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Fig 1. MR imaging and 1H-MR spectroscopy in a patient with FXTAS (case 1). Sagittal T1-weighted gradient recalled-echo image (TR = 25 ms, TE = 4.6 ms, flip angle = 30°; 1 NEX) (A) shows diffuse brain atrophy with sparing of the oval shape of the basis pontis. Axial T2-weighted spin-echo images (TR = 2400 ms, TE = 200 ms, 1 NEX) (B and C) show symmetric hyperintensity in the middle cerebellar peduncles (arrows in B) and diffuse hyperintensity in the cerebral periventricular white matter (C). 1H-MR spectroscopy of the pons (D) shows decreased NAA/Cr ratio and a normal Cho/Cr ratio.