| UCSF MACa |  | Snowden and colleagues[4] | Mahoney and colleagues[6] | Hsiung and colleagues[17] | Boeve and colleagues[7] | Chio and colleagues[20] | Simón-Sanchez and colleagues[23] | Byrne and colleagues[18] |
---|---|---|---|---|---|---|---|---|---|
Phenotype of carriersb | 14 bvFTD | Â | 19 bvFTD, 1 SD/FTD, 3 PNFA | 12 bvFTD, 1 PNFA | 15 bvFTD | 16 bvFTD | - | 28 bvFTD 7 PPA | - |
 |  | 11 FTD-MND | 9 FTD-MND | 3 FTD-MND | 7 FTD-MND | 3 FTD-MND | 30 FTD-MND | 6 FTD-MND, 1 PPA-MND | 10 FTD-MND |
 |  |  |  |  | 8 ALS | 1 ALS | 36 ALS | - | 11 ALS |
Controls (noncarriers)c | 45 bvFTD | 19 FTD-MND | 366 noncarriers | NA | NA | NA | NAd | NA | 166 ALS (± FTD) |
Number screened | 648 | Â | 398 | 234 | 22 | 604 | 182 | 363 | 435 |
Assessment method | RCR and NPI | Â | Structured pro-forma | RCR | RCR | RCR | FSBS and RCRe | RCR | FSBS |
Disinhibition | 85% | 63% | 66% | 63 → 76% | 40 → 70% | NA | 13%e | 26% | NA |
Apathy/inertia | 64% | 36% | 88% | 49 → 63% | 30 → 47% | NA | 100%e | 43% | NA |
Loss of sympathy/empathy | 57% | 45% | 77% | ~12 → ~18% | NA | NA | NA | NA | NA |
Perseverative/compulsive behavior | 50% repet., 50% comp. | 18% repet., 54% comp. | 75% repet./stereotypic | ~25 → 50% obsessive | 27 → 50% rigidity/persev. | NA | 1.5% acute OCD | 12% OCD | NA |
Hyperorality | 50% | 27% | 43% | NA | NA | 100% | NA | NA | NA |
Anxiety | NA | NA | NA | ~39 → 52% | NA | NA | NA | NA | NA |
Delusions | 42% | 36% | 50% | 0 → ~12% | 3% | 45% | 4%f | 0 | NA |
Hallucinations | 7% | 9% | 19% | ~6 → ~12% | 0 | 50% | 4%f | 5% | NA |
Lack of insight | NA | NA | 78% | NA | NA | NA | NA | NA | NA |