Abstract

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.

Highlights

  • Self-limited epilepsy with centrotemporal spikes (SECTS), well-known as Rolandic epilepsy is the most frequent among the childhood focal epilepsies and may account for about 15–25% of all epileptic syndromes diagnosed between the Epilepsy - Update on Classification, Etiologies, Instrumental Diagnosis and Treatment ages of 5 to 15 years [1]

  • Self-limited epilepsy with centrotemporal spikes is a syndrome of brief hemifacial motor seizures, frequently having associated somatosensory symptoms, usually without impairment of consciousness which tend to evolve into GTCS [3–6]

  • Mutations in PRRT2, KCNQ2, KCNQ3, RBFOX1, and DEPDC5 genes with an autosomal dominant transmission reported in patients with RE spectrum epilepsy syndromes, they have not been confirmed by the studies based on large case series [70]

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Summary

Introduction

Self-limited epilepsy with centrotemporal spikes (SECTS), well-known as Rolandic epilepsy is the most frequent among the childhood focal epilepsies and may account for about 15–25% of all epileptic syndromes diagnosed between the Epilepsy - Update on Classification, Etiologies, Instrumental Diagnosis and Treatment ages of 5 to 15 years [1]. It is termed ‘rolandic’ epilepsy because the focal seizures are originated from the region around the lower part of the central gyrus of Rolando. Many studies reported that RE may cause transient or long-lasting cognitive and behavioral disturbances [4, 5, 11–40]

Terminology and classification
Etiology
Clinical features
Interictal EEG
Ictal EEG
Prognosis
Atypical rolandic epilepsy
Findings
Treatment
Full Text
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