A Case of Wilson's Disease With Extensive Cortico-subcortical Lesions

J Korean Neurol Assoc > Volume 28(2); 2010 > Article

Journal of the Korean Neurological Association 2010;28(2): 112-114.

광범위한 피질-피질하 병변을 동반한 윌슨병

이재혁, 신진홍 김대성 a 박경필 a 조재욱 a 정대수

부산대학교 의학전문대학원 신경과학교실, 의학연구소, 양산부산대학교병원 신경과 a

A Case of Wilson's Disease With Extensive Cortico-subcortical Lesions

Jae-Hyeok Lee

Department of Neurology, Pusan National University School of Medicine, Medical Research Institute, Pusan National University Yangsan Hospital a , Yangsan, Korea

Abstract

Wilson’s disease with extensive cortico-subcortical lesions represents a rare pathological condition. We experienced an unusual case of prominent frontal cortico-subcortical lesions in association with epilepsy. One year after decoppering therapy, improvement of extrapyramidal signs was correlated with decrement of the abnormal signals in basal ganglia and thalamus, while atrophic change was apparent. However, the lesions in subcortical white matter indicated the presence of encephalomalacic change. These findings suggest that the prognosis of subcortical white-matter changes can differ from that of basal ganglia changes. Key Words: Wilson disease, White matter, Epilepsy.