The effects of copper-histidine therapy on brain metabolism in a patient with Menkes disease: A proton magnetic resonance spectroscopic study

Mitsutoshi Munakata, Osamu Sakamoto, Taro Kitamura, Mamiko Ishitobi, Hiroyuki Yokoyama, Kazuhiro Haginoya, Noriko Togashi, Hajime Tamura, Shuichi Higano, Shoki Takahashi, Toshihiro Ohura, Yasuko Kobayashi, Akira Onuma, Kazuie Iinuma

Research output: Contribution to journalArticle

13 Citations (Scopus)

Abstract

We report on metabolic changes in the brain of a boy with Menkes disease. He was treated with parenteral copper (Cu)-histidine supplementation, from 5 months of age, and assessed with proton magnetic resonance spectroscopy ( 1 H-MRS). The single-voxel 1 H-MRS before treatment revealed an accumulation of lactate and a reduced N-acetyl aspartate (NAA)/total creatine (tCr) ratio with a z-score of -3.0. During treatment, the lactate signal faded away, whereas the NAA signal gradually increased to a z-score of -1.5 at 120 days of treatment. The choline/tCr ratio did not deviate much initially (z-score +0.5), but the ratio increased markedly during treatment (z-score +4.8). Consequently, the Cu-histidine therapy initiated after the critical period still improved the neuronal metabolism, suggesting that some Cu was delivered to neurons. Nevertheless, the brain atrophy, impaired myelination, and severe neurological symptoms were not ameliorated.

Original languageEnglish
Pages (from-to)297-300
Number of pages4
JournalBrain and Development
Volume27
Issue number4
DOIs
Publication statusPublished - 2005 Jan 1

Keywords

  • Copper-histidine therapy
  • Menkes disease
  • Proton magnetic resonance spectroscopy

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Developmental Neuroscience
  • Clinical Neurology

Fingerprint Dive into the research topics of 'The effects of copper-histidine therapy on brain metabolism in a patient with Menkes disease: A proton magnetic resonance spectroscopic study'. Together they form a unique fingerprint.

  • Cite this

    Munakata, M., Sakamoto, O., Kitamura, T., Ishitobi, M., Yokoyama, H., Haginoya, K., Togashi, N., Tamura, H., Higano, S., Takahashi, S., Ohura, T., Kobayashi, Y., Onuma, A., & Iinuma, K. (2005). The effects of copper-histidine therapy on brain metabolism in a patient with Menkes disease: A proton magnetic resonance spectroscopic study. Brain and Development, 27(4), 297-300. https://doi.org/10.1016/j.braindev.2004.08.002