Clinical Trial of Vitamin B6 for Gyrate Atrophy of the Choroid and Retina

Richard G. Weleber, Nancy G. Kennaway

Research output: Contribution to journalArticle

48 Scopus citations

Abstract

Seven patients with gyrate atrophy and deficiency of ornithine-8-aminotransferase were studied for in vivo pyridoxine responsiveness; three responded to oral vitamin B6 with over 50% reduction of serum ornithine levels and return to normal of serum lysine levels. Electrophysiologic studies were performed on two B6-responsive patients and one B6-non responder over various time periods with and without pyridoxine supplementation. Electroretinogram (ERG) amplitudes improved 100% in one patient when initially given high doses of vitamin B6. Electro-oculogram light-to-dark ratio also improved for this patient. Withdrawal followed by resumption of B6 supplementation was associated with mild worsening followed by improvement of ERG responses respectively in both patients. Long-term follow-up will be needed to assess whether pyridoxine treatment will slow or halt the progression of the disease.

Original languageEnglish (US)
Pages (from-to)316-324
Number of pages9
JournalOphthalmology
Volume88
Issue number4
DOIs
StatePublished - Jan 1 1981

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Keywords

  • amino acids
  • choroid
  • choroidal and retinal gyrate atrophy
  • electro-oculography
  • electroretinogram
  • enzyme deficiency
  • lysine
  • ornithine
  • ornithine aminotransferase
  • retina
  • retinal degeneration
  • treatment trial
  • vitamin B

ASJC Scopus subject areas

  • Ophthalmology

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