BECTS (Benign Epilepsy with CentroTemporal Spikes) Seizures / Benign Rolandic Epilepsy of Childhood

Seizures are super scary for a parent to witness, but not all seizures mean danger. There is actually a diagnosis of seizures that has “BENIGN” in the name! Have you ever had that feeling of falling that rapidly wakes you from sleep? While it’s unlikely that you were having Benign Epilepsy with CentroTemporal Spikes (BECTS) seizures or a benign Rolandic seizure, that tenuous state between sleeping and waking is a time when some odd neurologic things can happen, including BECTS. We have previously talked about other seizure presentations such as Non-Convulsive Status Epilepticus, Gelastic Seizures, Pyridoxine-Dependent Seizures, and Neonatal Seizures. We have even talked about using a ketogenic diet to control seizures, and seizure mimics. But what is this “benign” seizure disorder of which I speak? Let’s learn more about BECTS or Benign Rolandic Seizures:

BECTS Seizures / Benign Rolandic Epilepsy- Basics

Known as:
- Benign Epilepsy with CentroTemporal Spikes (BECTS)
- Benign Epilepsy of Childhood with CentroTemporal Spikes (BECCTS)
- Benign Rolandic Epilepsy (BRE)
- Childhood Epilepsy with CentroTemporal Spikes (CECTS)
The Rolandic area of the brain controls movements of face and oropharynx
“Benign” means that they grow out of it by adolescence, and it typically doesn’t cause any long term effects
Can happen while awake or asleep
- When asleep the seizures depend on the sleep cycle

BECTS Seizures / Benign Rolandic Epilepsy- Etiology and Epidemiology

Accounts for 15% of epilepsy in children
- Most common epilepsy syndrome of childhood
Usually starts to affect children ages 6-8 years
- Can start as young as 1 year old or up to 14 years old
- Peaks at 7-10 years of age
- 95% resolve by 16 years of age
- More frequently in children with family members with epilepsy
Prevalence 10-20 per 100,000 children
Most will only have 10 or fewer seizures total
- Up to 20% will only have ONE seizure
- However earlier seizure onset may portend more seizures or longer period of seizure activity
Adults are not affected
Often considered to be associated with a genetic disorder as 25% have a family member with febrile seizures or epilepsy
- Suspect autosomal dominant transmission
- No ONE specific genetic mutation known yet
  - Chromosome 11p13 and chromosome 15p14 are implicated
  - Mutations of KCNQ2, ELP4, FOXP2, and GRIN2A in families with BRE (among others)

BECTS Seizures / Benign Rolandic Epilepsy- Presentation and Exam

Typically happens when sleeping (70%)
When sleeping, can start with facial twitching, then generalize to generalized tonic/clonic movements
- Happens shortly after child goes to sleep or before child wakes up
- Can happen during daytime naps
When awake, symptoms include twitching, numbness, tingling on one side of face or tongue
- Interferes with speech and can cause speech arrest
- Can cause drooling, gurgling noises, hypersalivation
- Child is fully awake and aware
- <2 minutes duration usually, up to 3, but always are very brief, and not frequent
- Can spread to ipsilateral arm/leg
- Can generalize as well
  - Bilateral stiffening and shaking
  - Loss of bowel/bladder
  - Loss of consciousness
  - Post-ictal state
Status epilepticus and sudden unexplained death in epilepsy (SUDEP) are rare

BECTS Seizures / Benign Rolandic Epilepsy- Diagnosis

Diagnosed by EEG – needs awake and asleep recording
- Slow, biphasic discharges in centrotemporal area
- Positive discharges frontal area
- High voltage centrotemporal sharp spike discharges followed by slow waves
- Sharp spikes in repetitive bursts
- Sometimes will only see spikes during sleep, so must get sleep recording
- Non-REM (NREM) sleep most clearly demonstrates the epileptiform activity
- Background EEG activity is otherwise normal
MRI and other brain imaging typically normal
- Not necessary to do if EEG is classic and typical
- If done, should be with and without contrast to evaluate for structural lesion
Differential includes the following. These are considered different diagnoses, but many are part of the same spectrum.
- Centrotemporal spikes without seizures
- Centrotemporal spikes with a brain lesion
- Temporal lobe epilepsy
- Panayiotopoulos syndrome
- Landau-Kleffner syndrome
- Atypical benign childhood focal epilepsy (ABCFE)
- Status epilepticus of BECTS (SEBECTS)
- Epileptic encephalopathy with continuous spike-and-wave during sleep (CSWS)

