PMC2626699_01
Initial MR images obtained at age of 10 months.. A. T2-wieghted axial image (TR/TE = 4700/104 msec, 5 mm thickness) demonstrates symmetric hyperintense lesion in both deep periventricular white matters (arrows).. B. Diffusion weighted image (TR/TE = 3500/92, b maximum = 1000 s/mm2, three directions, 5 mm thickness) shows the bright signal intensity of the lesion.. C. On the apparent diffusion coefficient map, the lesion shows diffuse low signal intensities, suggesting restricted diffusion.. D. Three dimensional MR angiography shows markedly tortuous intracranial and extracranial vessels, which are characteristics of Menkes disease.
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F1
T2-weighted images demonstrated symmetric hyperintense lesions in both deep cerebral white matters (Fig. 1A). There was mild atrophy of the cerebellum and brainstem, yet atrophy of the cerebral hemispheres was not prominent. DWI demonstrated high signal intensity of the lesions, and their apparent diffusion coefficients (ADCs) were decreased on the ADC maps (Figs. 1B, C). Three dimensional MR angiography (Fig. 1D) showed markedly tortuous intracranial and extracranial vessels, which are characteristic of Menkes disease.
Follow up diffusion study obtained at the age of 13 months.. A, B. Consecutive diffusion-weighted images (TR/TE = 3500/95, b maximum = 1000 s/mm2) demonstrate marked extension of the white matter lesions to the adjacent white matter. The lesion also involves the corpus callosum (arrows). Note there is no definite progression of cortical atrophy.. C, D. Apparent diffusion coefficient maps of the lesion show diffusely decreased apparent diffusion coefficient, similar to that of the initial lesions (Fig. 1C).
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F2
The lesions extended to the deep white matter and corpus callosum (Figs. 2A, B). The extended lesions also showed high signal intensity on DWI and decreased ADC, similar to those of the initial lesions (Figs. 2C, D).