Adrenoleucodystrophy

Adrenoleucodystrophy is a sex-linked recessive inherited condition (that is one which, like haemophilia, is carried by the mother on one of her two X - chromosomes. If her son inherits his single X chromosome - males are XY rather than XX - from her, he will develop the disease). It involves progressive degeneration of what is called the white matter of the brain (leuco - means white, dystrophy means degeneration). The white matter represents the myelin lining of the nerve fibres in the brain, and as these degenerate there is progressive disability, immobility, seizures and ultimately death. At the same time there may be degeneration of the cortex of the adrenal gland (see kidney in the urinary tract section) causing the symptoms of adrenal failure - fatigue, vomiting and low blood pressure. It is caused by the failure of one of the cellular components to degrade chemicals called long-chain fatty acids.
 
 
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