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Table 2: 
Neuroradiologic findings in brain MR images in patients with Wolfram syndromea
Radiologic SignFirst Visit (n = 30)Last Visit (n = 30)
Negative PPBS signb16 (53)21 (70)
T1/T2 pons signal abnormalities16 (53)20 (67)
Optic nerve atrophy9 (30)24 (80)c
White matter T2 hyperintensitiesd8 (27)10 (33)
Cerebellum atrophy7 (23)21 (70)c
  • ↵a Numbers and percentages are reported.
  • ↵b Absent and diminished PPBS sign.
  • ↵c P value < .01 (χ2 test).
  • ↵d FLAIR images were not available to evaluate white matter T2 hyperintensity for 1 scan, and T2-weighted images were used instead.