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E S Herini
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Health Professions Medicine & Pharmacology

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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
Rett syndrome in childhood: the clinical characteristics E S Herini; Irawan Mangunatmadja; Purboyo Solek; Hardiono D Pusponegoro
Paediatrica Indonesiana Vol 44 No 4 (2004): July 2004
Publisher : Indonesian Pediatric Society

Show Abstract | Download Original | Original Source | Check in Google Scholar | Full PDF (689.964 KB) | DOI: 10.14238/pi44.4.2004.160-4

Abstract

The prevalence rate of RS in various coun-tries is from 1:10,000 to 1:23,000 female livebirths. RS is most often misdiagnosed as autism,cerebral palsy, or non-specific developmental de-lay. While many health professionals may not befamiliar with RS, it is a relatively frequent cause ofneurological dysfunction in females. There are nobiological markers for this disease, the diagno-sis is established by history taking and clinical find-ings. We reported two patients, both girls, with RS.
Co-Authors Hardiono D Pusponegoro I Mangunatmadja Irawan Mangunatmadja Purboyo S Purboyo Solek Sunartini Sunartini