What is benign Rolandic epilepsy?

Benign Rolandic Epilepsy

Benign Rolandic Epilepsy (BRE) is a common, age-dependent epilepsy syndrome of childhood characterized by brief, nocturnal or sleep-related seizures affecting the face and oropharyngeal muscles, with distinctive centrotemporal spike-wave discharges on EEG that typically resolves spontaneously by adolescence.

Definition and Epidemiology

• BRE, also known as benign epilepsy with centrotemporal spikes (BECTS), is the most common childhood epilepsy syndrome 1
• Typically presents between ages 3-13 years with spontaneous resolution by age 16 1
• Represents a primary partial epilepsy syndrome with a generally favorable prognosis 2

Clinical Characteristics

• Seizures are typically brief and predominantly occur during sleep 1
• Classic presentation includes:
  • Unilateral facial clonic activity 1
  • Dysphasia during seizures 1
  • Excessive drooling or hypersalivation 1
  • Preservation of consciousness during focal seizures 1
• Less common presentations include:
  • Sensorimotor seizures affecting the limbs, including rare cases with leg involvement 3
  • Nocturnal generalized tonic-clonic seizures that may develop from focal onset 1
• Seizures are usually infrequent, though clusters can occur 1

Diagnostic Features

• Diagnosis is primarily clinical, based on characteristic seizure semiology and EEG findings 4
• EEG shows distinctive high-amplitude centrotemporal spikes or spike-and-wave complexes that are:
  • Often bilateral and asynchronous 3
  • Significantly activated during sleep 1
  • Typically located in the rolandic/centrotemporal regions 2
• Neuroimaging is not routinely required for typical cases with classic clinical and EEG features 4
  • MRI may be considered in cases with atypical features to rule out structural lesions 2

Atypical Features That Warrant Further Investigation

• Early age of onset (before age 3) 5
• Frequent seizures or status epilepticus 5
• Daytime seizures predominating over nocturnal events 2
• Developmental regression or significant cognitive/behavioral issues 5
• Focal neurological deficits on examination 2
• Atypical EEG patterns or excessive frequency of epileptiform discharges 5

Cognitive and Behavioral Aspects

• While traditionally considered "benign," mild cognitive and behavioral difficulties are increasingly recognized 5
• Recent studies have identified:
  • Fine motor dysfunction 6
  • Visuomotor integration deficits 6
  • Expressive language difficulties 6
  • Calculation problems (dyscalculia) 6
• These cognitive issues are typically mild and may resolve after seizure remission 5

Treatment Considerations

• Treatment is often unnecessary due to infrequent seizures and spontaneous resolution 1
• When treatment is indicated due to frequent or distressing seizures:
  • Seizures are usually easily controlled with appropriate antiepileptic medication 1
  • Carbamazepine or oxcarbazepine may be preferred options based on favorable response profiles 4

Atypical Evolution

• A small subset of patients may develop more severe forms:
  • Atypical benign focal epilepsy of childhood (ABFEC) 5
  • Status epilepticus of BRE 5
  • Continuous spike-and-waves during slow sleep (CSWSS) 5
  • Landau-Kleffner syndrome (LKS) 5
• Risk factors for atypical evolution include early age at onset and frequent epileptiform discharges 5

Prognosis

• Excellent seizure prognosis with spontaneous remission by adolescence 1
• Complete resolution of seizures typically occurs by age 16 1
• Long-term cognitive outcome is generally good, though subtle deficits may persist in some cases 6