Benign Rolandic Epilepsy (BRE)
Also known as Self-Limited Epilepsy with Centro-Temporal Spikes (SeLECTS) or
Childhood Epilepsy with Centro-Temporal Spikes (CECTS)
The International League Against Epilepsy (ILAE) has updated its classification of epilepsy syndromes.
The term “benign” is now often replaced with “self-limited” to acknowledge that while the epilepsy resolves, the condition may still have a significant impact on a child’s quality of life, including potential neurodevelopmental and psychosocial challenges.
Presentation
Epidemiology and Age: CECTS is the most common self-limited epilepsy syndrome in childhood, typically starting between the ages of 4 and 10, and almost always resolving by puberty (age 15-16).
Genetics: A genetic predisposition is well-established, with a significant number of cases clustering in families. The inheritance pattern is thought to be autosomal dominant with age-dependent penetrance.
Neurodevelopmental Impact: While children with CECTS generally have normal development, recent literature and updated guidelines recognize that some may experience subtle learning difficulties (e.g., in language or executive function) or behavioral problems. These issues are often transient and tend to improve once the seizures stop. This recognition is a key reason for the move away from the “benign” label.
Seizure Characteristics
Focal Seizures: Seizures are brief and focal, usually occurring during sleep or on waking. They originate from the centro-temporal region of the brain.
Semiology: Seizures are characterized by a sensory-motor experience affecting the face and oropharynx. This includes:
Tingling or numbness on one side of the face or tongue.
Difficulty speaking (anarthria) or making guttural noises (e.g., gurgling or clicking).
Hypersalivation (drooling).
Twitching or tonic-clonic movements of the face and, sometimes, the ipsilateral arm and leg.
Progression: Consciousness is often preserved. However, seizures can evolve into focal to bilateral tonic-clonic seizures, which may be followed by postictal drowsiness and, occasionally, Todd’s paresis (transient unilateral weakness).
Frequency: Seizure frequency is typically low, with most children experiencing fewer than 10 seizures in their lifetime.
Investigation
EEG: An electroencephalogram (EEG) is the cornerstone of diagnosis. It shows a normal background with characteristic, high-amplitude, centro-temporal spikes. These spikes are strongly activated by sleep, so a sleep-deprived EEG is crucial if the initial study is normal.
Neuroimaging: Routine neuroimaging, such as an MRI, is not required for a typical presentation of CECTS. It should be considered only if there are atypical features, such as an abnormal neurological exam, unusual seizure types, or an atypical EEG.
Management
The management of CECTS has evolved to a shared decision-making model that balances the potential risks of seizures against the side effects of anti-epileptic drugs (AEDs) and the condition’s self-limited nature.
Observation: A “watch-and-wait” approach is the most common and appropriate strategy for the majority of children with infrequent, nocturnal seizures that don’t significantly impact their daily life. This is supported by evidence that treatment does not alter the long-term prognosis.
Indications for Treatment: Treatment is typically considered for:
Frequent seizures (e.g., more than once a month).
Daytime seizures that disrupt school or activities.
Seizures that progress to generalised tonic-clonic seizures.
Significant neurocognitive or behavioral issues linked to seizure activity.
First-Line Medications:
Levetiracetam is increasingly considered a first-line agent due to its good efficacy, favorable side-effect profile, and minimal cognitive impact.
Carbamazepine and Oxcarbazepine are also highly effective, although there is some concern about potential cognitive side effects with Carbamazepine.
Lamotrigine can be used as an alternative.
Discontinuation of Treatment: Medication can generally be tapered and discontinued after a child has been seizure-free for 1-2 years. The decision should be made in consultation with a neurologist, without necessarily requiring a repeat EEG.