Brain MR imaging features of patients with NCL
| NCL Subtypes | T2 hypointense thalami n (%) | T2 Hyperintensity n (%) | Volume Loss n (%) | ||||
|---|---|---|---|---|---|---|---|
| Corpus Striatum | STWM (PVWM and/or DWM and/or SCWM and/or PLIC) | Insular/Sub-Insular | Pons | Supratentorial | Cerebellar | ||
| CLN1 disease (n = 6) | 6 (100) | 6 (100) | 6 (100) | 4 (67) | 6 (100) | 6 (100) | 6 (100) |
| CLN2 disease (n = 5) | 5 (100) | 3 (60) | 5 (100) | 4 (80) | 3 (60) | 4 (80) | 4 (80) |
| CLN3 disease (n = 2) | 0 (0) | 0 (0) | 1 (50) | 1 (50) | 0 (0) | 2 (100) | 0 (0) |
| CLN5 disease (n = 1) | 1 (100) | 1 (100) | 1 (100) | 1 (100) | 1 (100) | 1 (100) | 1 (100) |
| CLN6 disease (n = 2) | 2 (100) | 1 (50) | 2 (100) | 1 (50) | 2 (100) | 1 (50) | 1 (50) |
| CLN7 disease (n = 6) | 6 (100) | 2 (33) | 6 (100) | 5 (83) | 5 (83) | 5 (83) | 6 (100) |
| CLN8 disease (n = 2) | 2 (100) | 2 (100) | 2 (100) | 2 (100) | 2 (100) | 2 (100) | 2 (100) |
Note:—STWM indicates supratentorial white matter.