Table 1 Main demographic, clinical, imaging, and biological data at baseline for the patient and control groups (mean (SD)).
Whole ASHT group n = 36 | SNAP n = 15 | AD n = 21 | Controls n = 30 | ||||
Demographic data | Age (years) | 72.9 (6.8) | 77.5* (5.5) | 69.5 (5.6) | 68.8 (5.6) | ||
Sex (F/M) | 16/20 | 6/9 | 10/11 | 20/10 | |||
Education (years) | 14.3 (4.2) | 13.6 (4.7) | 14.7 (3.9) | 12.7 (4.1) | |||
Disease duration (years) | 4.8 (3.4) | 4.2 (2.7) | 5 .2 (3.8) | – | |||
Functional status | CDR | 0 | 0 | 0 | 0 | 30 | |
0.5 | 26 | 9 | 17 | 0 | |||
1 | 10 | 6 | 4 | 0 | |||
Neuropsychological assessment | |||||||
Global cognitive efficiency | MMSE | 24.5 (2.8) | 24.1 (2.6) | 24.8 (2.9) | 29.2 (0.9)* | ||
Short term/working memory | Digit spans | 9.7 (1.8) | 9.9 (2.2) | 9.5 (1.6) | 10 (1.3) | ||
Episodic memory | verbal | FCSRT (Free + total recall) | 33.3 (17) | 36 (16.2) | 31.4 (17.7) | 80.2 (4.5)* | |
Free recall (/48) | 9.1 (6.3) | 9 (5.6) | 9.2 (6.8) | 32.7 (4)* | |||
Total recall (/48) | 24.2 (11.4) | 27 (11.2) | 22.1 (11.4) | 47.4 (0.9)* | |||
Sensitivity index (%) | 41.1 (21.7) | 48.4 (22.2) | 35.9 (20.2) | 96.7 (5.1)* | |||
visual | Rey memory (/36) | 7.25 (5.1) | 7.3 (5.4) | 7.2 (5.1) | 18.7 (5.5)* | ||
Instrumental functions | Naming (/80) | 77.7 (2.8) | 78.5 (1.6) | 77.1 (3.4)# | 79.8 (0.5) | ||
Rey copy (/36) | 34.4 (2.2) | 34.7 (2.6) | 34.1 (1.9) | 35 (1.7) | |||
Praxis (/72) | 69.1 (2.9) | 69.6 (1.8) | 68.7 (3.4) | 71 (1.3) | |||
Executive functions | TMT A (seconds) | 53.7 (16.6) | 54.5 (17.1) | 53.1 (16.6) | 39.8 (14.1) | ||
TMT B-A (seconds) | 104.9 (63.3) | 106.5 (60.6) | 103.7 (66.6) | 37.9 (23.6)* | |||
Letter Fluency (2 min) | 18.2 (5.8) | 16.1 (5.1)# | 19.7 (5.9) | 24.6 (7.1) | |||
Category Fluency (2 min) | 20.4 (6.4) | 20.9 (7.8) | 20 (5.4) | 35.5 (9.6)* | |||
Similarities (WAIS) | 19.6 (4.4) | 20.7 (3.9) | 18.9 (4.7)# | 22.3 (3.4) | |||
Social cognition | Emotion recognition (/35) | 28.5 (3.4) | 27.8 (3.3) | 29 (3.5) | 29.6 (2.3) | ||
Negative emotions (fear, sadness, anger, disgust) (/20) | 14.8 (2.8) | 14.3 (2.9) | 15.1 (2.7) | 15.6 (2) | |||
Faux pas test (/40) | 30.4 (5.2) | 30.9 (4.7) | 30 (5.7) | 35.1 (2.9)* | |||
Behavioral changes | CBI-R (/180) | 38 (19.4) | 42.1 (19.9) | 35.4 (19.1) | – | ||
Genetic status | ApoE genotype (n with at least one E4 allele) | 18 | 2 | 16 | 2 | ||
Progranulin plasma level (μg/l) | 108.5 (26) | 114.6 (20.2) | 104.1 (29.1) | 127.3 (25.5) | |||
c9orf72 mutation | None | None | None | – | |||
Molecular PET imaging | PiB PET GCI | 2.28 (0.9) | 1.35 (0.2) | 2.93 (0.6)* | 1.25 (0.1) | ||
n with GCI > 1.45 | 24 | 3 | 21 | 0 | |||
Tau PET GCI | 1.54 (0.65) | 1.15 (0.1) | 1.81 (0.7) | 1.23 (0.11) | |||
MRI | Fazekas score (0/1/2/3) | 20/12/3/1 | 7/5/2/1 | 13/7/1/0 | 20/8/2/0 | ||
Left HV | 1.78 (0.3) | 1.64 (0.27) | 1.88 (0.29) | 2.48 (0.2)* | |||
Right HV | 1.85 (0.34) | 1.68 (0.3) | 1.97 (0.32) | 2.51 (0.23)* | |||
Left EC | 0.92 (0.32) | 0.82 (0.26) | 0.99 (0.34) | 1.36 (0.23)* | |||
Right EC | 0.93 (0.27) | 0.91 (0.3) | 0.94 (0.26)# | 1.25 (0.23) | |||
Left Amygdala | 0.66 (0.14) | 0.64 (0.15) | 0.67 (0.13) | 0.93 (0.13)* | |||
Right Amygdala | 0.8 (0.16) | 0.79 (0.18) | 0.82 (0.14) | 1.13 (0.15)* | |||
Left Insula | 3.88 (0.42) | 3.76 (0.45) | 3.97 (0.38) | 4.4 (0.47)* | |||
Right Insula | 3.84 (0.5) | 3.74 (0.64) | 3.92 (0.36) | 4.3 (0.46)* | |||