4.2 Article

New treatment of free-radical scavenger in adrenoleukodystrophy

Journal

JOURNAL OF CLINICAL PHARMACY AND THERAPEUTICS
Volume 36, Issue 3, Pages 412-415

Publisher

WILEY-BLACKWELL
DOI: 10.1111/j.1365-2710.2011.01267.x

Keywords

adrenoleukodystrophy; d-ROM; edaravone; free-radical scavenger; nitrite; nitrate; short latency somatosensory evoked potential

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P>What is known and Objective: Adrenoleukodystrophy (ALD) is an X-linked disorder and characterized by the accumulation of saturated very long-chain fatty acids. Treatment is still unsatisfactory. Our objective is to report on the effect of the free-radical scavenger, edaravone, in a patient with ALD. Case summary: The patient was given edaravone intravenously twice. D-ROM in cerebral spinal fluid decreased dramatically, and a shortening of neuronal transmission time as estimated on somatosensory evoked potential was observed. After terminating the treatment, his symptoms progressively reappeared. What is new and Conclusion: This is the first report of the use of edaravone in ALD. The drug is apparently effective in improving symptoms of ALD and should be evaluated more formally.

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