The Saudi experience

recessively inherited disorder due to a defect in adrenoleukodystrophy (ALD) protein essential for the activity of peroxisomal very long chain acyl CoA synthetase (VLCAS), sometimes called lignoceroyl CoA ligase, an important enzyme in the peroxisomal β-oxidation of very long chain fatty acids (VLCFAs).1-3 Childhood ALD is characterized by a T presymptomatic period that usually lasts from 4 to 8 years. This is followed by a period of behavioral changes and deterioration in intellect, vision and gait. Then a fairly progressive course ensues with spastic paraplegia, hemiplegia or quadriplegia, evolving into a terminal neurovegetative stage and, ultimately death, which typically follows 1 to 4 years after the disease onset.4 This disorder has a normal peroxisome structure Objectives: To evaluate the clinical, biochemical, neuroradiological, and neurophysiological findings of patients with X-linked adrenoleukodystrophy.