计软件,对两组的认知任务测试得分进行独立样本t检验和秩和检验,对影响认知功能损害的各项临床因素进行多元线性回归分析。结果与对照组相比,BECTS组患者的配对联结学习平均(19.56±2.91)分,词语辨析测验的平均(23.67±9.50)分,物体数量认知的平均(61.45±13.14)分,言语工作记忆的平均(6.54±1.47)分,声音知觉测验的平均(5.79±5.90)分,Taylor复杂图形测验的平均(35.10±2.33)分,选择反应时的平均(700.34±493.05)分,均显著低于对照组(P<0.05)。在BECTS组中有36例起病<8岁,与起病≥8岁组相比,前者在视觉追踪任务、空间记忆任务、简单减法任务、数字数量大小比较、汉字押韵测试、词语辨析测试和视觉感知任务等大部分神经心理测试中得分均较低(P<0.05);34例接受单药治疗,27例接受两种及以上抗癫痫药物治疗,多药治疗组在注意力、记忆力、视知觉、反应力等方面均低于单药治疗组(P<0.05)。结论 BECTS患者存在注意力、记忆力、视知觉、声音知觉及反应速度的损害。起病年龄越小其认知损害的程度越重;多药治疗患者的认知损害程度较单药治疗重。     

Benign epilepsy with centro-temporal spikes: spike triggered fMRI shows somato-sensory cortex activity

OBJECTIVE: We performed spike triggered functional MRI (fMRI) in a 12 year old girl with Benign Epilepsy with Centro-temporal Spikes (BECTS) and left-sided spikes. Our aim was to demonstrate the cerebral origin of her interictal spikes. METHODS: EEG was recorded within the 3 Tesla MRI. Whole brain fMRI images were acquired, beginning 2-3 seconds after spikes. Baseline fMRI images were acquired when there were no spikes for 20 seconds. Image sets were compared with the Student's t-test. RESULTS: Ten spike and 20 baseline brain volumes were analysed. Focal activiation was seen in the inferior left sensorimotor cortex near the face area. The anterior cingulate was more active during baseline than spikes. CONCLUSIONS: Left sided epileptiform activity in this patient with BECTS is associated with fMRI activation in the left face region of the somatosensory cortex, which would be consistent with the facial sensorimotor involvement in BECT seizures. The presence of BOLD signal change in other regions raises the possibility that the scalp recorded field of this patient with BECTs may reflect electrical change in more than one brain region.     

Abnormal neuroimaging in patients with benign epilepsy with centrotemporal spikes

PURPOSE: Neuroimaging procedures are usually unnecessary in benign epilepsy of childhood with centrotemporal spikes (BECTS) but are often performed before a specific diagnosis has been reached. By definition, BECTS occurs in normal children; however, recent reports have shown that it also can affect children with static brain lesions. We evaluated the prevalence of abnormal neuroimaging in BECTS and assessed whether the lesions had influenced the clinical and EEG expression of this epilepsy. RESULTS: Among 98 consecutive cases first referred between 1984 and 1999, neuroimaging had been performed in 71 (72%) [magnetic resonance imaging (MRI), 20; computed tomography (CT), 59; MRI+CT, eight]. In ten (14.8%), neuroradiologic procedures were abnormal: enlargement of lateral venticles in five cases including a shunted hydrocephalus in two (no etiology in one, neonatal intraventricular hemorrhage in one), a moderate ventricular dilation in one (neonatal distress), a slight ventricular dilation and hypersignal intensities in the white matter in one (premature birth at 27 weeks), and a moderate enlargement of the right temporal horn in one. A right hippocampal atrophy, a biopercular polymicrogyria, a cavum septum pellucidum, a small cystic lesion located in the epiphysis, and an agenesis of the corpus callosum with macrocrania also were observed once each. The outcome was benign in all, in accordance with the overall prognosis of BECTS. CONCLUSIONS: This study confirms that neuroimaging may be abnormal in patients with BECTS and shows that the presence of brain lesions has no influence on the prognosis. Conversely, BECTS can be diagnosed in patients with brain lesions with or without significant neurologic history or abnormalities.     

