Abstract:Purpose: To describe the EEG pattern of seizures in patients with benign childhood epilepsy with centro-temporal spikes (BCECTS). Methods: The clinical and EEG data of 701 BCECTS patients with at least a 3 years follow-up were reviewed from 10 epilepsy centers. Results: Thirty-four seizures were recorded in 30 patients. Four different ictal EEG patterns (A-D) were identified. The most frequent (pattern A) was characterized by low voltage activity of fast rhythmic spikes, increasing in amplitude and decreasing … Show more
“…In addition, a study combining EEG source imaging and fMRI showed propagation of the interictal activity from the rolandic region corresponding to the hand and face area, to the operculum and insula [11] . It has been previously suggested that involvement of insula likely explains the sensations of laryngeal constriction and choking that is often reported by patients with self-limited epilepsy with centrotemporal spikes [3] .…”
Section: Discussionmentioning
confidence: 99%
“…The largest number of patients was reported by Capovilla and co-workers [3] . They retrospectively collected 30 patients with ictal recordings, and they identified four types of ictal patterns, the most common being the low-voltage fast activity.…”
HighlightsWe analysed seizures in patients with self-limited epilepsy with centrotemporal spikes.Ictal source imaging showed activation of the operculo-insular area.Ictal EEG activity was time-locked to contralateral facial myoclonic jerks.The dynamics of the seizures was fragmented.
“…In addition, a study combining EEG source imaging and fMRI showed propagation of the interictal activity from the rolandic region corresponding to the hand and face area, to the operculum and insula [11] . It has been previously suggested that involvement of insula likely explains the sensations of laryngeal constriction and choking that is often reported by patients with self-limited epilepsy with centrotemporal spikes [3] .…”
Section: Discussionmentioning
confidence: 99%
“…The largest number of patients was reported by Capovilla and co-workers [3] . They retrospectively collected 30 patients with ictal recordings, and they identified four types of ictal patterns, the most common being the low-voltage fast activity.…”
HighlightsWe analysed seizures in patients with self-limited epilepsy with centrotemporal spikes.Ictal source imaging showed activation of the operculo-insular area.Ictal EEG activity was time-locked to contralateral facial myoclonic jerks.The dynamics of the seizures was fragmented.
“…Rolandic epilepsy (RE) or benign childhood epilepsy with centro‐temporal spikes is the most common childhood focal epilepsy (Fejerman, ; Panayiotopoulos et al ., ), usually starting between 7 and 10 years. The cardinal features are focal seizures consisting of unilateral facial sensory‐motor symptoms, oropharyngo‐laryngeal symptoms, speech arrest, and hypersalivation (Capovilla et al ., ). Centro‐temporal spikes, typically activated by drowsiness and slow sleep, indicate that the epileptogenic zone in Rolandic epilepsy involves neuronal networks within the Rolandic cortex surrounding the central fissure bilaterally.…”
Section: Involvement Of Autonomic Sensorimotor Auditory Vocal Andmentioning
The term idiopathic focal epilepsies of childhood (IFE) is not formally recognised by the ILAE in its 2010 revision (Berg et al., ), nor are its members and boundaries precisely delineated. The IFEs are amongst the most commonly encountered epilepsy syndromes affecting children. They are fascinating disorders that hold many “treats” for both clinicians and researchers. For example, the IFEs pose many of the most interesting questions central to epileptology: how are functional brain networks involved in the manifestation of epilepsy? What are the shared mechanisms of comorbidity between epilepsy and neurodevelopmental disorders? How do focal EEG discharges impact cognitive functioning? What explains the age‐related expression of these syndromes? Why are EEG discharges and seizures so tightly locked to slow‐wave sleep?
