Benign Rolandic Epilepsy (BRE). Benign Childhood Epilepsy with Rolandic (Centrotemporal) Spikes (BECRS). Sylvian Seizures.
Incidence
An autosomal dominant inheritance with age dependency and variable penetrance has been reported, although it is most likely idiopathic. Approx 40 % of children have a family history of BRE. Possible susceptibility region on chromosome 15q14. There is a slight male predominance. An association with ELP4 (Elongator Protein Complex 4) has been identified. It accounts for 15-20% of all childhood epilepsies.
Clinical Characteristics
BRE begins between 3 and 13 years of age. In 80% of patients, seizures appear between 5 and 10 years of age and they usually recover before age 16. Most of the seizures reflect discharges in the precentral and postcentral gyri in the suprasylvian region with motor, sensory, and autonomic manifestations in the face, mouth, and throat. The five criteria for the diagnosis of BRE are: onset between the ages of 3 and 13, a spike focus located in the centrotemporal area with normal background activity on the interictal EEG, absence of neurologic or intellectual deficit before the onset, partial seizures with motor signs, frequently associated with somatosensory symptoms or precipitated by sleep, and spontaneous remission before the age of 18. Seizure manifestations include drooling, guttural sounds, involuntary movements or tonic contractions of the tongue or jaw, unilateral paresthesia of the tongue, lips, gums, and cheek, anarthria, and myoclonic contraction of one side of the face. Sensorimotor phenomena involving a leg, or half body, and miscellaneous symptoms such as abdominal pain, can also occur. It is considered an epileptic syndrome.
Precipitants
As in any seizure disorder, lack of sleep, tiredness, withdrawal of medication, etc, may precipitate an attack.
Provocation Tests
The discharge rate is increased in drowsiness and in all stages of sleep, and in about one third of children, the spikes appear only in sleep. Sleep EEG should be done in every patient, especially when deciding about stopping treatment.
Diagnostic Procedures
It is a clinical-EEG diagnosis. The diagnosis can be confirmed when the characteristic centrotemporal spikes are seen on EEG. The discharge rate is increased in drowsiness and in all stages of sleep, and in about one third of children, the spikes appear only in sleep.