A Case of Primary Lateral Sclerosis Showing Degeneration ofCorticospinal Tract on Magnetic Resonance Imaging

J Korean Neurol Assoc > Volume 18(6); 2000 > Article

Journal of the Korean Neurological Association 2000;18(6): 801-804.

뇌 자기공명영상에서 피질척수로의 변성을 보인 일차성측삭경화증 1예

오지영, ·유현정 ·김지현 ·김정은 ·최경규 ·박기덕

이화여자대학교 의과대학 신경과학교실

A Case of Primary Lateral Sclerosis Showing Degeneration ofCorticospinal Tract on Magnetic Resonance Imaging

Jee-Young Oh, M.D., Hyun-Jung Yu, M.D., Jee-Hyun Kim, M.D., Jung-Eun Kim, M.D.,Kyoung-Gyu Choi, M.D., Kee-Duk Park, M.D.

Department of Neurology, College of Medicine, Ewha Womans University

Abstract

Primary lateral sclerosis (PLS) is a rare upper motor neuron disease characterized by selective degeneration of cor-ticospinal and corticobulbar tracts. It is important to rule out other diseases presenting as progressive spastic parapare-sis, such as multiple sclerosis (MS) or amyotrophic lateral sclerosis (ALS). Though that is not so difficult as the devel-opment of imaging technique and neurophysiological tool, if one shows slow progressive spastic paraparesis clinically, and there is no abnormal CSF or EMG findings, it is not easy to differentiate between primary progressive MS and PLS. In fact, PLS is so rare that it has long been debated whether PLS is a disease entity or syndrome or a nothing. But we present a PLS patient whose brain MRI shows diffuse signal change along the bilateral corticospinal tracts and the hypoperfusion of frontal motor cortex is proved by brain SPECT.J Korean Neurol Assoc 18(6):801~804, 2000Key Words : Primary lateral sclerosis, Corticospinal tract, Brain MRI, Brain SPECT