Complicated Spastic Paraparesis with Thin Corpus Callosum

J Korean Neurol Assoc > Volume 22(4); 2004 > Article

Journal of the Korean Neurological Association 2004;22(4): 402-405.

얇은 뇌량을 동반한 복합형 강직성 하지마비 2예

이재혁, 박영은 최광동 박경필 김대성 정대수 박규현

부산대학교 의과대학 신경과학교실

Complicated Spastic Paraparesis with Thin Corpus Callosum

Jae Hyeok Lee

Department of Neurology, Pusan National University College of Medicine, Busan, Korea

Abstract

Autosomal recessive hereditary spastic paraparesis (AR-HSP) associated with a thin corpus callosum (TCC) has recently been described in Japan. It is characterized by slowly progressive spastic paraparesis, mental retardation, and thalamic degeneration. We report two unrelated patients with progressive gait disturbance starting in the second decade of life, spastic paraparesis, pes cavus, and mental deterioration. One patient presented with acute psychosis, and the other had visual disturbances with normal tension glaucoma. A brain MRI of both patients showed a thin corpus callosum, and a brain SPECT revealed thalamic hypoperfusion in one patient. This is the first report of spastic paraparesis with a thin corpus callosum in Korea. Key Words: Spastic paraparesis, Corpus callosum