Adrenomyeloneuropathy Presenting With Adrenal Insufficiency
Annals of Rehabilitation Medicine
;
: 563-566, 2013.
Article
Dans Anglais
| 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.
Texte intégral:
Disponible
Indice:
WPRIM (Pacifique occidental)
Sujet Principal:
Moelle spinale
/
Nerf tibial
/
Insuffisance surrénale
/
Neuropathies périphériques
/
Maladies péroxysomiales
/
Adrénoleucodystrophie
/
Troubles neurologiques de la marche
/
Paraparésie spastique
/
Potentiels évoqués somatosensoriels
/
Miction impérieuse incontrôlable
langue:
Anglais
Texte intégral:
Annals of Rehabilitation Medicine
Année:
2013
Type:
Article
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