Table 3. Brain Magnetic Resonance Imaging (MRI) Characteristics Before and After Hematopoietic Stem Cell Transplantation (HSCT).
| Characteristic | MRI Characteristic (N=36) | |
|---|---|---|
| Favorable, No. (LS) | Unfavorable, No. (LS) | |
| Status Before HSCT | ||
| MRI patterna | ||
| Parieto-occipital | 16 (LS <9) | 6 (LS ≥9) |
| Frontal involvement | 2 (LS <4) | 4 (LS ≥4) |
| Others (n = 8) | ||
| Projection fibers only | NA | 2 (LS: 1, 5.5)b,c |
| Cerebellum or basal ganglia | NA | 4 (LS: 2.5, 8.5, 8.5, 12.5)b |
| Parieto-occipital + frontal involvement | NA | 2 (LS: 8, 10)b |
| Projection fibers involved, No. (%) | ||
| Pons | 3 (17) | 12 (67)d |
| Capsula interna, partial/completee | 5 (28) / 0 | 7 (39) / 5 (28) |
| ALD-DRS = 0 | 17 (94) | 1 (6)d |
| NFS = 0 | 17 (94) | 4 (22) |
| Status After HSCT | ||
| Gadolinium enhancement >6 mo after HSCT, No. (%)f | 0 of 16 | 2 of 15 (13) |
| Stable MRI during first year after HSCTg | 15 (83) | 4 (22)d |
| Epilepsy | 0 | 13 (72)d |
| Change in LS 12 mo After HSCT | ||
| Median (range [IQR]) | 1.0 (0 to 2.0 [0 to 2.0]) | 5 (-2.5 to 10.0 [2.5 to 7.0])d |
Abbreviations: ALD-DRS, adrenoleukodystrophy disability rating score; IQR, interquartile range; LS, Loes score; NA, not applicable; NFS, neurological function score.
Demyelination patterns according to Loes et al25 with modifications. Greater LS numbers indicate more demyelinating lesions.
Loes score of respective patients.
Patient 26 with demyelination of brainstem only without gadolinium enhancement.
Significance level (P < .05) between favorable and unfavorable for a respective characteristic.
Capsula interna partial is the incomplete or unilateral demyelination of tracts in the internal capsule.
All patients with transplant-related mortality and those who died within 12 months of disease progression were omitted (n = 5).
Change in LS of at least 2 points in the first year after HSCT.