The attention networks in benign epilepsy with centrotemporal spikes: A long-term follow-up study

PurposeTo evaluate the long-term prognosis of attention deficit in children with newly diagnosed benign childhood epilepsy with centrotemporal spikes (BECTS).MethodsAttention network test (ANT) was performed over a period of 7 years on 42 patients who were newly diagnosed with BECTS, in the Department of Neurology of Anhui Provincial Children's Hospital.ResultsIn the patients’ group, the accuracy of ANT was lower (P = 0.000), the total response time was longer (P = 0.000), and the efficiency of orienting (P = 0.000) and alerting (P = 0.041) networks was lower than that of the control group. Accuracy was positively correlated with age of onset (b = 1.184) and negatively correlated with number of seizures (b = −1.321). After 7 years, there was no significant difference in the accuracy (P = 0.385); total response time (P = 0.661); and alerting (P = 0.797), orienting (P = 0.709), and executive control (P = 0.806) network efficiencies between the patients and controls. Accuracy was positively correlated with age of onset (b = 0.8583) and negatively correlated with number of seizures (b = −1.017) and duration of antiepileptic drugs therapy (b = −3.203).ConclusionsIn our study, the newly diagnosed BECTS patients had impaired attention network, mainly in the alerting and orienting domains. Age of onset, number of seizures, and time of antiepileptic treatment may affect the attention networks. With the remission of BECTS, the attention network dysfunction was reversed.     

Electroclinical findings in four patients with karyotype 47,XYY

47,XYY karyotype is a Y chromosome aneuploidy characterized by an extra copy of the Y chromosome in each of the male cells, with an incidence of 1/1000 males. Most studies about 47,XYY have focused on growth, cognitive development, academic performance, behavioural problems, speech and language skills and neuromuscular status. Up-to-date reports on seizures and EEG characteristics concerning 47,XYY men have been sporadic and poorly detailed. The aim of this study is to describe the particular electroclinical patterns in a group of four subjects with 47,XYY karyotype. We performed neurological examinations, psychometric tests, brain MRIs, prolonged EEG recordings during awake and sleep on four unselected males 47,XYY. All four patients presented various degrees of neuropsychological impairment. An incidence of familial antecedents for epilepsy was confirmed by three families. When present, seizures were very similar to that of benign epilepsy with central-temporal spikes, (BECTS), for age of onset, clinical picture, evolution and good response to antiepileptic drugs. EEG recordings in all four subjects showed normal background activity and sleep organization, particular focal spikes and sharp-waves localized mostly over the vertex and/or central-temporal regions, which increased during sleep. In our opinion, these 47,XYY patients present a particular electroclinical pattern.     

The epileptic network and cognition: What functional connectivity is teaching us about the childhood epilepsies

Our objective was to summarize and evaluate the rapidly expanding body of literature studying functional connectivity in childhood epilepsy. In the self‐limited childhood epilepsies, awareness of cognitive comorbidities has been steadily increasing, and recent advances in our understanding of the network effects of these disorders promise insights into the underlying neurobiology. We reviewed publications addressing functional connectivity in children with epilepsy with an emphasis on studies of children with self‐limited childhood epilepsies. The majority of studies have been published in the past 10 years and predominantly examine childhood epilepsy with centrotemporal spikes and childhood absence epilepsy. Cognitive network alterations are commonly observed across the childhood epilepsies. Some of these effects appear to be nonspecific to epilepsy syndrome or even to category of neurological disorder. Other patterns, such as changes in the connectivity of cortical language areas in childhood epilepsy with centrotemporal spikes, provide clues to the underlying cognitive deficits seen in affected children. The literature to date is dominated by general observations of connectivity patterns without a priori hypotheses. These data‐driven studies build an important foundation for hypothesis generation and are already providing useful insights into the neuropathology of the childhood epilepsies. Future work should emphasize hypothesis‐driven approaches and rigorous clinical correlations to better understand how the knowledge of network alterations can be applied to guidance and treatment for the children in our clinics.     

