兒童模芽模芽病的電生理及穿顱超音波：兩病例報告

Electrophysiologic and Transcranial Doppler Studies in Childhood Moyamoya Disease: Report of Two Cases

模芽模芽病在兒童並不常見。關於模芽模芽病的電生理及穿顱多卜勒超音波的報告很少。我們報告兩例，起初出現抽蓄症狀而以抗癲癇藥治療，但隨後出現腦缺血性症狀，經核磁共振掃描血管攝影後證實是模芽模芽病。並且電生理學檢查（腦電圖和大腦誘發電位）和穿顱多卜勒超音波出現異常變化。在病例1，腦波有“放大（rebuild-up）現象。病例2的SSEP顯示皮質傳導異常，同時穿顱多卜勒超音波顯示血流異常。我們發現電生理學、穿顱多卜勒超音波的結果與其臨床癥狀有相當的關連性存在。所以認為這兩項檢查可做為模芽模芽病的輔助診斷與追蹤病情變化的利器。

Moyamoya disease is uncommon in childhood. We report on two children, aged 4 and 5 years, with moyamoya disease presenting. The presentation was seizures treated with carbamazepine. Both patients experienced subsequent transient ischemic attacks. Moyamoya disease was confirmed by magnetic resonance imaging and magnetic resonance angiography. Serial electroencephalography (EEG), evoked potentials, and transcranial doppler echography (TCD) studies were done. In case 1, EEG showed a ”re-build-up” phenomenon. in case2, somatosensory evoked potentials in the right median nerve revealed delayed latencies of N20 with poor waveform in the left cortical component in the second patient. TCD showed no traced flow on the left middle cerebral artery and ”to-and-fro” flow over the posterior communicating artery in case 2. These studies correlated well with their clinical features. We suggested that EEG, evoked potentials, and TCD could be adjunctive tools in the early diagnosis and clinical evaluation of moyamoya disease in children.