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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 27:208-211, January 2006
© 2006 American Society of Neuroradiology

CASE REPORT
PEDIATRICS

Sequential MR Imaging Changes in Nonketotic Hyperglycinemia

J. Mourmans^a, C.B.L.M. Majoie^b, P.G. Barth^a, M. Duran^c, E.M. Akkerman^b and B.T. Poll-The^a

^a Departments of Pediatric Neurology, Emma Children’s Hospital, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands
^b Radiology, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands
^c Laboratory of Genetic Metabolic Diseases, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands

Address correspondence to: Professor B.T. Poll-The, Department of Pediatric Neurology (G8-211), Academic Medical Center, Emma Children’s Hospital, Meibergdreef 9, P.O. Box 22660, 1100 DD Amsterdam, the Netherlands

SUMMARY: Serial diffusion-weighted (DWI) and diffusion tensor imaging (DTI) were performed in a patient with neonatal onset nonketotic hyperglycinemia (NKH). At 3 weeks areas that are normally myelinated at this time showed increased T2-signal intensity and restricted diffusion, consistent with vacuolating myelinopathy. At 3 months, these areas had increased in the topographic pattern of normal myelination, whereas fractional anisotropy was compatible with axonal sparing. At 17 months, diffusion restriction had disappeared, likely because of coalescence of myelin vacuoles. A decrease of fractional anisotropy was observed in the previously myelinated areas indicative of axonal loss. We conclude that DWI and DTI can be used to identify and characterize white matter tract abnormalities in patients with NKH.

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