Purboyo S
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Electroencephalogram and clinical manifestations of Rett syndrome in children E S Herini; I Mangunatmadja; Purboyo S; Hardiono D Pusponegoro; Sunartini Sunartini
Paediatrica Indonesiana Vol 43 No 4 (2003): July 2003
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (496.788 KB) | DOI: 10.14238/pi43.4.2003.121-5

Abstract

Background Rett Syndrome (RS) is a severe neurodevelopmentaldisorder. Epileptic seizures occur in 80-90%; grandmal, psychomo-tor (complex partial), and focal motor seizures have been reported.The electroencephalogram(EEG) is almost always abnormal.Objective This study aimed to investigate the EEG and clinicalmanifestations of children with RSResults We investigated EEG on 5 patients with RS aged 30–66month. One patient was in clinical stage II and 4 patients in clini-cal stage III. Four patients had history of seizures, however onlytwo patients suffered from epilepsy. The EEG demonstrated slow-ing background activity in occipital region in two patients. In addi-tion, epileptic form activities were observed in 4 of 5 patients.Conclusion We concluded that epileptic spike discharge with orwithout clinical seizures were found in almost all of our RS pa-tients. These paroxysmal discharges suggested the process andthe sequences of cortical involvement. Compelling clinical, neuro-physiological evidences were very important to decide the stageof Rett disorder
The Epilepsies of Rett Syndrome in Indonesia E. S. Herini; Sunartini H; I. Mangunatmadja; Purboyo S; Hardiono D. Pusponegoro
Paediatrica Indonesiana Vol 45 No 5 (2005): September 2005
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.14238/pi45.5.2005.203-6

Abstract

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