Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

Sequential MR imaging changes in nonketotic hyperglycinemia.

J Mourmans1, C B L M Majoie, P G Barth

  • 1Department of Pediatric Neurology, Emma Children's Hospital, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands.

AJNR. American Journal of Neuroradiology
|January 19, 2006
PubMed
Summary
This summary is machine-generated.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Cardiac thrombus dissolution in acute ischemic stroke: A substudy of Mind the Heart.

Heliyon·2023
Same author

Correlation between EEG spectral power and cerebral perfusion in patients with acute ischemic stroke.

Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia·2023
Same author

Detection of patent foramen ovale in patients with ischemic stroke on prospective ECG-gated cardiac CT compared to transthoracic echocardiography.

Journal of neurology·2023
Same author

Infarct Evolution in Patients with Anterior Circulation Large-Vessel Occlusion Randomized to IV Alteplase and Endovascular Treatment versus Endovascular Treatment Alone.

AJNR. American journal of neuroradiology·2023
Same author

Unsupervised Deep Learning for Stroke Lesion Segmentation on Follow-up CT Based on Generative Adversarial Networks.

AJNR. American journal of neuroradiology·2022
Same author

Tranexamic acid to prevent operation in chronic subdural haematoma (TORCH): study protocol for a randomised placebo-controlled clinical trial.

Trials·2022
Same journal

Multiparametric MRI Model Predicts Parenchymal Hematoma in Acute Ischemic Stroke After Reperfusion.

AJNR. American journal of neuroradiology·2026
Same journal

Age- and Region-Stratified Growth in Emergency Department Neuroimaging Utilization within Epic Cosmos, 2016-2025.

AJNR. American journal of neuroradiology·2026
Same journal

Neuroradiology Leads NIH Funding Among Clinician Diagnostic Radiologists: A 14-Year National Analysis.

AJNR. American journal of neuroradiology·2026
Same journal

Neutral Cervical Spine MRI is Not Enough: The Critical Role of Flexion Imaging in Hirayama disease in Pediatric Patients.

AJNR. American journal of neuroradiology·2026
Same journal

CT Evaluation of Osseous Trauma at the Craniocervical Junction: A Pattern-Based Overview.

AJNR. American journal of neuroradiology·2026
Same journal

Comprehensive Structural MRI Phenotyping in <i>Oligophrenin 1-</i>Related Disorder Reveals Characteristic Brain Malformations.

AJNR. American journal of neuroradiology·2026
See all related articles

Serial diffusion imaging reveals white matter changes in neonatal onset nonketotic hyperglycinemia (NKH). Diffusion-weighted imaging (DWI) and diffusion tensor imaging (DTI) show vacuolating myelinopathy and axonal loss, aiding diagnosis.

Area of Science:

  • Neuroimaging
  • Neurology
  • Pediatric Neurology

Background:

  • Neonatal onset nonketotic hyperglycinemia (NKH) is a rare metabolic disorder affecting glycine metabolism.
  • White matter abnormalities are a known complication of NKH, but their evolution is not well characterized.
  • Advanced neuroimaging techniques like DWI and DTI offer potential for detailed assessment.

Observation:

  • Serial diffusion-weighted (DWI) and diffusion tensor imaging (DTI) were conducted on a patient with neonatal onset nonketotic hyperglycinemia (NKH).
  • Early imaging (3 weeks) demonstrated increased T2 signal and restricted diffusion in normally myelinated areas, suggesting vacuolating myelinopathy.
  • Later imaging (3 months and 17 months) revealed evolving patterns of diffusion restriction and fractional anisotropy changes.

Findings:

Related Experiment Videos

  • At 3 weeks, vacuolating myelinopathy was evident with restricted diffusion in white matter tracts.
  • At 3 months, imaging showed increased affected areas in a normal myelination pattern with preserved fractional anisotropy, indicating axonal sparing.
  • By 17 months, diffusion restriction resolved, but decreased fractional anisotropy suggested subsequent axonal loss in previously affected regions.

Implications:

  • DWI and DTI are valuable tools for identifying and characterizing white matter tract abnormalities in NKH.
  • These imaging techniques can track the dynamic changes in white matter, including myelin vacuolation and axonal integrity.
  • Understanding these imaging patterns may aid in prognostication and management strategies for patients with NKH.