What is the role of visual evoked potential (VEP) testing in the workup of adrenoleukodystrophy?

Updated: Oct 25, 2019
  • Author: Andrew B Evans, MD; Chief Editor: Selim R Benbadis, MD  more...
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Answer

Answer

Adrenoleukodystrophy is an X-linked metabolic disorder with very-long-chain fatty acid (VLCFA) accumulation and multifocal nervous system demyelination, often with early involvement of visual pathways. Kaplan et al found that pattern-reversal VEPs were abnormal in 17% of men with adrenoleukodystrophy; no evidence indicated that reduction of VLCFA levels improved or retarded visual pathway demyelination. [20]


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