Overview
Definition:
Benign Rolandic Epilepsy (BRE), also known as Self-limited Epilepsy with Centrotemporal Spikes, is a common childhood epilepsy characterized by focal seizures originating from the central-temporal region of the brain
Absence seizures, previously called petit mal seizures, are generalized seizures characterized by brief episodes of altered awareness and unresponsiveness, typically starting in early to mid-childhood.
Epidemiology:
BRE is the most common epilepsy syndrome in childhood, affecting approximately 10-20% of children with epilepsy
It typically begins between ages 3 and 13 years, with a peak incidence around 7-10 years
Absence seizures are also common in childhood, with an incidence of about 0.04% per year in children aged 1-15 years
They often begin between 4 and 10 years of age.
Clinical Significance:
Differentiating BRE from absence seizures is crucial for accurate diagnosis, appropriate management, and prognosis
Misdiagnosis can lead to ineffective treatment, unnecessary investigations, and potential adverse effects from inappropriate antiepileptic drugs (AEDs)
Understanding their distinct electrophysiological and clinical features is vital for pediatricians and neurologists managing these common childhood epilepsy syndromes.
Clinical Presentation
Symptoms:
Benign Rolandic Epilepsy: Focal motor seizures, often involving the face or throat
Symptoms include tingling or numbness in the tongue, gums, or cheek
drooling
difficulty speaking
and sometimes tonic or clonic movements of the face
Seizures are typically brief (1-2 minutes) and often occur during sleep or upon awakening
Absence Seizures: Sudden, brief lapses of consciousness lasting 5-20 seconds
Patient appears to stare blankly, may stop mid-activity, and then resumes activity without awareness of the event
Automatisms like lip smacking, chewing, or eye blinking may occur.
Signs:
Benign Rolandic Epilepsy: During a seizure, observe focal motor activity
Postictally, children are typically alert and neurologically intact
Absence Seizures: During a seizure, the child is unresponsive but conscious
Vital signs are usually normal
Postictally, the child is aware of the seizure and resumes previous activity promptly.
Diagnostic Criteria:
Benign Rolandic Epilepsy: Typically diagnosed based on characteristic clinical seizure types and EEG findings
Normal neurological examination and developmental history
EEG shows characteristic high-amplitude sharp and slow waves in the centrotemporal regions, often bilateral and symmetric, activated by sleep
Absence Seizures: Diagnosed based on clinical history of typical absence spells and EEG findings
EEG shows generalized 3-Hz spike-and-wave discharges, typically triggered by hyperventilation.
Diagnostic Approach
History Taking:
For BRE: Detailed description of seizure semiology, particularly oral or pharyngeal symptoms
Timing of seizures (sleep-related?)
Family history of epilepsy
For Absence Seizures: Precise description of the event (staring spells, duration)
Frequency and triggers of spells
Impact on school and daily activities
Family history
Any preceding or subsequent motor activity.
Physical Examination:
A thorough neurological examination is essential for both
Look for any focal neurological deficits, developmental delay, or dysmorphic features
In BRE, the neurological exam is typically normal between seizures
In absence seizures, the neurological exam is usually normal.
Investigations:
Electroencephalography (EEG) is the cornerstone of diagnosis for both
For BRE: Sleep EEG is highly sensitive, showing characteristic centrotemporal spikes
For Absence Seizures: Routine EEG, especially during hyperventilation, will reveal generalized 3-Hz spike-and-wave complexes
Neuroimaging (MRI brain) is usually not indicated in typical BRE or absence seizures unless there are atypical features, focal neurological deficits, or failure to respond to treatment, to rule out structural lesions.
Differential Diagnosis:
Benign Rolandic Epilepsy: Other focal epilepsy syndromes, simple febrile seizures, psychogenic non-epileptic seizures
Absence Seizures: Complex partial seizures, brief focal motor seizures, absence status epilepticus, absence of attention or daydreaming.
Management
Initial Management:
For both conditions, the primary goal is seizure control and improving quality of life
Reassurance for parents regarding the typically benign prognosis is crucial.
Medical Management:
Benign Rolandic Epilepsy: Antiepileptic drugs (AEDs) are generally indicated if seizures are frequent, impact daily life, or occur during wakefulness
First-line agents include carbamazepine (e.g., 10-20 mg/kg/day divided BID) or ethosuximide (e.g., 10-20 mg/kg/day divided BID-TID)
Levetiracetam or valproate are also effective
Treatment is typically continued for 1-2 years after seizure freedom, with discontinuation usually planned around puberty
Absence Seizures: Ethosuximide is the drug of choice (e.g., 10-20 mg/kg/day divided BID-TID)
Valproate is also highly effective
Lamotrigine can be used, but caution is advised due to potential worsening of absence seizures with some agents like carbamazepine, oxcarbazepine, and vigabatrin.
Surgical Management:
Surgical management is not indicated for typical Benign Rolandic Epilepsy or absence seizures, as they are self-limiting and respond well to medical therapy.
Supportive Care:
Education for parents and school personnel regarding the condition, seizure precautions, and medication adherence
Psychological support for the child and family
Regular follow-up appointments to monitor seizure control, side effects, and developmental progress
Safe sleep practices are recommended for children with BRE.
Prognosis
Factors Affecting Prognosis:
For BRE: Generally excellent, with seizure remission occurring by adolescence in most cases
Factors that may indicate a less favorable prognosis include earlier onset, more frequent seizures, and occurrence of non-rolandic seizure types
For Absence Seizures: Generally good, with most children achieving seizure control with AEDs
However, some may develop generalized tonic-clonic seizures or continue to have absence seizures into adulthood.
Outcomes:
Benign Rolandic Epilepsy: Seizures typically resolve by late adolescence, with normal cognitive and neurological outcomes
Absence Seizures: Good prognosis for seizure control, but recurrence is possible
Long-term academic performance may be affected by the frequency of spells before diagnosis and treatment.
Follow Up:
Regular clinical and EEG follow-up is recommended, particularly during the titration of AEDs and as children approach the age of potential remission
For BRE, follow-up is typically continued until adolescence
For absence seizures, long-term follow-up may be necessary to monitor for recurrence or development of other seizure types.
Key Points
Exam Focus:
Differentiate EEG findings: Centrotemporal spikes in BRE vs
generalized 3-Hz spike-and-wave in absence seizures
Recognize the typical seizure semiology for each
Understand first-line AEDs and their indications/contraindications for each syndrome.
Clinical Pearls:
Always consider sleep EEG for suspected BRE
Hyperventilation is a key activator for absence seizures on EEG
Avoid carbamazepine/oxcarbazepine in absence seizures
Reassurance for parents is paramount for BRE
focus on quality of life for absence seizures.
Common Mistakes:
Misinterpreting EEG findings
Prescribing AEDs that can worsen absence seizures
Over-investigating with MRI in typical cases of BRE or absence seizures
Not addressing parental anxiety effectively, especially in BRE.