J Korean Neurol Assoc.
1991 Jun;9(2):262-268.
A Case of Adrenomyeloneuropathy
- Affiliations
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- 1Department of Neurology, Yonsei University, Korea.
- 2Department of Pathology, Seoul National University, Korea.
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.
MeSH Terms
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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
Adrenocorticotropic Hormone
Gadolinium
Hydrocortisone
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