Table 4.
Neuropathological features in standard and long-duration ALS.
| Non-ventilated§ | Ventilated§ | |||||
|---|---|---|---|---|---|---|
| Standard (n = 66) | Long (n = 54) | P-value | Standard (n = 30) | Long (n = 20) | P-value | |
| Loss of Anterior Horn Cells§, Mean ± SEM | 2.86 ± 0.47 | 2.31 ± 0.12 | <0.001† | 2.89 ± 0.62 | 2.88 ± 0.12 | 0.612† |
| Degeneration of Ventral Roots¶, Mean ± SEM | 2.61 ± 0.13 | 2.04 ± 0.19 | 0.016† | 2.69 ± 0.12 | 2.62 ± 0.24 | 0.736† |
| Motor Cortex Degeneration, Mean ± SEM | 2.30 ± 0.15 | 2.20 ± 0.14 | 0.566† | 2.21 ± 0.26 | 2.18 ± 0.33 | 0.976† |
| Lateral Cortical Spinal Tracts Degeneration¶, Mean ± SEM | 2.14 ± 0.14 | 1.71 ± 0.16 | 0.060† | 2.42 ± 0.22 | 2.15 ± 0.30 | 0.424† |
| Hypoglossal Nerve Degeneration, Mean ± SEM | 1.91 ± 0.15 | 1.74 ± 0.15 | 0.528† | 2.32 ± 0.17 | 2.00 ± 0.22 | 0.269† |
| TDP-43 Positive, ± (%) | 58/5 (92%) | 25/28 (47%) | <0.001‡ | 24/5 (83%) | 12/7 (63%) | 0.117‡ |
| TDP-43 Stage¶, Mean ± SEM | 1.59 ± 0.19 | 1.33 ± 0.33 | 0.373† | 1.50 ± 0.34 | 1.33 ± 0.33 | 0.819† |
| Nishihira TDP-43 Type† | 0.656‡ | 0.584‡ | ||||
| Type 1 | 45 (94%) | 21 (91%) | 18 (90%) | 8 (72%) | ||
| Type 2 | 3 (6%) | 2 (9%) | 2 (10%) | 3 (28%) | ||
| Lewy Body Disease, ± (%) | 4/39 (9%) | 10/36 (22%) | 0.147‡ | 2/19 (10%) | 3/11 (21%) | 0.369‡ |
| Arteriolosclerosis, Mean ± SEM | 1.66 ± 0.12 | 1.79 ± 0.10 | 0.404† | 1.71 ± 0.15 | 2.14 ± 0.18 | 0.084† |
| Atherosclerosis, Mean ± SEM | 0.66 ± 0.14 | 0.82 ± 0.15 | 0.455† | 0.47 ± 0.19 | 0.33 ± 0.17 | 0.829† |
| Braak Neurofibrillary Stage†, Median (25th, 75th) | II (I, III) | II (II, III) | 0.083† | I, (0, II) | II (0, II) | 0.467† |
| CERAD§§, Median (25th, 75th) | 0 (0, I) | 1 (0, 1) | 0.368† | 0 (0, 0) | 0 (0, 1) | 0.085† |
| Pathological changes in AD¶¶, ± (%) | 23/22 (51%) | 23/19 (55%) | 0.831‡ | 6/14 (30%) | 4/9 (31%) | 1.000‡ |
Mann-Whitney U test.
χ2 test of variance.
Cases where ventilation status prior to death could not be determined were excluded from analysis
Analysis conducted using global scores of cervical, thoracic and lumbar spinal cord regions
Analysis only includes cases positive for TDP-43 inclusions and assessed in all regions
Braak neurofibrillary tangle staging was rated as 0-VI based on established guidelines.
CERAD plague density was rated as none (0), sparse (1), moderate (2) or frequent (3).
Neuropathological changes in Alzheimer disease were determined by the NIA-Alzheimer Association’s guidelines. Cases were coded as positive if the degree of Alzheimer pathology was low, intermediate, or high.
Bold values indicate significance of P < 0.05