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, Syouki Takahashi, Toshihiro Ohura, Yasuko Kobayashi, Akira Onuma, Kazuie Iinuma

研究成果: Article

13 引用 (Scopus)

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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 ( 1H-MRS). The single-voxel 1H-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.

元の言語English
ページ(範囲)297-300
ページ数4
ジャーナルBrain and Development
27
発行部数4
DOI
出版物ステータスPublished - 2005 6

ASJC Scopus subject areas

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

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    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