Rolando-Epilepsie

Rolandic epilepsy (RE) belongs to the most frequent epilepsy syndromes in childhood. Short sensorimotor perioral seizures, frequently occurring during sleep are characteristic. The epilepsy is self-limiting and usually developmental outcome is good. However, in some patients, mostly transient, learning disorders occur. Centrotemporal spikes in the EEG are the neurobiological genetic marker. Atypical benign partial epilepsy of childhood (ABPE) is much rarer than RE. In addition to perioral sensorimotor seizures, atypical absences, astatic seizures, and focal motor seizures are observed. The EEG shows centrotemporal spikes, however, with a much more pronounced sleep activation, sometimes reaching a bioelectrical status epilepticus. Amazingly, genetic factors seem to play an even greater role in ABPE than in RE. Prognosis of the epilepsy is good in ABPE; however, it is guarded concerning developmental outcome. Epilepsy risk for siblings of children with RE is small. However, the EEG trait is present in about 15% of siblings. In siblings of children with ABPE, this rate is elevated to about 40%. Additionally, in a small number of siblings with RE/APRE, sequence variations in KCNQ2, KCNQ3, and ACHRNA7 were detected. A conclusive understanding and extension of these findings will only be possible using new comprehensive molecular genetic tools, e.g., whole exome sequencing.