BECTS Seizures / Benign Rolandic Epilepsy- Treatment

Often no antiepileptics are needed if seizures infrequent or only at night/while sleeping
May be prescribed antiepileptics if:
- Daytime seizures
- Frequent seizures
- Frequent discharges while sleeping
- Classic BECTS/CECTS/BRE seizures and a learning disorder
- Cognitive problems with these seizures
Keppra and carbamazepine most common antiepileptics prescribed. Also,
- Oxcarbazepine
- Valproate
- Phenytoin
- Gabapentin
- Zonisamide
- Lacosamide
Seizures do respond well to antiepileptics
- Typically only need one medication
One study shows there may be some benefit to using antiepileptics with [Tsai 2024]
- An increase in global efficiency
- Improvement in clustering coefficient
- Improved nodal strength
Most children only have seizures for 2-4 years and then get better
- Medications can be weaned and withdrawn under guidance of physician
- May recur after meds withdrawn, but meds can be restarted
- 95% of cases resolve by 15-16 years old

BECTS Seizures / Benign Rolandic Epilepsy- Complications

Children usually continue to develop normally despite these seizures
Sometimes can have learning difficulties and behavioral problems during the time they have seizures
- Issues usually resolve once seizures stop and EEG goes back to normal
Can have migraines or headaches
Sometimes difficult to differentiate between typical BECTS and other seizure disorders because of many similarities with other epilepsy disorders, and the centro-temporal spikes can be seen in other diagnoses/situations, so the EEG may be indeterminate.
There are associated co-morbiditiested [Lee 2017, Steinrueke 2023]

BECTS Seizures / Benign Rolandic Epilepsy- Moral of the Morsel

It’s not a stroke, it’s a seizure. Symptoms can be unilateral and involve the face and tongue, but it’s not a stroke! Symptoms should be short-lived and the patient remains awake and cognizant.
I’m down with EEG, yeah you know me! EEG may be the only testing needed to diagnose classic BECTS.
To medicate or not to medicate? Most children don’t need medications, but know the situations that may benefit from antiepileptics.
I have BECTS and… BECTS can be associated with other neurodevelopmental comorbidities, so they may benefit from enhancing screening and monitoring once diagnosed with BECTS.

References:

Hernandez, A., Holmes, G.L. and Fisher, R. (2015) Childhood epilepsy centrotemporal spikes (benign Rolandic Epilepsy), Epilepsy Foundation. Edited by E. Wirrell. Available at: https://www.epilepsy.com/what-is-epilepsy/syndromes/childhood-epilepsy-centrotemporal-spikes (Accessed: 14 November 2024).
Amrutkar CV, Riel-Romero RM. Rolandic Epilepsy Seizure. [Updated 2023 Aug 7]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK534845/
Lee YJ, Hwang SK, Kwon S. The Clinical Spectrum of Benign Epilepsy with Centro-Temporal Spikes: a Challenge in Categorization and Predictability. J Epilepsy Res. 2017 Jun 30;7(1):1-6. doi: 10.14581/jer.17001. PMID: 28775948; PMCID: PMC5540684.
Tsai ML, Wang CC, Wang AY, et al. Antiseizure Medications Normalize Electroencephalographic Functional Connectivity and Power in Children With Benign Epilepsy With Centrotemporal Spikes. Pediatr Neurol. 2024;156:41-50. doi:10.1016/j.pediatrneurol.2024.03.015
Steinruecke M, Gillespie C, Ahmed N, et al. Care and three-year outcomes of children with Benign Epilepsy with Centro-Temporal Spikes in England. Epilepsy Behav. 2023;148:109465. doi:10.1016/j.yebeh.2023.109465