Comparing cortical auditory processing in children with typical and atypical benign epilepsy with centrotemporal spikes: Electrophysiologic evidence of the role of non-rapid eye movement sleep abnormalities.

OBJECTIVE: The mismatch negativity (MMN) is an objective measure of central auditory discrimination. MMN alterations have been shown in children with language and/or developmental disorders. In benign focal epilepsies, neuropsychological disorders are often reported and linked to interictal epileptic discharges (IEDs) during non-rapid eye movement (NREM) sleep. There are few studies reporting MMN in children with benign epilepsy with centrotemporal spikes (BECTS) and sleep IEDs. Moreover, no MMN recording has yet been reported in atypical BECTS children with continuous spike-and-wave during sleep (CSWS). We retrospectively compared MMN in typical and atypical BECTS children, particularly addressing the impact of NREM sleep IEDs on auditory discrimination. Moreover, we attempted a neuropsychological characterization of patients. METHODS: The MMN was recorded in 9 normal controls and 23 patients (14 typical BECTS and 9 atypical BECTS) in an oddball paradigm with syllable stimuli. MMN, sleep electroencephalography (EEG) and neuropsychological evaluation were realized in the same testing session. RESULTS: Measurable MMN responses to speech stimuli were identified in both the control and patient groups. A significant difference between control and atypical BECTS children was found with respect to amplitude (p = 0.0061). Atypical BECTS also showed a lower MMN amplitude with respect to typical BECTS, but this difference did not reach statistical significance (p = 0.0545). Statistical comparisons between groups revealed no differences in latency. Among the neuropsychological variables, academic difficulties were significantly more prominent in the patients with atypical BECTS (p = 0.04). SIGNIFICANCE: CSWS EEG pattern affects auditory discrimination and may have a long-lasting impact on academic skills acquisition, whereas in typical BECTS children with a lower degree of IED NREM sleep, plastic brain reorganization or the preservation of participating networks may prevent such difficulty. Early electrophysiologic identification of auditory discrimination deficits in epileptic children could be used in early rehabilitation, thereby reducing the risk of developing neuropsychological disorders.

Interictal autonomic abnormalities in idiopathic Rolandic epilepsy.

We investigated 50 young patients with a diagnosis of Rolandic Epilepsy (RE) for the presence of abnormalities in autonomic tone compared with 50 young patients with idiopathic generalized epilepsy with absences and 50 typically developing children of comparable age. We analyzed time domain (N-N interval, pNN50) and frequency domain (High Frequency (HF), Low Frequency (LF) and LF/HF ratio) indices from ten-minute resting EKG activity. Patients with RE showed significantly higher HF and lower LF power and lower LF/HF ratio than controls, independent of the epilepsy group, and did not show significant differences in any other autonomic index with respect to the two control groups. In RE, we found a negative relationship between both seizure load and frequency of sleep interictal EEG abnormalities with parasympathetic drive levels. These changes might be the expression of adaptive mechanisms to prevent the excessive sympathetic drive seen in patients with refractory epilepsies.

Variantes clínicas de epilepsia rolándica y su implicación en el pronóstico / Rolandic epilepsy clinical variants and their influence on the prognosis

Introducción: La epilepsia benigna con puntas centro-temporales (EBPCT) es el síndrome epiléptico más frecuente de la infancia, tiene carácter edad-dependiente, elevada predisposición genética y curso benigno. El objetivo de este trabajo es describir el curso clínico y el pronóstico de 60 pacientes diagnosticados de EBPCT en nuestro centro. Pacientes y métodos: Revisión retrospectiva de los pacientes diagnosticados de EBPCT en un hospital universitario (1995-2009). Se dividieron en 2 grupos: a) pacientes que cumplían todos los criterios clásicos de EBPCT, y b) cumplían los criterios excepto uno (menos de 4 años; crisis en vigilia; alteraciones EEG no típicas).Resultados: Se seleccionó a 60 pacientes, 34 varones y 26 mujeres. Se incluyó a 31 pacientes en el grupo 1 y a 29 en el grupo 2. Edad media de inicio en el grupo 1: 7,45 años; grupo 2: 6,55 años. Se indicó tratamiento médico en 32,2% de pacientes del grupo 1, y en 41,3% del grupo 2. La evolución fue favorable en la mayoría: 58% en el grupo 1 y 62,1% en el 2 estaban libres de crisis tras un año. Edad media a la que desaparecieron: 8,54 años en el grupo 1 y 7,84 años en el grupo 2. No se encontraron diferencias estadísticamente significativas en ninguno de estos parámetros.Conclusiones: A diferencia de lo que algunos autores habían publicado, en este trabajo no se han identificado factores clínicos de mala evolución en pacientes con EBPCT, de modo que su diagnóstico se correlaciona con una evolución favorable y un excelente pronóstico neurológico (AU)

