Skip to main content
Advertisement

Main menu

  • Home
  • Content
    • Current Issue
    • Publication Preview--Ahead of Print
    • Past Issue Archive
    • Case of the Week Archive
    • Classic Case Archive
    • Case of the Month Archive
  • For Authors
  • About Us
    • About AJNR
    • Editors
    • American Society of Neuroradiology
  • Submit a Manuscript
  • Podcasts
    • Subscribe on iTunes
    • Subscribe on Stitcher
  • More
    • Subscribers
    • Permissions
    • Advertisers
    • Alerts
    • Feedback
  • Other Publications
    • ajnr

User menu

  • Subscribe
  • Alerts
  • Log in

Search

  • Advanced search
American Journal of Neuroradiology
American Journal of Neuroradiology

American Journal of Neuroradiology

  • Subscribe
  • Alerts
  • Log in

Advanced Search

  • Home
  • Content
    • Current Issue
    • Publication Preview--Ahead of Print
    • Past Issue Archive
    • Case of the Week Archive
    • Classic Case Archive
    • Case of the Month Archive
  • For Authors
  • About Us
    • About AJNR
    • Editors
    • American Society of Neuroradiology
  • Submit a Manuscript
  • Podcasts
    • Subscribe on iTunes
    • Subscribe on Stitcher
  • More
    • Subscribers
    • Permissions
    • Advertisers
    • Alerts
    • Feedback
  • Follow AJNR on Twitter
  • Visit AJNR on Facebook
  • Follow AJNR on Instagram
  • Join AJNR on LinkedIn
  • RSS Feeds
Research ArticlePEDIATRICS

Cranial MR Spectroscopy of Tetrahydrobiopterin Deficiency

Yin-Hsiu Chien, Shiin-Feng Peng, Tso-Ren Wang and Wuh-Liang Hwu
American Journal of Neuroradiology June 2002, 23 (6) 1055-1058;
Yin-Hsiu Chien
aDepartment of Pediatrics, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Shiin-Feng Peng
bDepartment of Medical Imaging, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Tso-Ren Wang
aDepartment of Pediatrics, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Wuh-Liang Hwu
aDepartment of Pediatrics, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan
cDepartment of Medical Genetics, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan
dDepartment of General Medicine, National Taiwan University College of Medicine, Taipei, Taiwan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • Article
  • Figures & Data
  • Info & Metrics
  • References
  • PDF
Loading

Article Figures & Data

Figures

  • Tables
  • Fig 1.
    • Download figure
    • Open in new tab
    • Download powerpoint
    Fig 1.

    MR imaging findings and MR spectroscopic findings for patient 1.

    A, Fluid-attenuated inversion recovery MR image (9000/110 [TR/TE]; inversion time, 2500 ms) of an 8-year-old male patient, obtained through the level of the centrum semiovale in the axial plane. Hyperintense areas (arrowhead) can be seen in the central white matter of the bilateral parietal cortex.

    B, Proton MR spectroscopy sampled at the left parietal cortex and central white matter of the same patient. A duplex peak resulting from lactate can be seen at approximately 1.3 ppm (arrow), and active demyelination was suspected.

Tables

  • Figures
  • MR imaging and spectroscopy data for individual patients affected by tetrahydrobiopterin deficiency

    Patient No.MR ImagesLactate PeakNAA:CRENAA:CHONAA:MIMI:CREMI:CHOCHO:CREPatient Age (yr) at MR ImagingPatient Age (yr) at IQ TestMR Image Phe (μmol/L)Average Phe (μmol/L)VIQPIQFSIQAverage Dosage (mg/kg/day)5-HTP
    BH4Levodopa
    1White matter T2 ↑+1.2591.4421.4440.8720.9990.8738.57.71771196455561.728.301.43
    2Negative−1.553 ↑1.3891.1188.56.91081038486831.204.040.65*
    3Negative−1.779 ↑1.5001.8880.9420.7941.1868.56.999695864571.747.841.21*
    4Prominent sulcation+5.944 ↑10.30 ↑2.3162.5674.4470.57786.4651047483762.339.532.43
    5Prominent sulcation−1.4872.659 ↑0.7991.8603.3280.55955.3451047882782.9210.532.19
    6Negative−0.645 ↓0.681 ↓1.52354.08310292106982.259.351.49
    7Negative−1.692 ↑3.192 ↑4.5770.3700.6970.53053.661068095921.949.022.64
    8Negative−1.4521.525 ↑1.2481.1631.2220.952474622.319.422.07
    • Note.—NAA indicates N-acetylaspartate; CRE, creatine; CHO, choline; MI, myo-inositol; IQ, intelligence quotient; Phe, phenylalanine; VIQ, verbal intelligence quotient; PIQ, performance intelligence quotient; FSIQ, full-scale intelligence quotient; BH4, tetrahydrobiopterin; 5-HTP, 5-hydroxytryptophan.

