Benign rolandic epilepsy (BRE)

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Other Names:

Benign rolandic epilepsy of childhood (BREC); Benign epilepsy with centro-temporal spikes (BECTS); Benign epilepsy of childhood with centrotemporal spikes (BECCT)

Categories:

Nervous System Diseases

Summary Summary

Benign rolandic epilepsy (BRE) is the most common form of childhood epilepsy. It is referred to as "benign" because most children outgrow the condition by puberty. This form of epilepsy is characterized by seizures involving a part of the brain called the rolandic area.^[1] These seizures typically begin between the ages of 3 and 12 years and occur during the nighttime.^[2] Other features of BRE include headaches or migraines and behavioral and/or learning differences.^[3] BRE is thought to be a genetic disorder because most affected individuals have a family history of epilepsy.^[3]^[4] Treatment for BRE may depend on the symptoms and severity in each person. Because BRE resolves on its own before adulthood, many children with BRE who have infrequent seizures that only occur at night do not take anti-epileptic drugs (AEDs).^[1] However, there have been studies suggesting that BRE may cause lasting cognitive or behavioral problems in some people.^[5] Medication is more likely to be recommended in children with frequent or daytime seizures, cognitive impairment, or a learning disorder.^[1] Each family must consult with their physician(s) and make their own decision about whether to treat BRE.^[3]

Last updated: 6/4/2018

Symptoms Symptoms

BRE typically begins between the ages of 3 and 13 years with nighttime seizures. The episodes usually begin with twitching and stiffness in the face, that often wakes up the individual.^[6] There may be a tingling feeling on one side of the mouth that involves the tongue, lips, gums and inside of the cheek.^[6]^[7] The seizure can also involve the throat, which may make speech unclear and difficult to understand.^[7] Occasionally, both sides of the body may be affected, which can lead to stiffness and jerking movements of the arms and legs, and loss of consciousness. Loss of bladder control (incontinence) may also occur.^[7]

Some individuals with BRE experience headaches or migraines, learning difficulties, and behavioral problems during the period of time that they have seizures. In many children, once seizures stop and brain activity returns to normal, these issues resolve.^[1]^[5] However, there have been studies suggesting that cognitive or behavioral problems may persist in some people.^[5] More studies regarding whether there is an increased chance of long-term impairments in those with BRE are needed.^[8]^[5]

Last updated: 6/4/2018

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Showing of 4 |

Medical Terms	Other Names	Learn More: HPO ID
Percent of people who have these symptoms is not available through HPO
Bilateral tonic-clonic seizure with focal onset		0007334
EEG with centrotemporal focal spike waves		0012557
Nocturnal seizures		0031951
Sporadic	No previous family history	0003745

Showing of 4 |

Do you have more information about symptoms of this disease? We want to hear from you.

Last updated: 2/1/2021

Do you have updated information on this disease? We want to hear from you.

Cause Cause

BRE is thought to be a genetic disorder. Studies suggest that certain regions on chromosome 11 (11p13) and chromosome 15 (15q14) may be involved in BRE, but a specific gene has not been identified.^[4]

Last updated: 7/26/2016

Treatment Treatment

Treatment for BRE may depend on the symptoms and severity in each person. In general, BRE typically does not require intensive therapy.^[3] Because seizures may be infrequent and usually occur at night, and because of the potential side affects of anti-epileptic drugs, many children with BRE do not take medication.^[1]^[9] However, emerging data on neuropsychological problems in people with BRE suggests that the syndrome may not be entirely without long-term effects.^[9] A recently recognized concern in children with BRE is a higher incidence of neuropsychological deficits.^[9] Each family must consult with their physician(s) and make their own decision about whether they are more comfortable treating or not treating BRE.^[3] The need for medication is generally bigger if a child has frequent seizures, daytime seizures, cognitive problems, or a learning disorder.^[1]

When BRE is treated, medications may include AEDs such as carbamazepine, gabapentin, levetiracetam, or others. Most children with BRE respond to a low dose of a single drug, but some have seizures that are more drug-resistant, requiring higher doses or more than one drug.^[3]

Last updated: 6/4/2018

Find a Specialist Find a Specialist

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

To find a medical professional who specializes in genetics, you can ask your doctor for a referral or you can search for one yourself. Online directories are provided by the American College of Medical Genetics and the National Society of Genetic Counselors. If you need additional help, contact a GARD Information Specialist. You can also learn more about genetic consultations from MedlinePlus Genetics.

