A 3-year-old boy with Lowe syndrome had bilateral periatrial hyperintense lesions intermixed with small cyst-like changes in the periatial regions of the deep white matter at MR imaging. Proton MR spectroscopy revealed prominent myoinositol peaks suggesting the presence of gliosis. b=1000s/mm(2) images of diffusion MR imaging were negative for the periatrial lesions. ADC maps, however, revealed high ADC values (1.76, and 1.66x10(-3)mm(2)/s), compared to the normal brain parenchyma (0.81x10(-3)mm(2)/s). These diffusion MR imaging findings likely represented gliosis.