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.