Table 2.
Clinical characteristics of bvFTD subgroups
| SN–FT (n = 21) |
SN–F (n = 27) |
SAN (n = 8) |
Subcortical (n = 30) |
|
|---|---|---|---|---|
| Age at imaging – yr | 62.8 (1.8) | 61.7 (1.6) | 58.3 (2.7) | 60.9 (1.4) |
| Disease duration – yr | 6.6 (1.2) | 5.8 (1.1) | 5.1 (1.9) | 6.1 (1.0) |
|
| ||||
| Global cognitive measures and GDS | ||||
| MMSEh | 19.9 (1.4)a | 21.2 (1.2) | 25.6 (2.0) | 25.3 (1.1) |
| FTLD-CDRSOBi | 11.5 (0.9)a | 10.5 (0.7) | 8.4 (1.5) | 8.0 (0.7) |
| CDRj | 1.5 (0.1)a | 1.5 (0.1)a | 1.3 (0.2) | 0.9 (0.1) |
| GDSk | 4.6 (1.6) | 9.6 (1.3) | 6.3 (2.2) | 8.5 (1.2) |
|
| ||||
| Neurological Exam – no. (%) | ||||
| Reduced facial expression | 7 (33.3) | 10 (37.0) | 3 (37.5) | 9 (30.3) |
| Rigidity | 10 (47.6) | 14 (51.8) | 4 (50.0) | 12 (40.0) |
| Action or postural tremor | 7 (33.3) | 11 (40.7) | 3 (37.5) | 9 (30.0) |
| Incoordination | 6 (28.6) | 17 (63.0) | 2 (25.0) | 15 (50.0) |
| Motor neuron related signs | 2 (9.5) | 5 (18.5) | 1 (10.0) | 2 (6.7) |
| Parkinsonism related signs | 6 (28.57) | 14 (51.9) | 3 (37.5) | 11 (36.7) |
| Abnormal gait | 0 (0.0) | 10 (37.0)b | 2 (25.0)b | 5 (16.7)b |
|
| ||||
| Socioemotional functioning | ||||
| Emotion namingl | 6.5 (0.8) | 6.8 (0.6) | 5.6 (1.2) | 8.2 (0.5) |
| Paralinguistic sarcasm detectionm | 11.7 (1.5) | 11.6 (1.2) | 5.4 (2.4) c | 14.3 (1.0) |
| Complex social cognitionn | 35.6 (3.0) | 35.8 (2.5) | 36.1 (4.4) | 43.9 (1.7) |
| Cognitive perspective takingo | 12.3 (1.1) | 9.4 (0.9) | 10.0 (1.7) | 11.4 (0.7) |
| Empathic perspective takingp | 11.8 (2.3) | 12.9 (2.1) | 21.6 (3.7) | 12.7 (2.0) |
| Empathic concernq | 18.8 (3.0) | 21.9 (2.6) | 28.0 (4.7) | 19.5 (2.5) |
| Interpersonal warmthr | 28.6 (4.0) | 30.0 (4.3) | 46.4 (8.8) | 31.0 (3.5) |
| Interpersonal assertivenessr | 36.9 (3.9) | 32.0 (4.2) | 41.6 (8.5) | 38.4 (3.2) |
|
| ||||
| Neuropsychological performance t | ||||
| Executive | ||||
| Error insensitivity | 0.3 (0.2) | 0.6 (0.2) | 0.6 (0.3) | 0.6 (0.2) |
| Modified Trails | 0.3 (0.1) | 0.2 (0.0) | 0.2 (0.1) | 0.3 (0.0) |
| Stroop color naming | 67.0 (6.1) | 54.5 (5.0) | 65.2 (8.4) | 52.9 (4.2) |
| Stroop inhibition | 37.1 (4.6) | 23.8 (3.9) | 41.2 (5.4) | 26.9 (3.1) |
| Digit span forward | 5.9 (0.4) | 5.5 (0.3) | 6.5 (0.6) | 5.2 (0.3) |
| Digit span backward | 4.1 (0.3) | 3.1 (0.2) d | 4.2 (0.4) | 3.4 (0.2) |
| Design fluency | 4.8 (0.9) | 4.0 (0.7) | 5.2 (1.2) | 5.0 (0.6) |
| D-words (lexical fluency) | 7.6 (0.9) | 4.3 (0.9) e | 8.3 (1.2) | 7.6 (0.7) |
| Animals (Category fluency) | 10.3 (1.4) | 9.4 (1.1) | 11.0 (1.9) | 11.3 (1.0) |
| Visuospatial | ||||
| Benson copy | 15.1 (0.6) | 13.8 (0.5) | 14.8 (0.9) | 13.7 (0.5) |
| Face recognition | 10.0 (0.6) | 9.7 (0.5) | 10.0 (1.0) | 10.5 (0.4) |
| Number location | 8.8 (0.7) | 7.4 (0.5) | 6.9 (0.8) | 7.8 (0.4) |
| Memory | ||||
| Benson delayed recall | 6.9 (0.9) | 7.9 (0.7) | 4.2 (1.3) a | 8.8 (0.7) |
| CVLT short delay recall | 3.4 (0.4) a | 4.3 (0.3) | 3.9 (0.6) | 4.9 (0.3) |
| CVLT delayed recall | 2.2 (0.5) a | 3.8 (0.4) | 2.3 (0.7) | 4.2 (0.4) |
| Affect matching | 7.5 (0.8) | 7.9 (0.7) | 8.3 (1.4) | 9.6 (0.6) |
| Language | ||||
| Repetition | 4.3 (0.3) | 4.3 (0.2) | 3.9 (0.4) | 3.6 (0.2) |
| Reading irregular words | 5.0 (0.3) | 5.8 (0.2) | 5.0 (0.3) | 5.6 (0.2) |
| Syntax comprehension | 4.8 (0.3) | 3.3 (0.2)f | 3.9 (0.4) | 3.6 (0.2)b |
| Verbal agility | 5.5 (0.3) | 5.2 (0.3) | 5.0 (0.4) | 5.0 (0.2) |
| Boston Naming Test | 10.6 (0.7) | 12.6 (0.5) | 9.2 (1.0) g | 12.5 (0.5) |
| CVLT total score | 18.6 (1.2) | 18.3 (1.0) | 18.2 (1.8) | 19.3 (1.0) |