Dysfunctional white‐matter networks in medicated and unmedicated benign epilepsy with centrotemporal spikes

Benign epilepsy with centrotemporal spikes (BECT) is the most common childhood idiopathic focal epilepsy syndrome, which characterized with white‐matter abnormalities in the rolandic cortex. Although diffusion tensor imaging research could characterize white‐matter structural architecture, it cannot detect neural activity or white‐matter functions. Recent studies demonstrated the functional organization of white‐matter by using functional magnetic resonance imaging (fMRI), suggesting that it is feasible to investigate white‐matter dysfunctions in BECT. Resting‐state fMRI data were collected from 24 new‐onset drug‐naive (unmedicated [NMED]), 21 medicated (MED) BECT patients, and 27 healthy controls (HC). Several white‐matter functional networks were obtained using a clustering analysis on voxel‐by‐voxel correlation profiles. Subsequently, conventional functional connectivity (FC) was calculated in four frequency sub‐bands (Slow‐5:0.01–0.027, Slow‐4:0.027–0.073, Slow‐3:0.073–0.198, and Slow‐2:0.198–0.25 Hz). We also employed a functional covariance connectivity (FCC) to estimate the covariant relationship between two white‐matter networks based on their correlations with multiple gray‐matter regions. Compared with HC, the NMED showed increased FC and/or FCC in rolandic network (RN) and precentral/postcentral network, and decreased FC and/or FCC in dorsal frontal network, while these alterations were not observed in the MED group. Moreover, the changes exhibited frequency‐specific properties. Specifically, only two alterations were shared in at least two frequency bands. Most of these alterations were observed in the frequency bands of Slow‐3 and Slow‐4. This study provided further support on the existence of white‐matter functional networks which exhibited frequency‐specific properties, and extended abnormalities of rolandic area from the perspective of white‐matter dysfunction in BECT.     

EEG characteristics related to educational impairments in children with benign childhood epilepsy with centrotemporal spikes

PURPOSE: Learning and behavioral difficulties often occur in benign childhood epilepsy with centrotemporal spikes (BCECTS). In recent years, several electroencephalogram (EEG) characteristics have been related to the occurrence of learning and behavioral problems. METHODS: From 28 children medical, school and psychological reports were present and children were rated according to a 4-point scale for educational and behavioral impairment (Part 1). Thirty 24-h EEG recordings were reanalyzed for spike frequency, the presence of atypical EEG criteria, and the presence of a nondipole spike. EEGs were scored during wakefulness, first hour of sleep and whole night sleep (minus the first hour of sleep) separately (Part 2). RESULTS: The presence of I: an intermittent slow-wave focus during wakefulness, II: a high number of spikes in the first hour of sleep (and during whole night sleep), and III: multiple asynchronous bilateral spike-wave foci in the first hour of sleep correlates significantly with a sum score > or = 3 which indicates a complicated course with educational or behavioral impairment. It is sufficient to analyze an EEG during wakefulness and a sleep EEG for only the first hour of sleep instead of a whole night recording to demonstrate those EEG criteria. CONCLUSIONS: On basis of our reanalysis we can possibly conclude that the aforementioned EEG characteristics correlate with educational impairments, and that analysing an EEG recording during wake and the first hour of sleep is sufficient to look adequately for those EEG criteria in children with BCECTS.     

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.     

Evoked spikes and giant somatosensory evoked potentials in a patient with fragile-X syndrome

We report the case of a 7 year old boy with fragile-X syndrome and epilepsy. In this patient, the detection of rolandic epileptiform potentials during sleep and hand tapping-evoked rolandic EEG spikes, together with giant somatosensory evoked potentials, further support the already suggested neurophysiological similarities between fragile-X syndrome and benign childhood epilepsy with centrotemporal spikes.

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Sommario Descriviamo il caso di un bambino di 7 anni affetto da sindrome del cromosoma X fragile ed epilessia. L'osservazione di potenziali epilettiformi rolandici durante il sonno, di punte rolandiche evocate dalla percussione della mano destra e di potenziali evocati somatosensoriali giganti, in questo paziente, fornisce unulteriore supporto alle somiglianze neurofisiologiche, già ipotizzate, tra sindrome del cromosoma X fragile ed epilessia a punte centrotemporali.