What is Rolandic Epilepsy Seizure?
Benign Rolandic epilepsy (BRE) is also known as benign epilepsy with centrotemporal spikes (BECTS) or benign epilepsy of childhood with centrotemporal spikes (BECCT). It’s the most common type of epilepsy found in children. The good news is that most kids who have this condition often grow out of it by the time they hit puberty, which is why it’s referred to as “benign,” meaning harmless in the long run. The seizures caused by this condition start in the Rolandic area of the brain, which is situated around the central brain groove, also known as the centrotemporal area. This area can be found around the Rolandic fissure in the brain. See Figure 1.
What Causes Rolandic Epilepsy Seizure?
Benign Rolandic epilepsy (BRE) is considered a hereditary condition because about 25% of people with this disease have relatives who also had febrile seizures or epilepsy. The thought is that this condition is passed down in families through what we call an “autosomal dominant” pattern.
In simpler terms, this means a parent with the condition has a 50% chance of passing it onto their children. There seems to be agreement among scientists and doctors that genes play a significant role in this disease.
While no specific gene has been definitively linked to BRE, two areas in our genetic material – one on chromosome 11 and the other on chromosome 15 – are suspected to have a connection.
Additionally, changes in certain genes named KCNQ2, ELP4, and GRIN2A have been identified in families who have members affected with BRE. However, research is still ongoing to definitively ascertain the exact genes responsible for causing this condition.
Risk Factors and Frequency for Rolandic Epilepsy Seizure
Benign Rolandic epilepsy, or BRE, typically starts in children between the ages of 1 to 14 years old, with the majority of cases happening around 7 to 10 years old. Boys tend to have BRE more often than girls, with a ratio of 1.5 boys for every 1 girl affected. The condition is not uncommon – about 10 to 20 in every 100,000 children up to the age of 15 years will have BRE. This means it accounts for about 15% of all cases of epilepsy in children, making it the most common epilepsy syndrome in childhood. Importantly, adults don’t get this condition.
- Benign Rolandic epilepsy (BRE) usually starts between the ages of 1 to 14 years.
- The majority of cases occur between 7 to 10 years old.
- Boys are more likely to have BRE than girls, with a ratio of 1.5 to 1.
- Up to age 15, about 10 to 20 per 100,000 children have BRE.
- BRE is the most common epilepsy syndrome in childhood, making up about 15% of all epilepsy cases in children.
- Adults are not affected by BRE.
Signs and Symptoms of Rolandic Epilepsy Seizure
Benign Rolandic epilepsy, often abbreviated as BRE, predominantly affects children and typically stops by adolescence. These seizures often happen at night or upon waking up and are more common than 70% of the time. They occur in the Rolandic area of the brain, which impacts the face and the back of the mouth.
These seizures are generally irregular and partial, or limited in nature, because they originate from the Rolandic area. This area of the brain controls the face and the back area of the mouth, and these seizures do not cause a loss of consciousness. Symptoms are usually seen on one side of the body and include facial twitching, stiffening, feelings of numbness or tingling in the face and throat (including the tongue, lips, gums, inside of the cheek, and teeth). This often leads to difficulty speaking, which might sound like gurgling noises, and can cause a halt in speech, excessive drooling, and over-production of saliva. These seizures tend to be brief, often lasting only about 2 to 3 minutes. The twitching in the face can sometimes spread to the same side arm and leg.
These seizures might spread, and on occasion can impact both sides of the body, becoming generalized. The symptoms may also evolve into widespread shaking, stiffening, losing control of bowel or bladder, loss of consciousness, and a state of confusion after the seizure. Since many of these seizures are partial and occur at nighttime, they go unnoticed until there is a major generalized seizure.
Severe conditions like extended seizures (status epilepticus) and sudden unexplained death in epilepsy are quite rare. Children with BRE may have related symptoms like frequent headaches or migraines along with learning and behavioral issues. These associated symptoms are more commonly seen when the child is having seizures and if there are anomalies in the brain’s electrical activity, as shown in an EEG, or electroencephalogram.
These difficulties can improve over time as the child matures and the seizures dissipate. This usually happens by the time the child reaches 15 years of age, and the EEG normalizes. Typically, no developmental issues are seen in patients of BRE over the long term.
