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Proton magnetic resonance spectroscopic imaging in childhood ataxia with diffuse central nervous system hypomyelination.

Publication ,  Journal Article
Tedeschi, G; Schiffmann, R; Barton, NW; Shih, HH; Gospe, SM; Brady, RO; Alger, JR; Di Chiro, G
Published in: Neurology
August 1995

The spatial distribution of metabolite signal intensities can be measured within entire sections of the brain by proton magnetic resonance spectroscopic imaging (1H-MRSI). A group of six patients (4 unrelated girls and 2 brothers from 5 families) with childhood ataxia with diffuse CNS hypomyelination (CACH) underwent long-echo-time, single-slice 1H-MRSI. Relative to controls, there was a decrease in the signal intensity of N-acetylaspartate, choline, and creatine throughout the white matter in all six patients. We identified lactate signals in white matter in three of them with advanced disease. The degree of white matter involvement was not homogeneous over the entire patient group, but did correlate with clinical presentation. Deep and posterior white matter tended to be more involved. There were no 1H-MRSI abnormalities in the gray matter. 1H-MRSI findings suggest that this syndrome is secondary to a metabolic defect causing hypomyelination, axonal degeneration, and, in the most compromised cases, accumulation of lactate. This study shows that CACH is not limited to girls.

Duke Scholars

Published In

Neurology

DOI

ISSN

0028-3878

Publication Date

August 1995

Volume

45

Issue

8

Start / End Page

1526 / 1532

Location

United States

Related Subject Headings

  • Protons
  • Neurology & Neurosurgery
  • Myelin Sheath
  • Male
  • Magnetic Resonance Spectroscopy
  • Magnetic Resonance Imaging
  • Lactic Acid
  • Lactates
  • Humans
  • Gaucher Disease
 

Citation

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MLA
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Tedeschi, G., Schiffmann, R., Barton, N. W., Shih, H. H., Gospe, S. M., Brady, R. O., … Di Chiro, G. (1995). Proton magnetic resonance spectroscopic imaging in childhood ataxia with diffuse central nervous system hypomyelination. Neurology, 45(8), 1526–1532. https://doi.org/10.1212/wnl.45.8.1526
Tedeschi, G., R. Schiffmann, N. W. Barton, H. H. Shih, S. M. Gospe, R. O. Brady, J. R. Alger, and G. Di Chiro. “Proton magnetic resonance spectroscopic imaging in childhood ataxia with diffuse central nervous system hypomyelination.Neurology 45, no. 8 (August 1995): 1526–32. https://doi.org/10.1212/wnl.45.8.1526.
Tedeschi G, Schiffmann R, Barton NW, Shih HH, Gospe SM, Brady RO, et al. Proton magnetic resonance spectroscopic imaging in childhood ataxia with diffuse central nervous system hypomyelination. Neurology. 1995 Aug;45(8):1526–32.
Tedeschi, G., et al. “Proton magnetic resonance spectroscopic imaging in childhood ataxia with diffuse central nervous system hypomyelination.Neurology, vol. 45, no. 8, Aug. 1995, pp. 1526–32. Pubmed, doi:10.1212/wnl.45.8.1526.
Tedeschi G, Schiffmann R, Barton NW, Shih HH, Gospe SM, Brady RO, Alger JR, Di Chiro G. Proton magnetic resonance spectroscopic imaging in childhood ataxia with diffuse central nervous system hypomyelination. Neurology. 1995 Aug;45(8):1526–1532.

Published In

Neurology

DOI

ISSN

0028-3878

Publication Date

August 1995

Volume

45

Issue

8

Start / End Page

1526 / 1532

Location

United States

Related Subject Headings

  • Protons
  • Neurology & Neurosurgery
  • Myelin Sheath
  • Male
  • Magnetic Resonance Spectroscopy
  • Magnetic Resonance Imaging
  • Lactic Acid
  • Lactates
  • Humans
  • Gaucher Disease