Introduction: Benign epilepsy with central-temporal spikes (BECTS) is the most common epileptic syndrome in childhood. It is an age-dependent, genetically determined and benign condition. The aim of this study is to describe the clinical course and prognosis in 60 patients with BECTS diagnosed in our hospital. Patients and methods: We made a retrospective review of patients diagnosed with BECTS in a University Hospital (1995-2009). They were divided into 2 groups: 1) Patients who met all BECTS classical criteria. 2) Patients who met all the criteria but one (less than 4 years; diurnal seizures; atypical EEG abnormalities). Results: A total of 60 patients, 34 males and 26 females were included, with 31 patients in group 1 and 29 in group 2. The mean age at onset in group 1: 7.45 years, group 2: 6.55 years. Medical treatment was indicated in 32.2% of patients in group 1 and 41.3% in group 2. The outcome was favourable in the majority: 58% in group 1 and 62.1% in group 2 were free of seizures after 1 year. Average age in which it disappeared: 8.54 years in group 1 and 7.84 years in group 2. There were no statistically significant differences in any of these parameters.Conclusions: Unlike that published by some authors, we have not identified any poor outcome factors in patients with BECTS in this study, meaning that an accurate diagnosis correlates with a good prognosis and excellent neurological outcome (AU)

Electroencephalography in clinical epilepsy research.

Electroencephalography (EEG) remains central to the investigation of epilepsy. This review discusses two clinical problems at the temporal extremes of neurophysiologic recording: evaluation of the clinical significance of individual spike discharges in benign epilepsy of childhood with centrotemporal spikes (BECTS), and prolonged (several days) continuous EEG monitoring in the ICU. BECTS is misdiagnosed often, and probably mis-treated often as well. Though the long-term outcome is usually excellent, it remains unclear whether the individual epileptiform discharges have a clinical effect. Answering this question is difficult, in part because of the natural evolution of the epilepsy and its different appearance depending on wakefulness or sleep state, and also due to substantial methodologic problems in measuring short and long-term cognitive effects. Continuous EEG (CEEG) recording has grown remarkably over the last 10 years. It has proved crucial in the diagnosis of nonconvulsive status epilepticus (NCSE), especially in the ICU, given the usual lack of obvious clinical signs of seizures in most of these patients, many of whom are critically ill. Much progress has been made in agreeing on terminology for the EEG findings, but diagnosis is still complicated. More efficient and reliable technology is being developed to help process the massive amount of data captured by CEEG and make it more useful (and in a timely fashion) clinically. Still, it is not completely clear which patients should be monitored, for how long, and what is the best role for CEEG in assessing and adjusting treatment once the diagnosis has been made. Investigators are using CEEG to study "seizure burden," to help determine what are the long-term effects of nonconvulsive seizures and NCSE, and to help guide treatment and improve outcome.

[Idiopathic focal epilepsies of infancy and childhood].

We studied 1036 children with epileptic seizures, aged from 1 to 18 years, during 2004-2008. One hundred and six patients were diagnosed with idiopathic focal epilepsy (IFE). The following forms of IFE were singled out: benign seizures of infancy (familial and non-familial) - Watanabe--Vigevano syndrome - 5,7%, occipital epilepsy of childhood with early manifestation (Panayiotopoulos syndrome) -26,4%, occipital epilepsy of childhood with late manifestation (Gastaut syndrome) - 12,3%, benign epilepsy of childhood with central-temporal spikes (rolandic epilepsy) - 51%, benign focal epilepsy with affective symptoms - 4,7%. The efficacy of the first monotherapy was significantly worse in rolandic epilepsy compared to the other IFE forms. Prescription of valproate or the combination of valproate, ethosuximidum and levetiracetam, in case of resistant course, as a starting therapy was found optimal.

Benign childhood epilepsy with centrotemporal spikes: an ictal EEG.

