小兒科CHEN, HUEY-LINGHUEY-LINGCHENWANG, PEN-JUNGPEN-JUNGWANGYOUNG, CHAINLLIECHAINLLIEYOUNGLIU, HON-MANHON-MANLIUSHEN, YU-ZENYU-ZENSHEN2008-12-102018-07-112008-12-102018-07-111994http://ntur.lib.ntu.edu.tw//handle/246246/90645　　A case of a female infant with tuberous sclerosis and hemimegalencephaly is reported. This full term female infant had tachyarrhythmia on the day of birth, and developed congestive heart failure several days later. Generalized convulsions started at 5 days old. Right hemiparesis had been noted since birth. Initial electroencephalograms (EEG) revealed continuous left hemispheric spike-and -wave discharges. Brain sonography revealed left himimegalecephaly with several hyperechoic spots over the left periventricular wall and bilateral multiple tubers. MRI showed himimegalencephaly, heterotopia, pachygyria on the left side and multiple nodules bilaterally. Electrocardiography revealed changing patterns of arhythmia including atrial flutter, ectopic atrial tachycardia, and Wolff-Parkinson-White complex. Echocardiography revealed multiple intracardiac tumors. There were several hypomelanotic papules over the trunk. There was no family history of tuberous sclerosis. Despite the frequent findings of histological similarity between hemimegalencephaly and tuberous sclerosis, there has been no classical multisystemic tuberous sclerosis complex reported to be associated with hemimegalencephaly. The literatures were reviewed regarding these two.# 0285#en-USjournal article