In the last few decades, the clinical symptomatology and the respective courses of many IFEs have been described, although they are still not widely appreciated beyond the specialist community. Most neurologists would recognise the core syndromes of IFE to comprise: benign epilepsy of childhood with centro‐temporal spikes or Rolandic epilepsy (BECTS/RE); Panayiotopoulos syndrome; and the idiopathic occipital epilepsies (Gastaut and photosensitive types). The Landau‐Kleffner syndrome and the related (idiopathic) epilepsy with continuous spikes and waves in sleep (CSWS or ESES) are also often included, both as a consequence of the shared morphology of the interictal discharges and their potential evolution from core syndromes, for example, CSWS from BECTS. Atypical benign focal epilepsy of childhood also has shared electro‐clinical features warranting inclusion. In addition, a number of less well‐defined syndromes of IFE have been proposed, including benign childhood seizures with affective symptoms, benign childhood epilepsy with parietal spikes, benign childhood seizures with frontal or midline spikes, and benign focal seizures of adolescence.
The term “benign” is often used in connection with the IFEs and is increasingly being challenged. Certainly most of these disorders are not associated with the devastating cognitive and behavioural problems seen with early childhood epileptic encephalopathies, such as West or Dravet syndromes. However, it is clear that specific, and sometimes persistent, neuropsychological deficits in attention, language and literacy accompany many of the IFEs that, when multiplied by the large numbers affected, make up a significant public health problem. Understanding the nature, distribution, evolution, risk and management of these is an important area of current research.
A corollary to such questions regarding comorbidities is the role of focal interictal spikes and their enduring impact on cognitive functioning. What explains the paradox that epilepsies characterised by abundant interictal epileptiform abnormalities are often associated with very few clinical seizures? This is an exciting area in both clinical and experimental arenas and will eventually have imp...
“…In 1990, Gutierrez et al described an ictal event with speech arrest only characterized by a short train of ictal alpha activity, and then two multiple spikes and wave complexes originated from the left centrotemporal region followed by marked attenuation of the left hemispheric background [115]. Subclinical rhythmic discharges of spike and wave in the centrotemporal region have been documented by several authors in RE [116,117]. Saint-Martin et al in 2001 described a series of patients presenting with typical and also atypical ictal manifestations such as falls, negative myoclonus and observed that positive motor phenomenon correlated to the spike component preceding a negative motor phenomenon, correlated with the slow-wave component of the spike and wave complex [118].…”
Section: Ictal Eegmentioning
confidence: 99%
“…Capovilla et al recorded 34 seizures in 30 patients with RE and described four electrographic seizure patterns thus emphasizing that ictal pattern for RE is not unique [116]:…”
Childhood epilepsy with centrotemporal spikes, had been previously considered as benign childhood epilepsy. According to the new classification proposed by Sheffer I. and colleagues the term “benign” has been changed to “self-limited”. Many studies reported that BECTS may cause transient or long lasting cognitive and behavioral disturbances. Rolandic epilepsy is the most frequent among the childhood focal epilepsy and may account for about 15–25% of all epileptic syndromes diagnosed between the ages of 5 to 15 years. The incidence range changes between 7.1–21 per 100000 in population younger than 15 years with male predominance. The age of onset in 90% of cases between 1 and 10 years with peak around 6–7 years. Seizures mainly occur during a night sleep, whereas the probability of awake seizures are less than 10%. The characteristic clinical features are: (1) focal motor seizure with unilateral orofacial tonic or clonic contractions; (2) speech arrest; (3) hypersalivation; (4) sensory symptoms represented by unilateral numbness or paresthesia of tongue, lips, gum and inner part of the check; (5) unilateral clonic jerk in leg and arm with postictal paresis; (6) generalized seizures. The EEG picture is distinctive in Rolandic epilepsy. The background activity is almost always preserved in awake state and during a sleep. The typical interictal EEG pattern is high voltage, diphasic spikes or sharp waves frequently with slow activity on central-midtemporal region. The centrotemporal spikes or rolandic spikes come from the lower rolandic region created a horizontal dipole with maximal electronegativity in the centrotemporal region and electropositivity in the frontal region usually seen unilateral or bilateral. In most cases children with RE have a good prognosis regarding both seizures and neurodevelopment. The remission of seizures usually occurs before the age of 18 years. The cognitive and behavior problem may happen in active period of disease which are reversable in most of patients.
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