Table 1.
Clinical symptoms in aFTLD-U cases
| ID | Sex | Diagnosis | Behavioural/personality | Psychiatric | Cognitive | Motor | ||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Apathy | Disinh | Abnormal eating | Compulsions | Inappropriate sexual | Delusions | Hallucinations | Aphasia | Parkinsonism | MND | |||
| UBC1 | F | bvFTD | 1 | 1 | 1 | 1 | 0 | 0 | 0 | 0 | 0 | 0 |
| UBC2 | F | bvFTD | 1 | 0 | 0 | 0 | 1 | 1 | 0 | 0 | 0 | 0 |
| UBC3 | F | bvFTD | 1 | 1 | 1 | 0 | 1 | 0 | 0 | 0 | 0 | 0 |
| UBC4 | F | bvFTD | 1 | 1 | 1 | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
| UBC5 | M | bvFTD | 1 | 1 | 1 | 0 | 1 | 1 | 0 | 0 | 0 | 0 |
| UBC6 | F | bvFTD | 1 | 1 | 0 | 0 | 0 | 0 | 1 | 0 | 0 | 0 |
| MUC1 | M | Dem | NA | NA | NA | NA | NA | NA | NA | NA | NA | NA |
| MUC2 | F | bvFTD | 1 | 1 | 0 | 0 | NA | NA | NA | 1 | 0 | 0 |
| MUC3 | F | Dem | NA | 1 | NA | NA | NA | NA | NA | NA | NA | NA |
| MUC4 | M | bvFTD | 1 | 1 | 1 | 1 | NA | NA | NA | 1 | 0 | 0 |
| MUC5 | F | bvFTD | 0 | 1 | 1 | 0 | NA | NA | NA | 1 | 0 | 0 |
| MUC6 | M | bvFTD | 1 | 1 | NA | NA | NA | NA | NA | NA | NA | NA |
| MUC7 | F | bvFTD | 1 | 1 | 1 | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
| MUC8 | M | bvFTD | NA | 1 | NA | NA | NA | NA | NA | NA | NA | NA |
| MUC9 | M | bvFTD | 0 | 1 | 1 | 1 | NA | NA | NA | 0 | 0 | 0a |
| UR1 | M | bvFTD | 1 | 1 | 1 | 1 | 1 | 0 | 1 | 0 | 0 | 0 |
| UR2 | F | bvFTD | 1 | 1 | 1 | 0 | 1 | 0 | 0 | 0 | 0 | 0 |
| UR3 | F | bvFTD | 1 | NA | 1 | 1 | 0 | 1 | 0 | 0 | 0 | 0 |
| UR4 | F | bvFTD | 1 | 1 | 0 | 1 | 0 | 0 | 0 | 0 | 0 | 0 |
| UT1 | M | bvFTD | NA | 1 | NA | NA | NA | 1 | 0 | 0 | 0 | 0 |
| UTSW1 | M | bvFTD | 1 | 1 | 1 | 1 | 1 | 0 | 0 | 1 | 0 | 0 |
| NWU1 | M | bvFTD | 0 | 1 | 1 | 1 | 1 | 0 | 0 | 0 | 0 | 0 |
| NWU2 | F | bvFTD | 1 | 1 | 1 | 1 | 1 | 0 | 0 | 1 | 0 | 0 |
| UP1 | F | bvFTD | 1 | 1 | 1 | 1 | 0 | NA | NA | 0 | 0 | 0 |
| UP2 | M | bvFTD | 1 | 0 | 1 | 1 | 0 | NA | NA | 0 | 0 | 0 |
| UP3 | M | bvFTD | 1 | 1 | NA | 1 | 0 | NA | NA | 0 | 0 | 0 |
| UNSW1 | F | bvFTD | NA | 1 | NA | 1 | NA | 0 | 0 | 1 | 1 | 0 |
| UNSW2 | M | bvFTD | NA | 1 | 1 | 0 | 0 | 0 | 0 | 1 | 0 | 0 |
| MC1 | M | bvFTD | 0 | 1 | 1 | 1 | 1 | 0 | 1 | 0 | 0 | 0 |
| MC2 | F | bvFTD | 0 | 1 | NA | 1 | 1 | NA | NA | 0 | 0 | 0 |
| MC3 | M | bvFTD | 0 | 1 | 1 | 1 | 1 | 0 | 0 | 0 | 0 | 0 |
| MC4 | M | bvFTD | 1 | 0 | 0 | 1 | 0 | 0 | 0 | 0 | 0 | 0 |
| MC5 | M | bvFTD | 0 | 1 | 1 | 0 | 1 | 0 | 0 | 0 | 0 | 0 |
| UCL1 | M | bvFTD | NA | 1 | 1 | NA | NA | 0 | 1 | NA | 0 | 0 |
| % with symptom | 74 | 91 | 81 | 61 | 52 | 18 | 18 | 24 | 3 | 0 | ||
Cases of aFTLD-U were scored for presence of the symptoms indicated
0 absent, 1 present, NA information not available, bvFTD behavioural variant FTD, Dem unspecified dementia, Disinh disinhibition
aOne patient exhibited tongue fasciculation, dysphagia and muscle hypotonia but there was insufficient evidence to diagnose MND