Benign childhood epilepsy with centrotemporal spikes (BECTS) is common during childhood, but there are few reports in the literature recording the EEG during a seizure. We studied an 8-year-old boy with oropharyngeal seizures during wakefulness and sleep. Both his neuropsychomotor development and neurological examination were normal. While awake, the subjects's electroencephalogram (EEG) showed normal background activity and epileptiform activity characterized by spikes in the temporal regions (mid and anterior), central region of the right cerebral hemisphere and in the median central and parietal regions. During sleep, his EEG recorded an epileptic seizure that lasted 46 seconds. In the initial phase, the EEG showed an increase in the number of spikes with higher potential in the median central and parietal regions, followed by slow waves associated with the increase in slow waves in the right hemisphere. This was followed by a brief decrease in amplitude of the background activity, and then by rhythmic, diffuse discharges predominantly in the right centrotemporal region, of sharp waves at 12-13 Hz, with increasing potential. Slow waves of high amplitude then occurred for 5 seconds, and finally very high potential spikes reappeared in the central and temporal regions of the right cerebral hemisphere with normalization of the background activity. During these critical phases of the EEG, clonic lip movements and pouting could be observed with the mouth locked shut, associated with "throat noises," but there were no other motor manifestations. The child did not wake up during the seizure and there were no postictal signs or symptoms. Although there are some aspects in common in recordings of BECTS seizures, such as a reduction in amplitude followed by rhythmic discharges of increasing amplitude, differences exist that possibly correspond to the diverse characteristics of the electrical generators.

Atypical presentations of benign childhood epilepsy with centrotemporal spikes: a review.

Benign childhood epilepsy with centrotemporal spikes is the most common epileptic syndrome in childhood. Atypical forms of benign childhood epilepsy with centrotemporal spikes are common. The different atypical forms of the condition are believed to represent a continuum of the same underlying genetic mechanism. The atypical forms of benign childhood epilepsy with centrotemporal spikes include electrical status epilepticus in slow waves sleep, Landau-Kleffner syndrome, status epilepticus of benign childhood epilepsy with centrotemporal spikes, "classic" atypical form, and others. This review delineates the different forms with emphasis on the cognitive hazards of the more malignant types.

Autonomic status epilepticus in Panayiotopoulos syndrome and other childhood and adult epilepsies: a consensus view.

PURPOSE: To discuss and propose a definition of autonomic status epilepticus (SE), describe its clinical and EEG features, and review what is known about its epidemiology, pathophysiology, differential diagnosis, and management. METHODS: An international consortium of established researchers in the field was identified from their published work, agreed the purpose of the project, searched the literature, and, by use of e-mail communication, agreed the consensus document. RESULTS: Autonomic SE is a condition lasting at least 30 min and characterized by epileptic activity causing altered autonomic function of any type at seizure onset or in which manifestations consistent with altered autonomic function are prominent (quantitatively dominant or clinically important) even if not present at seizure onset. It is best described, and probably most commonly encountered in children, with Panayiotopoulos syndrome. However, it also occurs in children with symptomatic epilepsies and, exceptionally, in adults. Its pathogenesis and most appropriate management are poorly understood. CONCLUSIONS: It is hoped that this document will help clinical recognition of Autonomic SE, reduce misdiagnosis, and promote further interest and studies into what has been a relatively neglected area.

Rolandic epilepsy: a challenge in terminology and classification.

History and present definition of rolandic epilepsy (RE) is briefly presented. In the literature there has often been misconceptions in the description of the syndrome, and the affinity to related conditions and structural abnormalities with rolandic discharges is often unclear. This has resulted in confusion regarding the definition and delineation of RE. A spectrum of RE based on a maturational continuum is possible. Until more is known about the genetic background of RE, a simple classification is proposed: 1) RE "pure" according to the original definition; 2) RE "plus"; 3) RE-related disorders; 4) Structural brain lesions with signs and symptoms as in RE. A summary of results from neuroimaging, neuropsychological and oromotor studies in RE "pure" is presented. Accurate clinical assessment and EEG analysis is essential for a proper classification of RE.

[Broad clinical and prognostic spectrum of the Rolandic epilepsies. Typical forms]. / Amplio espectro clínico y pronóstico de las epilepsias rolándicas. Formas típicas.

