Proposed MR imaging features of hypothalamic malformations in patients with DFNX2 compared with age-matched controlsa
| Patients with DFNX2 (n = 10) | Age-Matched Controls (n = 69) | |
|---|---|---|
| Axial T2 | ||
| Folded appearance | 70% (P < .001; κ = 0.95) | 0% |
| Bilateral abnormal internal or external cleft | 60% (P < .001; κ = 0.83) | 0% |
| Coronal T2 | ||
| Concave medial eminence | 75% (P < .001; κ = 0.78) | 0% |
| Right hypothalamic-septum angle | 115.5° ± 17.34° (P < .001) | 79° ± 8.05° |
| ICC = 0.88 (95% CI, 0.75–0.95) | ||
| Left hypothalamic-septum angle | 113.87° ± 16.87° (P < .001) | 81.87° ± 9.32° |
| ICC = 0.77 (95% CI, 0.58–0.89) | ||
| Right forebrain-hypothalamus length (mm) | 7.15 ± 3.02 (P < .001) | 4.26 ± 0.7 |
| ICC = 0.92 (95% CI, 0.70–0.97) | ||
| Left forebrain-hypothalamus length (mm) | 6.9 ± 2.1 (P < .001) | 4.21 ± 0.73 |
| ICC = 0.87 (95% CI, 0.70–0.95) |
Note:—κ indicates the Cohen κ coefficient.
↵a Data are mean values ± standard deviation. P values correspond to Wilcoxon/Mann-Withney test for differences in means of DFNX2 versus age-matched controls.