Epilepsy, Benign Rolandic

1. Occurs between 2-14 y.o. (peak onset at 9-10 y.o.)
2. Excellent prognosis...most resolve by age 14
3. Partial seizure with symptoms most often confined to the face with +/-impaired consciousness
4. Usually happens on awakening from sleep
5. EEG shows repetitive spikes in the centrotemporal area (diagnostic)
6. Treat only if seizures are frequent; best drug is Carbamazepime

While asleep one night, a 10-year-old boy experiences unilateral tonic-clonic contractions of the face and arm that his parents observe. This activity generalizes after 1 minute. Past history is otherwise normal. Later in the emergency department, results of the boy’s physical examination are normal. Outpatient electroencephalography (EEG) shows repetitive centrotemporal spikes with normal background activity.

The International Classification of Epileptic Seizures categorizes seizures by type: generalized (tonic, clonic, tonic-clonic, myoclonic, absence, and atonic), partial (simple and partial), and epilepsy syndromes. Epilepsy syndromes in children include infantile spasms, Lennox-Gastaut syndrome, juvenile myoclonic epilepsy, and perhaps the most common, rolandic epilepsy.

Rolandic epilepsy, also known as benign epilepsy of childhood with centrotemporal spikes, accounts for approximately 10% of childhood seizure disorders. This syndrome occurs in healthy children who have an unremarkable past history and normal findings on neurologic examination, such as the boy described in the vignette. The syndrome is characterized most often by nocturnal seizures that develop late in the first decade. The child is awakened by unilateral tonic-clonic contractions, often in the face, but sometimes involving the extremities. Tonic-clonic activity may become bilateral. Electroencephalography, particularly after sleep deprivation, demonstrates unilateral or bilateral repetitive centrotemporal spike discharges with a normal background.

Because rolandic epilepsy almost always remits by adolescence, occasional seizures require no therapy. In fact, 20% of children experience only one seizure. Very frequent, disruptive seizures may be treated, but there is no clear therapy of choice. Neurologists sometimes administer carbamazepine, valproic acid, or even gabapentin. The anticonvulsant is discontinued after puberty when seizures remit. Absence seizures are not associated with the rolandic epilepsy syndrome.

The ketogenic diet is a meal plan that is restricted to fats and protein, with limited carbohydrates, to induce ketosis. The mechanism by which this controls seizures in children is unclear. The diet typically is indicated for children who have generalized seizure disorders that are refractory to polytherapy or patients who experience intolerable side effects from anticonvulsants.

References:
CHLA Board Review 2005
Freeman JM, Kelly MT, Freeman JB. The Epilepsy Diet Treatment: An Introduction to the Ketogenic Diet. 3rd ed. New York, NY: Demos Vermande; 1994
Haslam RH. Nonfebrile seizures. Pediatr Rev. 1997;18:39-49
Haslam RH. Seizures in childhood. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 16th ed. Philadelphia, Pa: WB Saunders Co; 2000:1813-1829