<prompt> Wrong about X-linked adrenoleukodystrophy:Is a mitochondrial disorderMay present as Addisons diseaseInvolves accumulation of short chain fatty acidsInvolves progressive neuronal grey matter degeneration  <response>: Ans. A. Is a mitochondrial disorderX-linked adrenoleukodystrophy is a peroxisomal disorder of very long chain fatty acid VLCFA oxidation. It result in progressive adrenal cortex and neuronal white matter degeneration, and the phenotype is variable. It may present with Addisons disease, or it may present with neurological features behaviour disturbance, developmental regression, ataxia, seizures, and spasticity. Diagnostic investigations include VLCFAs, adrenal cortical function tests, MRI brain scan and neuropsychiatric assessment. Management is supportive with adrenal steroid replacement, anticonvulsants, NG or gastrostomy feeding. Supplementation with monounsaturated amino acids Lorenzos oil may be beneficial but is not curative. 