All issues > Volume 25(10); 1982

Case Report

J Korean Pediatr Soc. 1982;25(10):1053-1057. Published online October 31, 1982.

A Case of Moyamoya Disease with Focal Seizure.

Man Gee Hong, Hak Ki Kim, Kyung Tai Whang, Sung Hoon Cho

¹Department of Pediatrics, Catholic Medical College, Seoul, Korea

Abstract

We experienced a case of Moyamoya syndrome in a 4 year-old girl. The patient was well until 3 years of age when she first experienced numbness of the left arm. After 10 days of above episode, she had tonic-clonic movements of left sided extremities which lasted, approximately 2 hours and followed by paresis. During the following week the second attack of focal seizure followed by paresis of left arm occurred, and the patient was admitted for evaluation, and work-up of seizure disorder. Metabolic work-up consisted of calcium, phosphorus, electrolytes and blood sugar, and all of which were normal. EEG showed diffuse slowing into delta range activity and frequent sharp bursts in the right temporal head areas with phase reversal at T< area. Brain CT scan revealed mixed vat density in the right frontoparietal region (Fig. 1). To clarify the matter, right carotid angiography was performed and showed narrowing of internal carotid artery and its major branches, occlusion of middle cerebral artery, and abnormal capillary net-works (Fig. 2). Above findings were felt that the focal seizure was secondary to Moyamoya syndrome. On. 11th hospital days, she underwent operation named encephaloduroarteriosynangiosis. The patient was discharged in satisfactory condition at 24th hospital days, and has been seizure free over 8 months on phenobarbital 30mg twice daily.

Keywords :Moyamoya Disease, Focal Seizure, Carotid Angiography, Encephaloduroarteriosynangiosis.