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By week 10,plasma very-long-chain fatty acid levels declined nearly to normal.Nonetheless,over a mean follow-up of 33 months none of the 14 men with adrenomyeloneuropathy improved.In nine men there was functional deterioration,coincident in four with new cerebral lesions on MRI.In a single patient there was a reduction in cerebellar demyelination,but without clinical improvement.In one of the five asymptomatic boys signs of myelopathy developed.There were no changes in the symptomatic heterozygous women.There was some improvement in peroneal-nerve conduction,but no detectable clinical improvement.Conduction to the parietal cortex(T12-P37 interpeak latency)worsened in both the symptomatic men and the boys with preclinical adrenomyeloneuropathy.There was no change in other somatosensory evoked potentials or in brain-stem auditory evoked potentials.Asymptomatic thrombocytopenia(<100,000 cells per cubic millimeter)was noted in six patients.In this open trial we found no evidence of a clinically relevant benefit from dietary treatment with oleic and erucic acids("Lorenzo's oil")in patients with adrenomyeloneuropathy. |
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