All issues > Volume 33(2); 1990

Original Article

J Korean Pediatr Soc. 1990;33(2):178-188. Published online February 28, 1990.

Clinical Observation on Moyamoya Disease in Childhood.

Se Hee Hwang¹, Jae Il Sohn¹, Yong Seung Hwang¹

¹Department of Pediatrics, College of Medicine, Seoul National University, Seoul, Korea

Received: September 26, 1989;  Accepted: November 9, 1989.

Abstract

Although the etiology and treatment of moyamoya disease are unknown, the surgical correction is expected as a good treatment method. But its effectiveness is not confirmed until now. A clinical study was performed on 21 patients of moyamoya disease in childhood who admitted to the Pediatric and Neurosurgical department of SNUCH from Jan. 1980 till July 1989. The results were as follows: 1) The ratio of male to female was 1.6:1. Thirteen patients were male, 8 patients female. The mean age on admission was 5 years old. 2) We classified the past medical hisotry into two groups, previosuly healthy group (57%) and abnormal past history group (43%)-such as seizure, etc. 3) Chief complaints on admission were cerebral ischemic symptoms, which revealed motor weak- ness in 20 paitents (95%), bulbar symptoms in 10 patients (48%), hemiplegia in 2 patients (95%), loss of consciousness in 2 patients (95%) and seizure in 1 patient (4.8%). 4) The frequency of iscemic attack was recurrent in 15 patients (75%), the other 6 patients (25%) had one or two ischemic attacks on admission. 5) Provoking factors of the ischemic attacks were identified in 12 patients (75%). The characteris- tic provoking factors are eating hot food, being excited, crying and psychological stress such as quarrel. 6) All cases showed the characteristic angiographic findings of moyamoya disease. Seventeen cases (18%) were of bilateral carotid artery involvement, and 4 cases (19%) were of unilateral involvement. And there were 5 cases (24%) of posterior cerebral artery involvement. 7) All cases of CT scan showed cerebral infarction and 2 cases were combined with brain atr- ophy and 1 case combined with hydrocephalus. 8) EEG was checked in 14 patients and 13 patients had abnormal findings, which were focal slowing (47%, 8 cases), asymmetry (21%, 3 cases), focal epileptiform (21%, 3 cases), diffuse slowing (9.5%, 2 cases). 9) MRI was checked in 8 patients, and all of them showed major vessel obstruction and collateral vessels. Besides, there were 6(75%) cases of cerebral infarction and 2(27%) cases of brain atrophy. 10) SPECT was perfromed in 6 patients, and all of them showed perfusion defect in the area of infarction. 11) Cerebral blood flow was measured in 4 patients, which showed decreased cerebral blood flow in the infarcted area. 12) The newly developed surgical therapy of EDAS(encephalo-duro-arterio-synangfiosis) has been performed in 4 patients. The result of the surgical treatment should be evaluated after the further follow-up period.

Keywords :Moyamoya disease