Testing for Rolandic Epilepsy Seizure
If your doctor suspects that your child has Benign Rolandic Epilepsy (BRE), they will make a diagnosis mainly based on your child’s medical history, and confirming it with a specific brain wave test called an electroencephalogram (EEG). Typically, the EEG shows a unique pattern of slow, large, sharp spikes in two different areas of the brain—the centrotemporal area and the frontal area. These spikes usually appear in short repetitive bursts and are typically on one side of the brain but can sometimes appear on both sides. It’s important that the EEG captures brain activity when your child is awake and asleep. Sleep recording is important because these remarkable patterns or spikes sometimes only show up when your child is sleeping. The EEG typically shows the most useful information while your child is in non-rapid eye movement (or non-REM) sleep.
Aside from this, the EEG normally appears as a typical pattern when your child is sleeping and awake. Simple means of evaluating your child’s movement and development are generally normal in children with BRE.
Even though imaging tests like an MRI of the brain (with and without contrast) may be considered to exclude other structural problems in the brain, they are often not required. Diagnosis is often determined solely through your child’s history and the distinctive EEG results. However, if your child’s history includes atypical features and the EEG results are not clearly indicative, further investigations with imaging and laboratory studies might be considered. Remember that while the EEG findings mentioned earlier are characteristic of BRE, they can, in rare cases, be seen in children without any symptoms or with other forms of epilepsy.
Treatment Options for Rolandic Epilepsy Seizure
Benign Rolandic epilepsy (BRE) is usually a mild condition. Seizures from BRE tend to stop by the time a person reaches adolescence, and often, they don’t require treatment, especially if the seizures aren’t that common, only happen at night, and only impact a part of the body (partial seizures). The decision to treat or not usually depends on whether the seizures happen often, are severe, occur during the day, affect the whole body (generalized seizures), or are linked to changes in speech, cognitive function, or learning abilities. It’s also important for the patient and their family to feel comfortable with the decision.
If treatment is deemed necessary, the typical approach is to use a single anti-epileptic drug (AED). This is a type of medication that decreases the chances of having seizures. In rare cases, more than one AED may be needed. Some doctors may suggest taking the medication only at night time.
The first drug that is usually considered for treatment is carbamazepine. Other possible options include oxcarbazepine, gabapentin, levetiracetam, valproate, phenytoin, lacosamide, and zonisamide.
Treatment doesn’t generally last for long periods but can be stopped after experiencing 1 to 2 years without seizures. Doctors might use information from an electroencephalogram (EEG) – a test that measures electrical activity in the brain – to help decide when to lower the dose or stop the AEDs completely.
Finally, it’s good for the patient and their family to know that seizures can sometimes come back after stopping the AEDs, so it’s important to stay aware and alert.
What else can Rolandic Epilepsy Seizure be?
When a doctor is trying to diagnose a patient, they might consider other conditions that have similar symptoms. These are known as ‘differential diagnoses’. For this particular case, the conditions they might consider include:
- Centrotemporal spikes without seizures,
- Centrotemporal spikes with a cerebral lesion,
- Temporal lobe epilepsy,
- Panayiotopoulos syndrome, and
- Landau-Kleffner syndrome.
What to expect with Rolandic Epilepsy Seizure
If a child has been diagnosed with benign Rolandic epilepsy (BRE), they generally have a very good outlook, regardless of whether they receive treatment or not. The seizures typically happen for 2 to 4 years and then stop on their own by the time the child reaches 15 to 16 years of age. This happens in more than 95% of children with BRE.
Most kids with BRE have less than 10 seizures. In fact, between 10% to 20% of these kids will only ever have one seizure in their entire life. If a child starts having seizures at a younger age, they might have more seizures or have seizures for a longer period. It’s important to note that early onset seizures can cause cognitive, behavioral, and speech abnormalities, but these tend to clear up by the time the child reaches their teenage years as the seizures come to an end.
Lastly, BRE can sometimes be an early sign of other types of epilepsy.
Preventing Rolandic Epilepsy Seizure
Like other epilepsy conditions, it’s crucially important for patients and their families to learn as much as they can to achieve the best possible health outcome. This education becomes extremely important when it comes to making decisions about starting or stopping a treatment. In addition, the patient and their families should be informed about the potential of a severe, continuous seizure known as status epilepticus, and something called sudden unexplained death in epilepsy (SUDEP), which is where a person with epilepsy unexpectedly passes away without an obvious cause.
