RT Journal Article
SR Electronic
T1 Sequential MR Imaging Changes in Nonketotic Hyperglycinemia
JF American Journal of Neuroradiology
JO Am. J. Neuroradiol.
FD American Society of Neuroradiology
SP 208
OP 211
VO 27
IS 1
A1 J. Mourmans
A1 C.B.L.M. Majoie
A1 P.G. Barth
A1 M. Duran
A1 E.M. Akkerman
A1 B.T. Poll-The
YR 2006
UL http://www.ajnr.org/content/27/1/208.abstract
AB 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.