Table 1.
Clinical features
| Case | AAO (years) | Survival (years) | Initial presentation | ALS | Parkinsonism | Cognitive impairment | Executive dysfunction | Psychosis |
|---|---|---|---|---|---|---|---|---|
| 1 | 43 | 1.5 | Unknown | + | + | – | – | – |
| 2 | 31 | 12.6 | Dysphagia, dysarthria and quadraparesis | ++ | + | † | † | – |
| 3 | 52 | 1.9 | Frontal syndrome and dysarthria | ++ | – | ++ | ++ | – |
| 4 | 43 | 0.9 | Lower limb paresis | ++ | – | – | – | – |
| 5 | 38 | 8.3 | Frontal syndrome and Parkinsonism | † † | ++ | † | ++ | – |
| 6 | 44 | 3.3 | Upper limb paresis | ++ | – | † | † | – |
| 7 | 45 | 0.5 | Frontal syndrome with psychosis | † | – | ++ | ++ | + |
| 8 | 46 | N/A | Frontal syndrome with psychosis and Parkinsonism | † † | + | + | ++ | + |
| 9 | 40 | 1.0 | Dysarthria | ++ | – | + | – | – |
AAO age at onset, ++: main presenting feature, +: minor presenting feature, ††: late presenting features, †: minor late feature, –: absent or information not available