Table 2:
Characteristic brain MR imaging findings in WSS
| Patient | Sex/Age (yr) | White Matter |
Descriptive Features on MRI |
Comments | ||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Iron Deposition |
Pituitary Atrophy | Diffusion Restriction | ||||||||||
| Frontal | Parietal | Temporal | Occipital | Infratentorial | Caudate | Putamen | RN/SN | |||||
| 1 | F/18 | + | + | − | + | − | − | − | − | + | + | − |
| 2 | M/19 | − | − | − | − | − | − | + | − | + | − | Initial study showed no iron deposition |
| 3 | F/29 | +++ | +++ | − | − | − | + | + | + | − | − | Extension of iron deposition on follow-up study |
| 4 | F/38 | ++ | ++ | + | + | − | + | + | − | + | + | Prominent perivascular spaces |
| 5 | F/35 | + | + | + | − | − | − | + | − | − | − | Prominent perivascular spaces |
| 6 | F/37 | +++ | +++ | + | + | − | − | + | − | + | − | Calcification of the dentate nucleus |
| 7 | M/45 | +++ | +++ | + | + | − | + | + | − | + | + | Loss of corpus callosum volume |
| 8 | M/22 | + | + | − | − | − | − | + | − | + | − | − |
| 9 | M/24 | + | + | − | − | − | − | + | − | − | − | Prominent perivascular spaces |
| 10 | M/23 | + | + | − | − | − | − | − | − | + | − | − |
| 11 | F/21 | + | + | − | − | − | − | + | − | + | − | − |
| 12 | M/16 | + | + | − | − | − | − | − | − | + | − | Prominent perivascular spaces |
| 13 | M/26 | + | + | + | + | − | − | + | + | + | − | − |
| 14 | M/21 | − | − | − | − | − | − | + | + | − | − | − |
| 15 | M/20 | + | − | − | − | − | − | − | − | + | − | − |
| 16 | F/21 | − | − | − | − | − | − | + | + | + | − | − |
| 17 | F/32 | + | + | + | + | − | − | + | − | + | − | − |
| 18 | F/22 | − | − | − | − | − | − | − | − | + | − | − |
| 19 | F/35 | +++ | +++ | + | + | + | − | + | − | + | − | Pontine WM signal |
| 20 | M/21 | − | − | − | − | − | − | + | + | + | − | − |
| 21 | F/27 | − | − | − | − | − | − | + | − | + | − | − |
| 22 | M/16 | − | − | − | − | − | − | − | − | + | − | − |
| 23 | F/41 | ++ | ++ | + | + | − | + | + | + | + | − | − |
| 24 | M/37 | ++ | ++ | + | + | − | + | + | − | + | − | Dentate nucleus calcification |
| 25 | F/17 | − | − | − | − | − | − | + | − | − | − | − |
| 26 | F/29 | + | + | + | + | + | − | − | − | − | − | Cerebellar WM signal |
Note:—RN indicates red nucleus; SN, substantia nigra; −, absent; +, mild (predominantly scattered lesions or confluent periventricular); ++, moderate (patchy scattered); +++, severe (predominantly diffuse, vanishing lesions).