Demographics and the proportion of cases in each group with the main clinical features of the new FTD clinical phenotypes
| bvFTD (N=66) | sv-PPA (N=31) | nfv-PPA (N=16) | lv-PPA (N=22) | p Value | |
|---|---|---|---|---|---|
| % male/% female | 65/35 | 56/43 | 65/35 | 86/14 | 0.14 |
| Age at onset (years) | 57 (10) | 61 (7) | 65 (8) | 62 (9) | 0.004 |
| Age at diagnosis (years) | 61 (9) | 65 (7) | 70 (8) | 66 (8) | 0.002 |
| Age at death (years) | 66 (10) | 73 (7) | 75 (7) | 71 (8) | <0.0001 |
| Duration from onset (years) | 9 (5) | 12 (4) | 10 (3) | 9 (3) | 0.012 |
| Duration from diagnosis (years) | 5 (3) | 8 (3) | 6 (2) | 5 (4) | 0.001 |
| Behavioural features | |||||
| Disinhibition | 98 | 59 | 18 | 55 | – |
| Apathy | 65 | 47 | 41 | 45 | – |
| Loss of empathy | 33 | 28 | 18 | 14 | – |
| Stereotyped behaviours | 95 | 72 | 24 | 28 | * |
| Alt. food pref. | 58 | 50 | 41 | 23 | – |
| Executive deficits | 59 | 25 | 6 | 0 | * |
| Language features | |||||
| Word finding | 45 | 84 | 71 | 100 | * |
| Apraxia of speech | 5 | 6 | 94 | 32 | * |
| Agrammatism | 0 | 0 | 53 | 14 | * |
| Naming | 30 | 97 | 71 | 86 | – |
| Single word comp | 5 | 84 | 18 | 18 | * |
| Object agnosia | 2 | 34 | 0 | 5 | – |
| Phonologic errors | 2 | 9 | 71 | 73 | * |
| Word rep | 0 | 0 | 53 | 64 | * |
| Sentence rep | 2 | 6 | 35 | 68 | * |
| Sentence Comprehension | 23 | 72 | 88 | 68 | – |
| Surface dyslexia | 11 | 81 | 71 | 64 | * |
| Motor features | |||||
| Parkinsonism | 18 | 3 | 6 | 9 | – |
| Apraxia | 23 | 0 | 24 | 36 | – |
| Gait disturbance | 12 | 3 | 12 | 9 | – |
| Orobuccal apraxia | 8 | 3 | 24 | 0 | * |
| Neuropsychiatric features | |||||
| Delusions | 14 | 9 | 6 | 9 | – |
| Hallucinations | 20 | 6 | 6 | 14 | – |
| Other features | |||||
| Prosopagnosia | 3 | 47 | 0 | 5 | * |
| Rigidity of thought | 41 | 38 | 12 | 18 | – |
| Irritability | 33 | 50 | 47 | 45 | – |
| Aggression | 30 | 28 | 12 | 18 | – |
*Features with high (>80%) and low (<20%) prevalences indicated and the patterns of features discriminating each clinical subtype statistically identified in bold (Wilks’ lambda=0.024, p<0.0001).
bvFTD, behavioural variant frontotemporal dementia; lv-PPA, logopenic variant-primary progressive aphasias; nfv-PPA, non-fluent/agrammatic variant-PPA; sv-PPA, semantic variant-PPA.