Research Research

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

The Centers for Mendelian Genomics program is working to discover the causes of rare genetic disorders. For more information about applying to the research study, please visit their website.

Organizations Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

Epilepsy Action
New Anstey House Gate Way Drive
Yeadon, LEEDS LS19 7XY
United Kingdom
Telephone: 0113 210 8800 (UK) or +44 (0)113 210 8800 (international)
Fax: 0113 391 0300 (UK) or +44 (0)113 391 0300 (international)
E-mail: epilepsy@epilepsy.org.uk
Website: https://www.epilepsy.org.uk/

Organizations Providing General Support

Epilepsy Foundation
8301 Professional Place East
Suite 230
Landover, MD 20785
Toll-free: 800-332-1000 (24/7 Helpline)
Telephone: +1-301-459-3700
Fax: +1-301-577-2684
E-mail: contactus@efa.org
Website: https://www.epilepsy.com/
en Español 1-866-748-8008

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Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

The Epilepsy Foundation has an information page on benign rolandic epilepsy. Click on Epilepsy Foundation to view the information page.
MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.

In-Depth Information

Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine.
PubMed is a searchable database of medical literature and lists journal articles that discuss Benign rolandic epilepsy (BRE). Click on the link to view a sample search on this topic.

News & Events News & Events

News

Rare Disease Day at NIH 2021
March 1, 2021

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References References

Holmes GL, Fisher RS, Hernandez A. Benign Rolandic Epilepsy. Epilepsy Foundation. February 2, 2015; http://www.epilepsy.com/learn/types-epilepsy-syndromes/benign-rolandic-epilepsy.
Mellish LC, Dunkley C, Ferrie CD, Pal DK. Antiepileptic drug treatment of rolandic epilepsy and Panayiotopoulos syndrome: clinical practice survey and clinical trial feasibility. Arch Dis Child. January 2015; 100(1):62-67. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4283698/.
Kaddurah AK. Benign Childhood Epilepsy. Medscape Reference. December 23, 2015; https://emedicine.medscape.com/article/1181649-overview#a7.
Kniffen CL, Lopez A, McKusick VA. Centralopathic Epilepsy. Online Mendelian Inheritance in Man (OMIM). September 9, 2015; http://www.omim.org/entry/117100.
Camfield C, Camfield P. Cognitive Disabilities and Long-term Outcomes in Children with Epilepsy: A Tangled Tail. Send to Semin Pediatr Neurol. November, 2017; 24(4):243-250. https://www.ncbi.nlm.nih.gov/pubmed/29249504.
Blumstein MD, Friedman MJ. Childhood Seizures. Emerg Med Clin N Am. November 2007; 25(4):1061-86. https://www.ncbi.nlm.nih.gov/pubmed/17950136.
Appleton R. Benign rolandic epilepsy. Epilepsy Action. November 2016; http://www.epilepsy.org.uk/info/benign.html.
Verrotti A, Matricardi S, Di Giacomo DL, Rapino D, Chiarelli F, Coppola G. Neuropsychological impairment in children with Rolandic epilepsy and in their siblings. Epilepsy Behav. July, 2013; 28(1):108-112. https://www.ncbi.nlm.nih.gov/pubmed/23708147.
Kim H, Kim SY, Lim BC, et al. Spike persistence and normalization in benign epilepsy with centrotemporal spikes - Implications for management. Brain Dev. May, 2018; [Epub ahead of print]: