Article Figures & Data
Figures
- FIG 1.
Sagittal, coronal, and axial MR images show pons signal abnormalities as T1 hypointensity and T2 hyperintensity in a patient with Wolfram syndrome (white arrows). Brain stem atrophy is also evident on this MR image.
- FIG 2.
Examples of neuroradiologic findings in patients with Wolfram syndrome. A, Thinning of the optic chiasm (white box) as seen in coronal T1-weighted images (left, patient with Wolfram syndrome; right, healthy control). B, Abnormal PPBS signal (white circles) in midline sagittal T1-weighted images (from left to right, absent, diminished, and physiologic signal). C, White matter hyperintensities in FLAIR MR imaging (white arrowheads).
- FIG 3.
Marked (A) and mild (B) cerebellar atrophy in patients with Wolfram syndrome, as shown in sagittal, coronal, and axial T1-weighted MR images (white arrows), compared with a healthy control (C).
- FIG 4.
A, Frequency of neuroradiologic signs in patients with Wolfram syndrome at first and last visits. B, The relationship between age and the number of neuroradiologic signs. A line connecting a circle-shaped point (first visit) and a triangle-shaped point (last visit) represents each patient.
Tables
- Table 1:
The demographic and clinical characteristics in patients with Wolfram syndrome at first and last follow-up visitsa
First Visit (n = 30) Last Visit (n = 30) Age (yr) 14 ± 6 19 ± 6 Duration of disease (yr) 3 ± 3 8 ± 4 Diabetes mellitus 29 (97) 30 (100) Vision impairment 28 (93) 28 (93) Hearing loss 20 (67) 23 (77) Diabetes insipidus 15 (50) 19 (63) Bladder dysfunction 13 (43) 26 (86) ↵a For the age and duration of disease, means and SDs are reported. For comorbid conditions, numbers and percentages are reported.
