Table 3. Core behavioural features of patients with C9orf72 expansions.
| Frontal behavior Inventory (FBI) (Kertesz, et al, 2000) |
Case 1 (bvFTD) After 1 y |
Case 2 (FTD/ALS) After 1 y |
Case 3 (FTD-PSP) |
Case 4 (AD) After 2 y |
|||
|---|---|---|---|---|---|---|---|
| MMSE | 20 | 15 | 26 | 24 | 25 | 23 | 14 |
| FBI-A | 18 | 25 | 15 | 21 | 21 | 5 | 18 |
| • Apathy | 3 | 3 | 3 | 3 | 2 | 1 | 2 |
| • Aspontaneity | 3 | 3 | 3 | 3 | 2 | 1 | 2 |
| • Indifference | 1 | 2 | 1 | 2 | 2 | 0 | 2 |
| • Inflexibility | 2 | 3 | 2 | 3 | 3 | 1 | 2 |
| • Personal neglect | 2 | 3 | 1 | 2 | 3 | 0 | 2 |
| • Disorganization | 3 | 3 | 2 | 3 | 3 | 1 | 3 |
| • Inattention | 1 | 2 | 2 | 2 | 3 | 1 | 2 |
| • Loss of insight | 2 | 3 | 1 | 2 | 2 | 0 | 2 |
| • Logopenia | 1 | 3 | 0 | 1 | 1 | 0 | 1 |
| • Semantic dementia | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
| • Aphasia | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
| • Apraxia | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
| FBI-B | 14 | 25 | 5 | 8 | 17 | 2 | 10 |
| • Perseveration | 2 | 2 | 3 | 3 | 3 | 1 | 1 |
| • Irritability | 1 | 3 | 0 | 1 | 0 | 1 | 2 |
| • Excessive jocularity | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
| • Impulsivity | 3 | 3 | 1 | 2 | 2 | 0 | 0 |
| • Hoarding | 1 | 3 | 0 | 0 | 0 | 0 | 0 |
| • Inappropriateness | 2 | 3 | 1 | 1 | 3 | 0 | 1 |
| • Roaming | 1 | 3 | 0 | 0 | 0 | 0 | 2 |
| • Aggression | 1 | 2 | 0 | 0 | 0 | 0 | 1 |
| • Hyperorality | 1 | 2 | 0 | 0 | 2 | 0 | 2 |
| • Hypersexuality | 0 | 0 | 0 | 0 | 2 | 0 | 0 |
| • Utilization behavior | 1 | 1 | 0 | 0 | 2 | 0 | 0 |
| • Incontinence | 1 | 3 | 0 | 1 | 3 | 0 | 1 |
| FBI–total | 32 | 50 | 20 | 29 | 38 | 7 | 28 |
bBvFTD–behavioural variant of frontotemporal dementia; PSP–progressive supranuclear paralysis; ALS–amyotrophic lateral sclerosis; AD–Alzheimer’s disease; MMSE–Mini Mental State Examination.