Benign rolandic epilepsy of childhood (BRE), also known as benign epilepsy with centrotemporal spikes, is the most common epilepsy syndrome seen in children. BRE accounts for approximately 15 percent of all types of childhood epilepsy disorders. In the general population, the incidence of BRE is about 10 to 20 cases per 100,000 children.
The term "benign" alludes to the fact that children with BRE typically grow out of having seizures during adolescence. The term "rolandic" refers to the rolandic area of the brain, where BRE seizure activity comes from. This region specifically controls motor movements.
BRE has an early onset and can start in children as young as age 1, although most cases become apparent between ages 6 and 8. It is also slightly more likely to emerge in boys than in girls. BRE is currently believed to have a genetic component, as most children living with the disorder have a family history of epilepsy.
BRE is a seizure, or epilepsy, disorder. BRE begins with nighttime seizures in children who are typically between the ages of 3 and 12. Because the seizures originate in the rolandic area of the brain, the mouth and voice box (larynx) can be affected. Symptoms associated with BRE seizures include:
BRE seizures can also spread to both parts of the brain. This can cause generalized tonic-clonic seizures, with jerking movements and muscle contractions of the arms and legs.
There are also other problems associated with the onset of BRE. These include:
Learning difficulties and headaches are often related to the fact that children with BRE are waking up throughout the night due to their seizures. Therefore, poor sleep is a contributor to these problems. These headaches and behavioral issues also typically resolve once the child grows out of having seizures.
The causes of BRE have been debated and are not well understood. In the field of neurology, many doctors believe that BRE may have a genetic component. One study has found that BRE appears to be linked to the ELP4 gene, which codes for a protein known as elongator protein complex 4. It is the only gene that has been linked to BRE thus far. The ELP4 gene influences brain development, and mutations in this gene seem to result in susceptibility to seizures and neurodevelopmental disorders. However, other research has failed to show such an association between BRE and this gene.
More recent research has shed new light on the genetic risk factors of BRE. One study demonstrated that many genetic changes might influence susceptibility to BRE. These genes include GRIN2A and PRRT2. However, twin studies suggest there is no genetic component associated with BRE. Regardless, scientists agree that the genetics of BRE are complex.
The environment may also play a role in the development of BRE. However, these risk factors are yet to be defined. More research is needed to better understand the cause of BRE.
A doctor will make a diagnosis of BRE based on the description of the seizures, the age of the child, and a physical exam. Neurologists, physicians specialized in treating seizures, may also gather information from tests such as electroencephalogram (EEG) recordings. An EEG is a medical test that depicts brain wave abnormalities by attaching electrodes to the scalp. In children with BRE, an EEG will show spikes in the centrotemporal regions of the brain. Centrotemporal refers to the part of the brain where these seizures start. The centrotemporal area is also known as the central sulcus, which was previously known as the Rolandic sulcus, hence the name of the disorder.
If the diagnosis is not BRE, an MRI scan will sometimes be used to rule out other possible diagnoses.
Sometimes, children are prescribed antiepileptic drugs — such as Keppra (levetiracetam) — for the treatment of BRE seizures, if they are frequent or occur during the daytime. Other antiepileptic medications used for those with BRE include Tegretol (carbamazepine), Trileptal (oxcarbazepine), and Neurontin (gabapentin). However, most children with BRE have rare seizures and therefore do not need daily medication. These medications have many side effects, and the benefits must outweigh the risks.
Research shows that antiepileptic medication does reduce generalized seizures — but not focal seizures. Research also shows that medication may not be required in the long term. One study showed that children with BER who did not take antiepileptic medication stopped having seizures after four to 14 years and more than 900 seizures.
Although these seizures eventually go away, the choice of whether or not to put your child on antiepileptics should be made after careful consideration with a doctor.
It is generally thought that children with BRE have a very good prognosis (outlook), even without medication. In cases of BRE, most children stop having seizures within just a few years after they begin. In almost every case, complete remission is seen by age 15 with no impairments. Children seem to suffer no long-term negative consequences and have normal development.
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