Table 4.
Motor features of study subjects
| Subject | Final diagnosis | Pattern of ALS features |
Extra pyramidal | Other | |||
|---|---|---|---|---|---|---|---|
| UMN | LMN | Distal/Proximal | Bulbar | ||||
| A-1 | FTD–ALS | 1 | 2 | Distal | 0 | 2 | Unsteady gait |
| A-2 | ALS | 2 | 2 | Distal | 0 | 0 | ↓ Position/vibration sense |
| A-3 | ALS | 1 | 2 | Distal | 3 | 0 | |
| B-1 | FTD | 0 | 0 | 0 | 1 | 1 | |
| B-2 | FTD | NA | NA | NA | NA | 1 | Incontinence |
| C-1 | FTD–ALS | 1 | 1 | Distal/prox | 2 | 1 | |
| C-2 | FTD | 0 | 1 | NA | NA | 0 | Incontinence |
| C-3 | FTD–ALS | 1 | 2 | Distal | 2 | 1 | |
| C-4 | ALS | 2 | 2 | Distal/prox | 1 | 0 | |
| D-1 | FTD–ALS | 2 | 2 | Distal | 1 | 1 | |
| D-2 | ALS | 2 | 1 | Distal | 2 | 0 | |
| E-1 | FTD–ALS | 2 | 2 | Distal | 1 | 2 | Gaze palsy |
| F-1 | FTD | 0 | 0 | 0 | 0 | 0 | |
| G-1 | FTD | NA | NA | NA | NA | NA | |
| G-2 | FTD | 0 | 0 | 0 | 0 | 0 | |
| H-1 | ALS | 2 | 2 | Distal | 1 | 0 | |
| H-2 | FTD | NA | NA | NA | NA | NA | |
| H-3 | ALS | NA | NA | NA | NA | NA | |
| I-1 | FTD–ALS | 2 | 1 | Distal | 2 | 0 | |
| I-2 | ALS | 2 | 1 | NA | 2 | 0 | |
| J-1 | FTD | NA | NA | NA | NA | NA | |
| K-1 | FTD–ALS | 2 | 2 | Distal | 2 | 0 | |
| K-2 | ALS | NA | NA | NA | NA | NA | |
| L-1 | FTD | 1 | 0 | 0 | 1 | 1 | ↓ Balance |
| M-1 | FTD + park | 2 | 0 | Distal/prox | 1 | 2 | ↓ Balance, dystonia, ataxia, incontinence |
| M-2 | FTD | NA | NA | NA | NA | 1 | Incontinence |
| M-3 | FTD | 0 | 0 | 0 | 0 | 1 | ↓ Balance |
| N-1 | FTD | NA | NA | NA | NA | 1 | Unsteady gait |
| O-1 | FTD | 0 | 0 | 0 | 0 | 0 | |
| P-1 | FTD | 0 | 0 | 0 | 0 | 0 | Gaze palsy |
Semiquantitative grading: 0, absent; 1, mild; 2, moderate; 3, severe.
NA = not available; bvFTD = behavioural variant frontotemporal dementia; park = parkinsonism; LMN = lower motor neuron; prox = proximal; UMN = upper motor neuron; ↓ = reduced.