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Adrenoleukodystrophy (ALD) is a metabolic disease inherited as an X-linked recessive trait. An enzymatic defect in subcellular peroxisomal degradation causes an accumulation of saturated very-long-chain fatty acids. Current classifications distinguish several variants: presymptomatic or asymptomatic ALD, childhood cerebral ALD, adolescent cerebral ALD, adult cerebral ALD, adrenomyeloneuropathy, and “Addison only” ALD.1 The predominant feature in ALD is cerebral white matter involvement with widespread demyelination. Most patients present clinically with spastic paraplegia, urinary incontinence, and epileptic seizures; in patients with adrenomyeloneuropathy, signs of myelopathy and peripheral nerve involvement dominate the clinical course. A frequent feature is adrenal gland insufficiency. In adult patients with ALD the diagnosis relies on plasma assay of very-long-chain fatty acids and neuroradiologic and neurophysiologic evidence of cerebral white matter involvement.1,2
For the first time in a case of ALD, we report a study of central nociceptive pathways, by means of laser evoked potentials.
Case report.
A 22-year-old man presented because of difficulty in walking. Clinical examination showed a bronzed skin …
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