Benign Epilepsy of Childhood with Rolandic Spikes
An Overview for Parents
BY MICHAEL SHEVELL, MD, CM, FRCPC, ASSOCIATE PROFESSOR, DEPARTMENTS OF NEUROLOGY/NEUROSURGERY & PEDIATRICS, MCGILL UNIVERSITY, DIVISION OF PEDIATRIC NEUROLOGY, MONTREAL CHILDREN'S HOSPITAL-MCGILL UNIVERSITY HEALTH CENTER, MONTREAL, QUEBEC
A major goal of a child's first evaluation for epilepsy is to find out whether they have an epileptic syndrome. About half of all children with epilepsy have a specific epileptic syndrome. These syndromes share a core of features that include:
• age of onset
• type of seizure
• family history
• physical examination
• and results of investigations, e.g., electroencephalogram (EEG ) and neuro-imaging studies
The recognition of a specific epileptic syndrome guides the selection of appropriate medication and shapes expectations about the child's prognosis.
The most common epilepsy syndrome in children is known by a plethora of names: benign epilepsy of childhood with rolandic spikes (BECRS), benign epilepsy of childhood with central temporal spikes (BECTS), benign childhood epilepsy with central temporal spikes (BCECTS), benign rolandic epilepsy (BRE), sylvian seizures, and rolandic epilepsy. All of these terms refer to the same entity.
DIAGNOSIS
Children with BECRS usually have their initial seizure between 3 and 10 years of age. Most often, the first seizure occurs between 5 and 8 years of age. These children experience two different types of seizures: partial and generalized.
Partial seizures may occur while awake or asleep and involve the face and mouth region. The mouth pulls to one side, one cheek may twitch repetitively, and the child may salivate excessively or be unable to speak. If awake, the child does not lose consciousness but remains aware and responsive to directions.
Generalized seizures only occur while asleep, usually prior to awakening. Often parents hear unusual sounds from the child's bedroom and, on arrival, find that the child is having a "grand mal" type of seizure.
Children with BECRS have an entirely normal, age-appropriate, neurodevelopmental examination. They may or may not have a family history of epilepsy in a first- or seconddegree relative. Appropriate investigations include an EEG and neuro-imaging study, e.g., a CT scan.
The EEG abnormality in BECRS consists of isolated central temporal spikes, which may occur on one side exclusively or independently on both sides of the brain. The spikes are enhanced during sleep. The EEG background is typically normal, as are neuro-imaging studies.
TREATMENT
Treatment for the child with BECRS is individualized. Many children with BECRS experience only a single seizure, even when no medical treatment is given. For this reason, most physicians wait for a second seizure to occur before starting any medical treatment.
The spectrum of anticonvulsants is thought to be equally effective in controlling this particular seizure type. In Canada, carbamazepine and clobazam are most often prescribed.
The overwhelming majority of children with BECRS (80%) are controlled by a single medication. In fact, when seizures continue despite medication, prompt and careful reconsideration of the accuracy of the original diagnosis is recommended.
The standard of practice is to treat BECRS until the child is seizure-free for two years, before tapering off the medication. Often the physician repeats an EEG before deciding to halt medication.
The risk of seizure recurrence varies and depends on several factors, such as:
• the number of seizures prior to starting medication
• the ease of seizure control age
• the continued presence of EEG abnormalities.
Typically, the risk of recurrence varies from 20% to 30%. Most recurrences (75% to 90%) occur within the first year off medication.
In addition to seizure control, other aspects of management include careful attention to the child's performance in school, monitoring for potential side effects of prescribed medication and encouragement of full participation in leisure, recreational and social activities. Seizure precautions in this setting make common sense and need not ever be an excuse to deny a child's participation in desired activities.
PROGNOSIS
The prognosis for children with BECRS is excellent. It is indeed a developmental epilepsy that has a beginning and end. Resolution of seizures is almost invariable as the child enters puberty and adolescence.
The vast majority of children with BECRS are indistinguishable from their peers without epilepsy from the age of 15 and into adulthood. It is this normality that parents must keep in the mind when BECRS is first diagnosed and during the need for medical management.
Printed with permission of Epilepsy Canda.
