TABLE 2.
Groupwise Comparisons of Average Pathologic Scores
| Disease Duration (Quartiles) |
C9orf72 Expansion |
|||||||
|---|---|---|---|---|---|---|---|---|
| Variable | Q1 (n = 10) | Q2 (n = 9) | Q3 (n = 9) | Q4 (n = 10) | p value | No (n = 28) | Yes (n = 9) | p value |
| Months Survival (Average) | 11 − 24 (18.6) | 25 − 36 (28.4) | 37 − 58 (44.0) | 60 − 117 (81.6) | <0.0005 | 50.8 | 25.4 | <0.0005 |
| Progression Rate* | Very Fast | Fast | Intermediate | Slow | − | − | − | − |
| Age at Onset | 66.0 | 59.1 | 56.3 | 62.2 | 0.036 | 60.5 | 62.1 | 0.60 |
| H&E Pathologic Rating | ||||||||
| Lumbar Cord | 2.1 | 2.1 | 2.6 | 2.4 | 0.3981 | 2.4 | 2.0 | 0.1804 |
| Hypoglossal Nucleus | 1.9 | 2.1 | 2.1 | 2.5 | 0.3115 | 2.0 | 2.3 | 0.3555 |
| Motor Cortex | 1.4 | 1.2 | 1.4 | 1.6 | 0.7936 | 1.4 | 1.4 | 0.9583 |
| TDP-43 Density | ||||||||
| Lumbar Cord | 18.4 | 15.3 | 17.9 | 6.9 | 0.0196 | 13.3 | 19.5 | 0.2412 |
| Hypoglossal Nucleus | 23.0 | 13.6 | 14.1 | 5.7 | 0.0118 | 14.3 | 15.0 | 0.8907 |
| Motor Cortex | 16.4 | 18.9 | 21.2 | 18.1 | 0.9165 | 19.8 | 16.6 | 0.5984 |
*
Approximate progression rate of each duration quartile. Progression rate and duration were significantly correlated (p < 0.01) (see “Results”).