Case 94 - Atypical Evolution in a Case of Benign Childhood Epilepsy with Centrotemporal Spikes

Abstract

This chapter presents a case, which exemplifies the risk of atypical evolutions of benign focal epilepsies in childhood (BFEC). The girl's first seizures occurred at 3 years of age and were brief focal motor seizures affecting her left hand. After several months free of seizures, she presented motor facial seizures (on the left side) plus sialorrhea while awake lasting 10–60 s. She had been medicated with carbamazepine and free of seizures for 6 months. Carbamazepine was switched to valproic acid but improvement lasted only 1 month, and the new episodes with head-drops and myoclonic jerks in upper limbs occurred. At the same time several episodes with dysarthria and aphasic features were seen and frank dysgraphia was noted in her schoolwork. Sulthiame 300 mg/day was added and since 2 days later she has been seizure-free until her last visit at 14.7 years of age. The diagnosis at onset corresponds to benign childhood epilepsy with centrotemporal spikes (BECTS) syndrome with some atypical features (age of onset, frequency of seizures). During the evolution of her disorder, the girl presented manifestations of atypical benign partial epilepsy of childhood (ABPEC) plus episodes of status epilepticus of BECTS (SEBECTS) (sialorrhea during 2 h, prolonged anarthria), plus some symptoms of Landau–Kleffner syndrome (LKS; language deterioration). All three groups of symptoms and signs were associated with electrical status epilepticus during slow sleep (ESES).