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

研究成果: Article査読

13 被引用数 (Scopus)

抄録

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.

本文言語English
ページ(範囲)297-300
ページ数4
ジャーナルBrain and Development
27
4
DOI
出版ステータスPublished - 2005 6

ASJC Scopus subject areas

  • 小児科学、周産期医学および子どもの健康
  • 発達神経科学
  • 臨床神経学

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