What is rolandic epilepsy?

What is Rolandic Epilepsy?

Rolandic epilepsy (also called benign epilepsy of childhood with centrotemporal spikes or BECT) is the most common idiopathic focal epilepsy syndrome of childhood, characterized by brief nocturnal seizures involving the face and mouth, distinctive centrotemporal spikes on EEG, and spontaneous resolution by puberty. 1, 2, 3

Clinical Features

Seizure Characteristics

• Seizures are predominantly nocturnal and sleep-related, consisting of simple partial seizures with unilateral facial clonic activity (twitching of one side of the face), dysphasia (difficulty speaking), and drooling 3
• Generalized tonic-clonic seizures can occur, particularly during sleep 3
• Seizures are typically brief in duration and of variable frequency, often infrequent though clusters may occur 2, 3
• Less commonly, seizures may involve arm jerking or, rarely, leg sensorimotor manifestations 2

Age and Demographics

• Onset typically occurs in the first decade of life (mean age around 6-7 years), affecting otherwise healthy children 4, 3
• The condition affects both males and females, with a slight male predominance 4
• Seizures spontaneously resolve by puberty (almost always by age 16 years) 3

Diagnostic Approach

EEG Findings

• The hallmark is high-amplitude centrotemporal spikes and spike-and-wave complexes on EEG, often bilateral and asynchronous 1, 2
• These characteristic spikes are markedly activated by sleep 2, 3

Neuroimaging

• Neuroimaging is not routinely required for typical cases with classic clinical and EEG features, as diagnosis is primarily clinical 1
• The American College of Radiology recommends against routine MRI when presentation is typical with characteristic seizure semiology and EEG findings 1

Treatment Considerations

When to Treat

• Treatment is often unnecessary given the benign nature and infrequent seizures 2, 3
• Medication should be considered when seizures are frequent or distressing to the child and family 1

Medication Options

• When treatment is indicated, carbamazepine or oxcarbazepine are preferred options based on favorable response profiles 1
• When physicians elect to treat, seizures are usually easily controlled 3
• Approximately 73-74% of children receive medication, with good response rates of 76-78% 4

Prognosis and Associated Features

Overall Prognosis

• The prognosis is excellent, with spontaneous remission by the teenage years 2, 4
• The syndrome is considered one of the most benign epileptic syndromes of childhood 2

Cognitive and Behavioral Considerations

• Mild cognitive and behavioral difficulties are increasingly recognized during the course of the condition and should not be considered atypical features 5
• The condition usually courses with normal psychomotor development, though it may coexist with learning disabilities 4

Atypical Presentations

Risk Factors for Atypical Evolution

• Early age at onset and frequent spike-wave discharges are risk factors for neuropsychological deficits and atypical evolution 5
• Atypical evolutions may include severe neuropsychological impairments and continuous spike-and-waves during slow sleep (CSWSS) 5

Recognition Challenges

• When leg sensorimotor seizures represent the only manifestation, exact recognition can be difficult, potentially leading to unnecessary investigation and therapy 2
• Complex partial seizures may occur in atypical presentations, though simple partial seizures predominate in typical cases 4

Genetic Basis

• The condition has long been presumed to have a genetic component, though clinical and genetic studies show a complex inheritance pattern 6
• Many genes or loci have been associated with the rolandic epilepsy/atypical rolandic epilepsy spectrum, though the full genetic basis remains incompletely understood 6