A Case of Suspected Hallervorden-Spatz Disease

J Korean Neurol Assoc > Volume 10(3); 1992 > Article

Journal of the Korean Neurological Association 1992;10(3): 407-412.

Hallervorden-Spatz씨병이 의심되는 1예

송창원, 이성훈,아상익,김대성,박규현,김상욱,김상호

부산대학교 신경과. 성요셉병원 신경과.

A Case of Suspected Hallervorden-Spatz Disease

Chang-Won Song, M.D., Sung-Hoon Lee, M.D., Sang-Ik Lee, M.D., Dae-Seong Kim, M.D., Kyu-Hyun Park, M.D., Sang-Wook Kim, M.D., Sang-Ho Kim, M.D.

Department of Neurology, College of Medicine, Pusan National University, Department of Neurology, Saint Joseph's Hospital

Abstract

Hallervorden-Spatz disease is a rare, autosomal recessive disorder of mainly early childhood which is characterized by pigmentary degeneration of the globus pallidus, substantia nigra, and red nucleus. Clinically it manifests various symptoms and signs of extrapyramidal and pyramidal involvement. Authors report a 28-year-old female patient with suspected Hallervorden-Spatz disease in the aspects of clinical and MRI findings suggesting metal deposition in the globus pallidus, substantia nigra, and red nucleus on both side.