Adrenomyeloneuropathy Presenting With Adrenal Insufficiency
Annals of Rehabilitation Medicine
;
: 563-566, 2013.
Article
in English
| WPRIM
| ID: wpr-173386
ABSTRACT
Adrenomyeloneuropathy (AMN), one of the variants of X-linked adrenoleukodystrophy (ALD), is inherited peroxisomal disorder associated with the accumulation of very long chain fatty acids (VLCFA). AMN is characterized primarily by involvements of long ascending and descending tracts of the spinal cord and peripheral neuropathy, which leads to spastic paraparesis and urinary and erectile dysfunction. We experienced the AMN case of a 33-year-old man presenting bilateral progressive spastic paraparesis, impotence and urge incontinence with primary adrenal failures, as confirmed by increased serum of VLCFA concentrations. Considering that somatosensory evoked potentials in posterior tibial nerve was the only abnormal finding in electrophysiologic findings when compared with the severe spastic gait pattern shown, it is necessary to follow up with electrophysiologic studies.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Spinal Cord
/
Tibial Nerve
/
Adrenal Insufficiency
/
Peripheral Nervous System Diseases
/
Peroxisomal Disorders
/
Adrenoleukodystrophy
/
Gait Disorders, Neurologic
/
Paraparesis, Spastic
/
Evoked Potentials, Somatosensory
/
Urinary Incontinence, Urge
Language:
English
Journal:
Annals of Rehabilitation Medicine
Year:
2013
Type:
Article
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