Table 2.
Pathologic features and phosphorylated TDP-43 pathology in three types of sporadic ALS
| Type 1 | Type 2a | Type 2b | P-value | |
|---|---|---|---|---|
| (n = 63) | (n = 22) | (n = 11) | ||
| Neuronal lossa | ||||
| Motor cortex | 2.1 ± 0.7 | 2.1 ± 0.7 | 2.5 ± 0.7 | 0.073 |
| Anterior horns (cervical and lumbar) | 2.2 ± 0.5 | 2.1 ± 0.6 | 1.5 ± 0.4* | 0.002 |
| Hypoglossal nucleus | 2.3 ± 0.7 | 2.1 ± 0.7 | 1.4 ± 0.5** | <0.001 |
| Frontotemporal degeneration (cases) | 4 (6 %)*** | 8 (36 %) | 7 (64 %) | <0.0001 |
| pTDP-43 pathology (cases) | ||||
| NCIs: temporal-predominant type | NA | 13 (59 %) | 5 (45 %) | NA |
| DNs: many in neostriatum and globus pallidus | 1 (2 %) | 2 (9 %) | 9 (82 %)**** | <0.0001 |
ALS amyotrophic lateral sclerosis, pTDP-43 phosphorylated TDP-43, NCI neuronal cytoplasmic inclusion, DN dystrophic neurite, NA non-applicable
a0 = absent, 1 = mild, 2 = moderate, 3 = severe. Data are expressed as mean ± standard deviation
*P <0.001 vs. Type 1 and P = 0.018 vs. Type 2a, **P = 0.001 vs. Type 1 and P = 0.022 vs. Type 2a (Kruskal-Wallis test with post-hoc Steel-Dwass test), ***P <0.001 vs. Type 2a and P = 0.00001 vs. Type 2b (Fisher’s exact test with Ryan’s multiple comparison test), ****P <0.00001 vs. Type 1 and P <0.0001 vs. Type 2a (Fisher’s exact test with Ryan’s multiple comparison test)