A Case of Adrenomyeloneuropathy

J Korean Neurol Assoc > Volume 9(2); 1991 > Article

Journal of the Korean Neurological Association 1991;9(2): 262-268.

Adrenomyeloneuropathy 1예

석승한, 손영호,최영철,김진수,지제근

연세대학교 신경과. 서울대학교 병리학.

A Case of Adrenomyeloneuropathy

Seung Han Suk, M.D., Young Ho Sohn, M.D., Young Chul Choi, M.D., Jin Soo Kim, M.D. Je Geun Chi, M.D.*

Dep. Of Neurology, Yonsei University Dep. Of Pathology, Seoul National University*

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.