The Epilepsies of Rett Syndrome in Indonesia

Objective This study aimed to evaluate various types of seizures, EEG findings, and last evolution of epileptic seizures found in children with Rett Syndrome (RTT). Methods A case series of ten female subjects from Sardjito Hospital, Yogyakarta; Cipto Mangunkusumo Hospital, Jakarta; and Hasan Sadikin Hospital, Bandung from February 2001-April 2004. Diagnosis of RTT was established using necessary and supportive criteria by the RTT Diagnostic Criteria Work Group and epilepsy was diagnosed according to clinical and EEG findings. Results The age during initial diagnosis of RTT ranged from 2 years 6 months -10 years 2 months (average: 4 years 6 months). The diagnosis of epilepsy, on the other hand, was established from 2 years - 5 years 10 months (average: 3 years 8 months). All subjects (10/10) had abnormal EEG results. Eight out of ten
had epileptiform discharge; the remaining had slowing background activity. The average age of initial seizure onset was 18 months. Five were diagnosed as epileptic subjects. Febrile convulsions were the most common initial seizure. General tonic clonic seizure was major type of seizure which manifested in 2 subjects. Conclusion All EEG results showed abnormality, although seizure did not appear. Five out of ten subjects experienced epilepsy