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Diffusion-Weighted MR Imaging in Neonatal Nonketotic Hyperglycinemia

Pek-Lan Khonga, Barbara C. C. Lamb, Brian H. Y. Chungb, Ka-Yin Wongb and Gaik-Cheng Ooia

a Department of Diagnostic Radiology, Queen Mary Hospital, University of Hong Kong
b Department of Pediatrics, Queen Mary Hospital, University of Hong Kong



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  		FIG 1. Patient 1, a 15-day-old neonate who presented with encephalopathy.

Axial fast spin-echo T2-weighted images (140/3800/2[TE/TR/NEX]) of the midbrain (A) and pons (B) show hyperintense lesions in the dorsal midbrain and pons (arrows). Axial diffusion-weighted MR images (100/10000/1000 [TE/TR/b factor]) of the midbrain (C) and pons (D) taken at the same levels show hyperintense lesions of restricted diffusion. The lesions are more conspicuous than on T2-weighted images, and additional lesions of restricted diffusion are seen in the bilateral cerebral peduncles (C, arrowheads). Susceptibility artifact is noted in the right frontal region from an ECG lead. Apparent diffusion coefficient maps show corresponding reduced apparent diffusion coefficient in the midbrain (E) and pons (F). Axial diffusion-weighted MR image (G) and apparent diffusion coefficient map (H) show restricted diffusion in the posterior limbs of the internal capsules (arrows). Susceptibility artifact is noted in the right frontal region from an ECG lead. Region of interest measurements of the posterior limbs of the right and left internal capsule show reduction of apparent diffusion coefficients to 0.87 µm2/msec and 0.97 µm2/msec, respectively. No signal intensity abnormality is seen in the corresponding fast spin-echo T2-weighted image (I).



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  		FIG 2. A 7-day old neonate, the older sister of patient 1, also presented with neonatal encephalopathy.

Axial fast spin-echo T2-weighted image (130/4200/1[TE/TR/NEX]) of the midbrain shows similar hyperintense lesions in the posterior midbrain (arrowheads).





Copyright © 2009 by the American Society of Neuroradiology. Print ISSN: 0195-6108 Online ISSN: 1936-959X