Electroencephalogram and clinical manifestations of Rett syndrome in children

• Main Authors: E S Herini (Author), I Mangunatmadja (Author), Purboyo S (Author), Hardiono D Pusponegoro (Author), Sunartini Sunartini (Author)
• Format: Book
• Published: Indonesian Pediatric Society Publishing House, 2016-09-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.
• Notes: 0030-9311; 2338-476X; 10.14238/pi43.4.2003.121-5
• Keywords: Rett Syndrome; EEG; clinical manifestation; Medicine; R; Pediatrics; RJ1-570
• Source: Paediatrica Indonesiana, Vol 43, Iss 4, Pp 121-5 (2016)

Background Rett Syndrome (RS) is a severe neurodevelopmental disorder. 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 clinical manifestations of children with RS Results We investigated EEG on 5 patients with RS aged 30-66 month. One patient was in clinical stage II and 4 patients in clini- cal stage III. Four patients had history of seizures, however only two 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 or without clinical seizures were found in almost all of our RS pa- tients. These paroxysmal discharges suggested the process and the sequences of cortical involvement. Compelling clinical, neuro- physiological evidences were very important to decide the stage of Rett disorder