    • ↵* Patients 2 and 3 stopped 5-HTP supplementation at the ages of 7 and 6 years, respectively.

PreviousNext
Back to top

In this issue

American Journal of Neuroradiology: 23 (6)
American Journal of Neuroradiology
Vol. 23, Issue 6
1 Jun 2002
  • Table of Contents
  • Index by author
Advertisement
Print
Download PDF
Email Article

Thank you for your interest in spreading the word on American Journal of Neuroradiology.

NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.

Enter multiple addresses on separate lines or separate them with commas.
Cranial MR Spectroscopy of Tetrahydrobiopterin Deficiency
(Your Name) has sent you a message from American Journal of Neuroradiology
(Your Name) thought you would like to see the American Journal of Neuroradiology web site.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.
Citation Tools
Cranial MR Spectroscopy of Tetrahydrobiopterin Deficiency
Yin-Hsiu Chien, Shiin-Feng Peng, Tso-Ren Wang, Wuh-Liang Hwu
American Journal of Neuroradiology Jun 2002, 23 (6) 1055-1058;

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero
Share
Cranial MR Spectroscopy of Tetrahydrobiopterin Deficiency
Yin-Hsiu Chien, Shiin-Feng Peng, Tso-Ren Wang, Wuh-Liang Hwu
American Journal of Neuroradiology Jun 2002, 23 (6) 1055-1058;
del.icio.us logo Digg logo Reddit logo Twitter logo CiteULike logo Facebook logo Google logo Mendeley logo
  • Tweet Widget
  • Facebook Like
  • Google Plus One

Jump to section

  • Article
    • Abstract
    • Methods
    • Results
    • Discussion
    • References
  • Figures & Data
  • Info & Metrics
  • References
  • PDF

Related Articles

  • No related articles found.
  • PubMed
  • Google Scholar

Cited By...

  • Central Tegmental Tract Involvement in an Infant with 6-Pyruvoyltetrahydropterin Synthetase Deficiency
  • Diffusion Tensor Images in Children with Early-Treated, Chronic, Malignant Phenylketonuric: Correlation with Intelligence Assessment
  • Crossref
  • Google Scholar

This article has not yet been cited by articles in journals that are participating in Crossref Cited-by Linking.

More in this TOC Section

  • Transient Hyperintensity of the Infant Thyroid Gland on T1-Weighted MR Imaging: Correlation with Postnatal Age, Gestational Age, and Signal Intensity of the Pituitary Gland
  • Widening the Neuroimaging Features of Adenosine Deaminase 2 Deficiency
  • Evaluation of DISORDER: Retrospective Image Motion Correction for Volumetric Brain MRI in a Pediatric Setting
Show more PEDIATRICS

Similar Articles

Advertisement

News and Updates

  • Lucien Levy Best Research Article Award
  • Thanks to our 2020 Distinguished Reviewers
  • Press Releases

Resources

  • Evidence-Based Medicine Level Guide
  • How to Participate in a Tweet Chat
  • AJNR Podcast Archive
  • Ideas for Publicizing Your Research
  • Librarian Resources
  • Terms and Conditions

Opportunities

  • Share Your Art in Perspectives
  • Get Peer Review Credit from Publons
  • Moderate a Tweet Chat

American Society of Neuroradiology

  • Neurographics
  • ASNR Annual Meeting
  • Fellowship Portal
  • Position Statements

© 2021 by the American Society of Neuroradiology | Print ISSN: 0195-6108 Online ISSN: 1936-959X

Powered by HighWire