X-linked adrenoleukodystrophy
ORPHA:43· ICD-10 E71.3
Definition
A rare progressive peroxisomal disorder characterized by endocrine dysfunction (adrenal failure and sometimes testicular insufficiency), progressive myelopathy, peripheral neuropathy and, variably, progressive leukodystrophy.
- Prevalence
- 1-9 / 1 000 000
- Inheritance
- X-linked dominant
- Age of onset
- Adolescent, Adult, Childhood, Elderly