Which leukodystrophy is virtually always associated with a particular endocrinologic deficiency?
Adrenoleukodystrophy, an X-linked recessive disorder, is one of the peroxisomal disorders.
It is characterized by impaired beta-oxidation of the very long-chain (C26) fatty acids, leading to their accumulation.
In addition, patients also have adrenocortical insufficiency.
Onset is usually between 4 and 6 years of age.
An adult form of the disease called adrenomyeloneuropathy is characterized by progressive spastic paraparesis and peripheral neuropathy.