Table 2.
Non-ALS and ALS patient information
| non-ALS patients | Neural tissue | Diagnosis | Age | Gender | Disease duration | Pathology |
|---|---|---|---|---|---|---|
| Negative 1 | TSC, MCx | – | 67 | Male | – | Mild patchy ischemic changes, moderate patchy atherosclerosis |
| Negative 2 | TSC, MCx | – | 71 | Male | – | Mild hypoxic ischemic changes |
| Negative 3 | TSC, MCx | – | 70 | Male | – | Moderate hypertensive vasculopathy |
| Negative 4 | TSC, MCx | – | 75 | Male | – | Focal hypoxic ischemic changes |
| Negative 5 | TSC, MCx | – | 71 | Male | – | Mild acute hypoxic encephalopathy, moderate atherosclerosis |
| Negative 6 | CSC | – | – | – | – | – |
| Negative 7 | CSC | – | – | – | – | – |
| Negative 8 | CSC, TSC, MCx | CBD | 84 | Female | – | Corticobasal degeneration (cortical pTau inclusions) |
| Negative 9 | CSC, TSC, MCx | Clinical FTD | 65 | Male | – | N/A |
| Negative 10 | CSC, TSC, MCx | AD/DLBD | 73 | Male | – | AD/DLBD |
| Negative 11 | CSC | Alcoholic cirrhosis | 46 | Male | – | Cerebral atrophy |
| Negative 12 | CSC | Respiratory failure | 81 | Male | – | LM fibrosis of spinal cord, no ALS pathological change |
| ALS patients | Neural tissue | Diagnosis | Age | Gender | Disease duration (months) | Neuropathology |
|---|---|---|---|---|---|---|
| SOD1 fALS patient 1 | CSC | SOD1 ALS | 67 | Female | 24 | ALS-SOD |
| SOD1 fALS patient 2 | CSC | SOD1 ALS | 76 | Female | 6 | ALS-SOD |
| SOD1 fALS patient 3 | CSC | SOD1 ALS | 46 | Male | 36 | ALS-SOD |
| SOD1 fALS patient 4 | CSC | SOD1 ALS | 67 | Male | 120 | ALS-SOD |
| SOD1 fALS patient 5 | CSC | SOD1 ALS | 63 | Male | 192 | ALS-SOD |
| sALS patient 1 | CSC | sALS | 57 | Male | 12 | ALS-TDP, FTLD-TDP |
| sALS patient 2 | CSC | sALS | 65 | Female | 24 | ALS-TDP |
| sALS patient 3 | CSC | sALS | 67 | Male | 24 | ALS-TDP, senile plaques (CERAD moderate) |
| sALS patient 4 | CSC | sALS | 75 | Male | 12 | ALS-TDP, AD diffuse LBD |
| sALS patient 5 | CSC | sALS | 51 | Male | 24 | ALS-TDP, FTLD-TDP |
| sALS patient 6 | TSC, MCx | sALS | 81 | Male | 13 | ALS w/ TDP-43 inclusions in motor cortex and lumbar spinal cord, PART |
| sALS patient 7 | TSC, MCx | sALS | 69 | Male | 14 | ALS w/TDP-43 inclusions in motor cortex |
| sALS patient 8 | TSC, MCx | sALS | 79 | Female | 19.5 | ALS |
| sALS patient 9 | TSC, MCx | sALS | 42 | Male | 22.5 | ALS w/TDP-43 inclusions |
| sALS patient 10 | TSC, MCx | sALS | 64 | Female | 26.6 | ALS w/TDP-43 inclusions in brain stem and spinal cord |
| C9ORF72 fALS patient 1 | CSC | C9 ALS | 51 | Male | 24 | ALS-TDP, DPR pathology |
| C9ORF72 fALS patient 2 | CSC | C9 ALS | 59 | Female | 12 | ALS-TDP, DPR pathology, tauopathy |
| C9ORF72 fALS patient 3 | CSC | C9 ALS | 51 | Male | 12 | ALS-TDP, DPR pathology |
| C9ORF72 fALS patient 4 | CSC | C9 ALS | 70 | Female | 24 | ALS-TDP, DPR pathology, FTLD-TDP |
| C9ORF72 fALS patient 5 | CSC | C9 ALS | 58 | Female | 24 | ALS-TDP, DPR pathology, FTLD-TDP |
CSC cervical spinal cord, TSC thoracic spinal cord, MCx motor cortex, CBD corticobasal degeneration, FTD frontotemporal dementia, AD/DLBD Alzheimer’s disease and dementia with Lewy body disease, ALS-SOD ALS with SOD1 mutation, ALS amyotrophic lateral sclerosis, sALS sporadic ALS, C9 ALS ALS with C9ORF72 mutation, ALS-TDP ALS with transactivation response DNA binding protein of 43 kD (TDP-43) inclusions, FTLD-TDP frontotemporal lobar degeneration with TDP-43 inclusions, CERAD Consortium to Establish a Registry for Alzheimer’s disease neuropathology protocol for dementia with Lewy bodies, LBD Lewy Body dementia, DPR dipeptide repeat pathology