Proton MR spectroscopy in hyperhomocysteinemia with elevated blood methionine levels

L. Patricia Franco, Jada Anderson, James Okoh, Martin G. Pomper, Nancy Braverman, Peter B. Barker

Research output: Contribution to journalArticlepeer-review

3 Scopus citations


Proton magnetic resonance spectroscopy imaging (MRSI) and serial MRI were performed on a 10-year-old girl with B6 unresponsive cystathionine B-synthase (CBS) deficiency who developed high methionine levels while on betaine therapy. At presentation, T2-weighted sequences showed diffuse white matter (WM) hyperintensity and sulcal effacement, while MRSI metabolite concentrations were normal. Four months later, after the betaine therapy was discontinued and a methionine-restricted diet with vitamin B6, B12, and folate supplementation was initiated, blood methionine levels and MRI findings returned to normal. Normal MRSI at presentation was predictive of a positive outcome despite the markedly abnormal initial MRI results.

Original languageEnglish (US)
Pages (from-to)404-407
Number of pages4
JournalJournal of Magnetic Resonance Imaging
Issue number3
StatePublished - Mar 2006


  • Brain
  • Edema
  • Hyperhomocysteinemia
  • Magnetic resonance spectroscopy
  • Methionine

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging


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