ABSTRACT
Adrenoleukodystrophy is a progressive cerebral degenerative disorder with onset in childhood, in which there is increased pigmentation of the skin and laboratory evidence of degenerative disease of the adrenals [1,2]. The disease appears to have been first described by Haberfield and Spielerwere in 1910 [3], and the neuropathological findings by Schilder [4]. Siemerling and Creutzfeld were in 1923 [5] the first to put together the adrenal and cerebral disease in the definitive description; they referred to it as a bronzed disease in which there was sclerosis and encephalomyelitis, and it has been referred to as a bronzed Schilder disease, but in spite of the pigment, which may serve as the alerting sign to the diagnosis, most patients have full progression of the cerebral manifestations without the clinical symptomatology of adrenal insufficiency [2,6,7]. The lipid inclusions in the adrenal were first recognized by Schaumberg and colleagues [6,7] who found that they were composed of cholesterol esters [8]. The term adrenoleukodystrophy was first employed by Blaw [9]. This disease appears to be the cause of Schilder disease in most males [9].
