Neonatal Citrullinemia: Comparison of Conventional MR, Diffusion-Weighted, and Diffusion Tensor Findings
Charles B. L. M. Majoiea,
Jeroen M. Mourmansb,
Erik M. Akkermana,
Marinus Duranc and
Bwee Tien Poll-Theb
a Department of Radiology, Academic Medical Center, Amsterdam, the Netherlands
b Department of Pediatric Neurology, Academic Medical Center, Amsterdam, the Netherlands
c Laboratory of Genetic Metabolic Diseases, Academic Medical Center, Amsterdam, the Netherlands
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FIG 1. Images from the case of a 8-day-old girl with citrullinemia. MR imaging was performed 2 days after a blood ammonia peak of 2083 µmol/L.
A, Axial fast spin-echo T2-weighted MR image (3500/90/1) shows high signal intensity in the bilateral basal ganglia, thalami cortex, and subcortical white matter.
B, Diffusion-weighted MR image demonstrates restricted diffusion in these areas.
C, ADC map corresponding to area shows decreased ADC in these areas consistent with cytotoxic edema.
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FIG 2. Follow-up MR imaging at age 4 months, when the patient had severe neurodevelopmental delay.
A, Axial fast spin-echo T2-weighted MR image (3500/90/1) shows atrophy, ulegyric changes, and subcortical cysts, especially in the occipital lobes.
B, Color-coded fractional anisotropy map demonstrates fractional anisotropy (FA) decrease throughout the brain. Red refers to FA = 1 and purple to FA = 0.
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