A Case of Adrenomyeloneuropathy

J Korean Neurol Assoc > Volume 19(4); 2001 > Article

Journal of the Korean Neurological Association 2001;19(4): 431-434.

부신척수신경병증 1예

정종필, 김철호 이상암

울산대학교 의과대학 서울중앙병원 신경과학교실

A Case of Adrenomyeloneuropathy

Jong-Pil Jeong, M.D., Chol-Ho Kim, M.D., Sang-Ahm Lee, M.D.

Department of Neurology, Asan Medical Center, Ulsan University College of Medicine

Abstract

"We report a 37-year-old man with adrenomyeloneuropathy who presented as progressive gait disturbance. He had spastic paraparesis, hyperreflexia with Babinski’s signs, a sensory level at T-4, and loss of the vibration sense in the legs. No adrenal insufficiency was noted. There were frontal white matter abnormalities but no cervical spinal lesions on MRI. A nerve conduction study showed distal axonal neuropathy predominantly in the lower extremities. The plas-ma level of the saturated very long chain fatty acids was elevated. J Korean Neurol Assoc 19(4):431~434, 2001 Key Words : Adrenoleukodystrophy, Adrenomyeloneuropathy, Spastic paraparesis"