Benign rolandic epilepsy (BRE)

Alternate names

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

Definition

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.

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.

Inheritance

BRE is thought to be a genetic disorder because most affected individuals have a family history of epilepsy.

Signs and symptoms

• The episodes usually begin with twitching and stiffness in the face, that often wakes up the individual.
• There may be a tingling feeling on one side of the mouth that involves the tongue, lips, gums and inside of the cheek.
• The seizure can also involve the throat, which may make speech unclear and difficult to understand.
• 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.
• 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.
• However, there have been studies suggesting that cognitive or behavioral problems may persist in some people.
• More studies regarding whether there is an increased chance of long-term impairments in those with BRE are needed.

Diagnosis

• The diagnosis can be confirmed when the characteristic centrotemporal spikes are seen on electroencephalography (EEG).
• Neuroimaging, usually with an MRI scan, is only advised for cases with atypical presentation or atypical findings on clinical examination or EEG.

Treatment

• Treatment for BRE may depend on the symptoms and severity in each person.
• In general, BRE typically does not require intensive therapy.
• 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.
• However, emerging data on neuropsychological problems in people with BRE suggests that the syndrome may not be entirely without long-term effects.
• A recently recognized concern in children with BRE is a higher incidence of neuropsychological deficits.
• Each family must consult with their physician(s) and make their own decision about whether they are more comfortable treating or not treating BRE.
• The need for medication is generally bigger if a child has frequent seizures, daytime seizures, cognitive problems, or a learning disorder.
• 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.

NIH genetic and rare disease info

• NIH info on Benign rolandic epilepsy (BRE)

Benign rolandic epilepsy (BRE) is a rare disease.