J Korean Neurol Assoc.
2001 Jul;19(4):431-434.
A Case of Adrenomyeloneuropathy
- Affiliations
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- 1Department of Neurology, Asan Medical Center, Ulsan University College of Medicine.
Abstract
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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)