A Case of Adrenomyeloneuropathy.
- Author:
Jong Pil JEONG
1
;
Chol Ho KIM
;
Sang Ahm LEE
Author Information
1. Department of Neurology, Asan Medical Center, Ulsan University College of Medicine.
- Publication Type:Case Report
- Keywords:
Adrenoleukodystrophy;
Adrenomyeloneuropathy;
Spastic paraparesis
- MeSH:
Adrenal Insufficiency;
Adrenoleukodystrophy*;
Adult;
Axons;
Fatty Acids;
Gait;
Humans;
Leg;
Lower Extremity;
Magnetic Resonance Imaging;
Neural Conduction;
Paraparesis, Spastic;
Reflex, Abnormal;
Reflex, Babinski;
Vibration
- From:Journal of the Korean Neurological Association
2001;19(4):431-434
- CountryRepublic of Korea
- Language:Korean
-
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)
