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BACKGROUND: "Rebuild-up" phenomenon, induced by hyperventilation, is a characteristic finding on EEG in children with Moyamoya disease. Its mechanism, however, remains obscure. In this study, we examined the relationship between cerebral lesions on MRI, stenosis or occlusion of cerebral vessel on cerebral angiography, and EEG findings in children with Moyamoya disease. METHODS: We have reviewed medical records of 33 patients, who were confirmed as Moyamoya disease by cerebral angiography at Asan Medical Center. EEG and brain MRI were carried out in all subjects. RESULTS: 1) Epidemiologic data were : the male to female ratio was 1:1.1; highest rate(90.7%) of onset in age group below 10 years; mean age at clinical onset was 7.4 years; average diagnostic interval from clinical onset to diagnosis was 1.9 years. 2) The most common initial and recurrent or residual symptoms were motor deficit. 3) The most common site of occlusion or stenosis of cerebral vessel on cerebral angiography was anterior cerebral area(>97%) and the most common cerebral infarction area on brain MRI was anterior cerebral area, too. 4) The hyperventilation(for 3 minutes) on EEG were carried out in 25 patients and the prolonged build-up or rebuild-up phenomenon was observed in 13 patients(52%). 5) The prolonged build-up or rebuild-up phenomenon on EEG was observed in 6 of 15 patients who were occlusion of cerebral vessel, and in 7 of 10 patients who were stenosis of cerebral vessel on angiography. CONCLUSION: 1) The background slowings on EEG maybe suggestive of the infarction stage of Moyamoya disease in children. 2) The prolonged build-up or rebuild-up phenomenon might indicate the preinfarction stage of Moyamoya disease in children.
Angiography ; Brain ; Cerebral Angiography ; Cerebral Infarction ; Child ; Chungcheongnam-do ; Constriction, Pathologic ; Diagnosis ; Electroencephalography ; Female ; Humans ; Hyperventilation ; Infarction ; Magnetic Resonance Imaging ; Male ; Medical Records ; Moyamoya Disease*

"Idiopathic long QT syndrome" is characterized by prolongation of the QT interval due to unusual electrocardiographic repolarization abnormality and associated with variable clinical manifestations from no specific symptoms in lifetime to syncope or even sudden death. The prognosis of this syndrome is very grave and motality is approximately 50% within 10 years among untreated symptomatic patients after the initial syncope. But this sudden onset syncope may be misdiagnosed as epilepsy, being treated with antiepileptic drug for many years. However, this high mortality has been significantly reduced to less than 5% by the effective therapy. Therefore, it is crucial to make an early and accurate dianosis. We exprienced a case of 34 months old male who presented with recurrent syncopal attacks. He had no specific neurological abnomal finding except congenital deafness. He had normal EEG and brain MRI findings but ECG showed prolonged QT interval (QTc= 0.5), findings of which were compatible with long QT syndrome. He is currently being followed at OPD, but the pateint is still experiencing syncopal attack despite of treatment with beta-blocker, atenolol. Therefore, we are considering an insertion of pacemaker or performing thoracic sympathectomy.
Atenolol ; Brain ; Child, Preschool ; Deafness ; Death, Sudden ; Electrocardiography ; Electroencephalography ; Epilepsy* ; Humans ; Long QT Syndrome* ; Magnetic Resonance Imaging ; Male ; Mortality ; Prognosis ; Sympathectomy ; Syncope