Idiopathic Familial Intracerebral Calcification with Pyramidal Tract Degeneration

J Korean Neurol Assoc > Volume 9(1); 1991 > Article

Journal of the Korean Neurological Association 1991;9(1): 101-106.

추체로퇴화가 동반된 유전성 특발성 뇌석회화증

최영철, 손영호,김윤중,정재우,김진수,황연미

연세대학교 신경과. 울산대학교 신경과. 서울중앙병원 신경과.

Idiopathic Familial Intracerebral Calcification with Pyramidal Tract Degeneration

Young Chul Choi, M.D., Young Ho Sohn, M.D., Yoon Joong Kim, M.D. Jae Woo Jung, M.D., Jin Soo Kim, M.D. Youn Mee Hwang, M.D.*

Dep. Of Neurology, Yonsei University Dep. Of Neurology, Ulsan University, Asan Medical Center*

Abstract

A l9-year-old boy was admitted because of progressive spastic quadriparesis and bulbar palsy which developed from 3 years ago. The results of all performed laboratory tests were normal, but neuroradiologic tests revealed intracerebral calcification and degeneration of oilateral pyramidal tracts. Idiopathic familial intracerebral calcification was rarely occurred and presented usually with various extrapyramidal and cortical symptoms. Mild pyramidal symptoms were also associated in some reported cases, but none of them showed predominant spasticity as well as suspicious pyramidal tract degeneration on magnetic resonance imaging.