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A case of cerebral infarction with transient cerebral arteriopathy in a 12-year-old female is described. The child showed with an acute onset of left hemiplegia, central facial palsy and aphasia. She was diagnosed with right internal carotid artery obstruction with no predisposing factors for cerebral infarction. A serial MR image and MR spectroscopy of the brain was performed. MR angiography revealed reversible vascular changes in the right middle cerebral artery after several months. The ratio of choline/creatine was elevated and that of N-acetylaspartate (NAA)/creatine was depressed in the cerebral lesion, however, they were normalized during the remission phase with long echo time-MR spectroscopy. The elevation of the lactate peak was clarified with short echo time-MR spectroscopy. The longitudinal follow up using MR angiography, and MR spectroscopy with long and short echo time was useful for the evaluation of reversible vascular change with cerebral infarction in children. [Copyright &y& Elsevier]

Abstract: Serial study using MR imaging and 1H-MR spectroscopy (1H-MRS) of unidentified bright objects (UBO) in a 9-year-old boy with neurofibromatosis type 1 (NF1) is described. UBO was indicated in the left globus pallidum at 3 years old, then appeared in the right globus pallidum at 5 years old along with left hemiconvulsion. These UBO gradually disappeared over several years. On 1H-MRS, the ratios of N-acetylaspartate (NAA)/creatine and that of choline/creatine were each mildly reduced in the UBO. These ratios improved in parallel with disappearance of the UBO. In addition, elevation of the lactate/lipid peak was shown in UBO during the early stage, however, it disappeared in the latter. The longitudinal follow-up using MR image and 1H-MRS was useful for metabolic evaluation of UBO in patients with NF1. [Copyright &y& Elsevier]

Abstract: Van der Knaap disease, characterized by megalencephalic leukoencephalopathy and subcortical cysts, is a rare and recently defined condition. We discuss here the MR image (MRI) and MR spectroscopy (MRS) features in a 30-year-old man with S93L homozygous mutation in the MLC1 gene. MRI demonstrated high intensity diffuse white matter with T2-weighted image and subcortical cysts in the parietal and temporal lobes and MRS showed mildly reduced N-acetylaspartate (NAA) in areas of severe T2 elongation with a long TE sequence. A peak of lactate/lipid was indicated at a chemical shift of 1.3ppm with a short TE sequence. The peak for myo-inositol was normal in areas of severe and mild T2 elongation with short TE MRS. These findings suggest that demyelination progresses slowly in van der Knaap disease and that MRS with long and short TE is useful for the evaluation of neural metabolization associated with van der Knaap disease. [Copyright &y& Elsevier]