| Calculations | 3.8 (0.3) | 3.1 (0.3) | 3.6 (0.4) | 3.4 (0.2) |
Abbreviations: bvFTD = behavioral variant frontotemporal dementia; CDR = Clinical Dementia Rating; CVLT = California Verbal Learning Test – short form; FTLD-CDRSOB = Frontotemporal Lobar Degeneration - modified CDR Sum of Boxes; GDS = Geriatric Depression Scale. MMSE = Mini Mental State Exam; SAN indicates semantic appraisal network–predominant subgroup; SN-FT, salience network–predominant frontal/temporal subgroup; SN-F, salience network–predominant frontal subgroup.
All values, except for the neurological exam findings, indicate the least-square-means and standard errors, derived from a general linear model comparing the four subgroups. Pairwise comparisons that were statistically significant after correcting for multiple comparisons between groups (Tukey- Kramer) are indicated in the footnote. Socioemotional and neuropsychological test sores that appear in bold text indicate values that are <1.5 standard deviations below compared to an age-matched control group. Socioemotional and neuropsychological test performances were controlled for MMSE and sex in this model. Motor neuron related signs referred to the presence of one or more of the following: reduced muscle power, muscle atrophy, and fasciculations. Parkinsonism related signs referred to the presence of one or more of the following: rest tremors, body bradykinesia, and positive retropulsion test. Details of patient evaluations including, neurological examination, neuropsychiatric and socioemotional testing are provided in detail in the eMethods.
P < 0.05 compared to subcortical.
P < 0.05 compared to SN-FT.
P < 0.01 compared to subcortical.
P < 0.05 compared to SN-FT and SAN.
P < 0.05 compared to SN-FT, SAN and Subcortical.
P < 0.01 compared to SN-FT.
P < 0.05 compared to SN-F and subcortical.
scores range from 0-30 with higher scores indicating better cognition.
scores range from 0-24 with higher scores indicating more advanced dementia.
scores range from 0-3 with higher scores indicating more advanced dementia.
scores range from 0-30 with higher scores indicating greater depression.
Emotion naming was evaluated using the TASIT-EET (The Awareness of Social Inference Test-Emotion Evaluation Test), with scores ranging from 0-14 and higher scores indicating better performance. 1
Paralinguistic elements of sarcasm was evaluated using TASIT-SIM (TASIT-Social Inference Minimal), with scores ranging from 0-20 and higher scores showing better performance.1
Complex social cognition was evaluated using TASIT SIE (The Awareness of Social Inference Test-Social Inference Enriched), with scores ranging from 0-64 and higher scores indicating better performance.1
Cognitive perspective taking was evaluated using the UCSF-Theory of Mind test (TOM), with scores ranging from 0-16 and higher scores indicating better performance.2
Empathic perspective taking was evaluated using the Perspective Taking (PT) subscale of the Interpersonal Reactivity Index (IRI), with scores ranging from 7-35 and higher scores indicating better performance.3
Empathic concern was evaluated using the Empathic Concern (EC) subscale of the IRI, with scores ranging from 7-35 and higher scores indicating better performance.3
Interpersonal warmth and interpersonal assertiveness were evaluated using the Interpersonal Adjective Scale (IAS) and scored using the IAS computer scoring program which generates T-scores (mean=50, SD 10) by comparing subject scores to a gender-matched, community-based normative sample data.4