OBJECTIVE: We reviewed studies on Rolandic epilepsies (ER) and analyzed them according to definite criteria to establish the typical clinical and electroencephalographic phenotypes and therapeutic strategy to be used. DEVELOPMENT: The criteria for definition leave gaps which permit inclusion, as typical forms of ER, variant forms or syndromes related to ER. Clinical phenotypes: 1) Classical phenotypes with senso motor, phonatory or autonomic seizures; 2) Benign partial state which is rarely seen; 3) ER with cognitive involvement usually of attention, reading, auditory verbal learning, semantic fluency and visuomotor coordination; 4) Idiopathic ER with interictal facial myoclonus; and 5) Other phenotypes include ER coinciding with a pre existing cerebral disorder and transient EPOCS of iatrogenic origin. EEG phenotypes: 1) Classical EEG with unilateral or bilateral, synchronous or asynchronous Rolandic paroxysms; 2) Phenotypes with generalized PO; 3) Phenotype with centroparietal slow waves, rare and usually transient. The various authors do not agree as to whether treatment should be recommended although in the studies we consulted there was a high proportion of ER and it was recognized that the risk of inducing EPOCS on treatment was low. CONCLUSIONS: Analysis of the literature of typical forms of ER shows that apart from the classical form there is a continuous range of epileptic dysfunction, both clinical and of EEG, which includes the related variant forms and syndromes, although the long term prognosis of all is good, some with and some without treatment

Semiology of typical and atypical Rolandic epilepsy: a video-EEG analysis.

Since the first descriptions of Rolandic Epilepsy or benign epilepsy with centrotemporal spikes (BECTS), typical and atypical forms have been reported. Indeed, classical focal seizures are sometimes associated with various atypical ictal symptoms and cognitive or behavioural disorders. In an effort to define early clinical and EEG criteria allowing early distinction between typical and atypical forms, we recently conducted a prospective study in a cohort of children with Rolandic Epilepsy. The results of this study have been reported elsewhere. We now discuss the semiological characteristics, and comment on the video-EEG data collected during this study. Symptoms were classified into three major categories: "classical focal seizures"; "spike and wave related symptoms"; and "paraictal symptoms". Classical focal seizures constitute the electroclinical expression of the development and the propagation of a focal cortical neuronal discharge. "Spike and wave related symptoms" are brief neurological or neuropsychological phenomena having a relatively strict temporal relation with individual components of isolated focal or generalized spikes and waves. "Paraictal symptoms" consist of acquired progressive and fluctuating motor or cognitive deficits and are not directly correlated with Todd paralysis. We present detailed video-EEG material of selected cases and discuss the usefulness of such distinctions in terminology. We suggest that variability in clinical expression probably reflects the implication of different pathophysiological mechanisms, which in turn could explain differences in sensitivity to treatment. (Published with videosequences.)

Treatment of benign epilepsy syndromes throughout life.

Classification of epilepsy has been refined to a degree that begins to allow identification of syndromes that are benign, if not in clinical pattern, at least with regard to ultimate outcome. Infants, children, adolescents, and even adults, on occasion, develop seizures that challenge judgment regarding selection of drugs and duration of treatment. Childhood absence epilepsy tends to follow a benign course, with abatement of need for treatment in many patients. However, in approximately 40% of patients with onset of this syndrome, there is evolution to juvenile myoclonic epilepsy (JME), a form of seizure disorder that requires life-long treatment. One syndrome that is clearly almost always benign is benign epilepsy with centrotemporal spikes (BECTS). This clinical problem is defined by complaints, observations of behavior, and by a specific electroencephalographic pattern. Some patients are not treated; many undergo brief administration of medication. Infantile convulsive seizures that may be partial or with myoclonus tend to be familial and require treatment for 1 or 2 years. Time is required to make a decision about treatment because observation over time is required to be sure patients are developing normally, a hallmark of these syndromes. Familial groupings of partial seizures in adolescents appear to follow a benign course, as do those in many elderly patients with seizures after cerebral infarction. Treatment decisions require clinical judgment and observations over time. Drug selection is governed more by experience of individual clinicians, because benign syndromes occur infrequently and prospective studies are seldom performed.

Benign rolandic epilepsy: clinical and electroencephalographic correlates.

Benign rolandic epilepsy (BRE) is known for its dissociation from structural alterations. Nevertheless, the number of cases with reported organic lesions has been increasing. This led to the creation of two subgroups, "benign" and "non benign" BRE, and resulted in the need for additional parameters to define electrographic benignity. We assessed the possible associations between interictal electroencephalographic findings and clinical behavior in 60 BRE cases, testing four parameters of electrographic benignity (paroxysm morphology, horizontal dipole, base rhythms, laterality of rolandic spikes). We also assessed the relationship between neuroimaging findings and electrographic and clinical classifications, and found a statistically significant association (sensitivity=73.5%; specificity=81.8%; positive predictive value=94. 8%; negative predictive value=40.9%). Three of the electrographic parameters proposed were associated with clinical classification: paroxysm morphology, horizontal dipole, and base rhythms. Cases electrographically classified as benign have 21 times more chances to be equally classified as clinically benign according with the tested criteria.