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Updated: Jun 4, 2026

Assessment and Evaluation of the High Risk Neonate: The NICU Network Neurobehavioral Scale

Assessment and Evaluation of the High Risk Neonate: The NICU Network Neurobehavioral Scale

Published on: August 25, 2014

Neonatal non-ketotic hyperglycinemia.

M Nicolasjilwan¹, H Ozer, M Wintermark

¹University of Virginia Health System, Department of Radiology, Division of Neuroradiology, Charlottesville, VA 22908, United States. manaljilwan@hotmail.com

Journal of Neuroradiology = Journal De Neuroradiologie

|March 1, 2011

Summary

Neonatal non-ketotic hyperglycinemia imaging findings are rare. This case highlights characteristic MRI and MR spectroscopic findings in a neonate with this condition.

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Area of Science:

Neurology
Radiology
Biochemistry

Background:

Neonatal non-ketotic hyperglycinemia (NKH) is a rare inherited metabolic disorder.
It results from glycine cleavage system dysfunction, leading to elevated glycine levels.
Typical imaging findings in neonatal NKH are infrequently documented.

Observation:

This study presents a case of neonatal NKH diagnosed at 6 days of age.
The patient underwent Magnetic Resonance Imaging (MRI) and Magnetic Resonance Spectroscopy (MRS).

Findings:

Characteristic MRI findings included specific patterns of white matter abnormalities and cerebral atrophy.
MR spectroscopic analysis revealed elevated glycine levels in the brain.
These findings are crucial for early diagnosis and management.

Implications:

Accurate and early diagnosis of NKH is vital for timely intervention.
Understanding characteristic imaging findings aids radiologists in identifying this rare condition.
This case contributes to the limited literature on NKH imaging, aiding future diagnostic efforts.