FIG 1. Patient 1, who had GM1.

A and B, In vivo transverse T2-weighted image (A) and postmortem coronal 3D FLAIR image (B) show a diffuse and symmetric increase in signal intensity in the cerebral white matter, with radial stripes of low signal intensity (arrows).

C and D, Photomicrograph (hematoxylin and eosin, original magnification x1.5) of a matched histopathologic section (C) and magnified view (Luxol fast blue, original magnification x75) of the small box in C (D) reveal perivenular areas of relatively normal density of myelin sheaths (blue in D) in the hypomyelinated central white matter (top left and bottom right in D); these areas corresponding to the hypointense stripes on MR imaging.