A Case of Adrenomyeloneuropathy.
- Author:
Seung Han SUK
1
;
Young Ho SOHN
;
Young Chul CHOI
;
Jin Soo KIM
;
Je Geun CHI
Author Information
1. Department of Neurology, Yonsei University, Korea.
- Publication Type:Case Report
- MeSH:
Adrenocorticotropic Hormone;
Adrenoleukodystrophy*;
Biopsy;
Brain;
Evoked Potentials;
Gadolinium;
Hair;
Humans;
Hydrocortisone;
Hypesthesia;
Lower Extremity;
Magnetic Resonance Imaging;
Male;
Muscle Spasticity;
Neural Conduction;
Neurology;
Paraparesis, Spastic;
Peripheral Nervous System Diseases;
Pyramidal Tracts;
Skin;
Sural Nerve
- From:Journal of the Korean Neurological Association
1991;9(2):262-268
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
A 19 yar-old male patient was admitted to the department of neurology in Severance hospital beause of slowly progressive spastic paraparesis. On examination, dark brown skin color, sparse hair, spastic weakness and hypesthesia of lower extremities were noted. The laboratory studies showed high serum ACTH level with lowest normal limit of serum cortisol level. Brain MRI scan revealed high signal intensities in bilateral corticospinal tracts on T, Weighted images with gadolinium enhancement and T2eighted images. The results of three modal evoked potential studies(SEP. BAEP. VEP) were abnormal bilaterally. Nerve conduction studies and sural nerve biopsy suggested the presence of peripheral neuropathy.