Table 1.
Baseline demographics, neuropsychology and VSTM performance by participant group for N = 99.
| Controls (N = 67) | Early PMCs (N = 12) | Late PMCs (N = 11) | Symptomatic carriers (N = 9) | |
|---|---|---|---|---|
| Demographics | ||||
| Sex: N (%) Male | 34 (50.7) | 3 (25.0) | 7 (63.6) | 6 (66.7) |
| Age (yrs) | 39.4 (8.1) | 34.8 (6.4) | 37.0 (5.0) | 48.1 (9.8)* |
| EYO (yrs) | NA | −12.9 (4.7) | −5.8 (1.8) | 3.0 (4.1) |
| AYO (yrs) | NA | NA | NA | 3.1 (4.0) |
| Education (yrs) | 15.4 (2.7) | 14.3 (2.5) | 13.3 (2.5)* | 13.9 (2.9) |
| MMSE | 29.5 (0.8) | 29.3 (0.9) | 29.5 (0.8) | 25.1 (3.7)** |
| CDR global | 0.0 (0.0) | 0.0 (0.0) | 0.0 (0.0) | 0.6 (0.2)** |
| Anxiety | 6.1 (3.8) | 7.9 (4.6) | 3.9 (3.9)* | 7.0 (4.5) |
| Depression | 3.2 (2.8) | 2.9 (4.0) | 1.3 (1.6)* | 2.4 (2.1) |
| Neuropsychology tests | ||||
| Performance IQ | 110.5 (16.3) | 106.0 (15.7) | 101.4 (10.1) | 100.4 (12.1) |
| Verbal IQ | 109.9 (14.9) | 96.1 (15.1)* | 95.4 (13.5)** | 99.4 (18.8) |
| Arithmetic total/24 | 16.7 (6.8) | 13.9 (5.0) | 14.3 (4.6) | 10.3 (5.8)** |
| RMT faces | 41.1 (7.2) | 41.0 (4.2) | 43.8 (4.5) | 40.3 (3.7) |
| RMT words | 47.0 (5.0) | 48.7 (2.2) | 46.5 (2.8) | 35.3 (10.0)** |
| Digit span forwards/8 | 7.1 (1.2) | 6.8 (1.0) | 7.4 (1.1) | 6.0 (1.5)* |
| Digit span backwards/7 | 5.2 (1.2) | 5.7 (1.3) | 5.4 (1.1) | 4.3 (1.6) |
| BPVS | 142.5 (8.8) | 135.0 (14.4)** | 139.8 (10.1) | 135.9 (11.8) |
| GNT/30 | 20.9 (4.6) | 17.8 (5.8) | 19.2 (5.4) | 18.8 (7.2) |
| NART/50 | 31.8 (8.9) | 24.1 (8.6)** | 27.7 (10.7) | 25.4 (13.2) |
| VOSP OD/20 | 18.0 (2.8) | 17.8 (1.8) | 18.3 (1.3) | 17.6 (1.5) |
| Stroop (s) | 50.3 (14.0) | 45.8 (12.2) | 52.6 (14.1) | 78.2 (22.4)** |
| VSTM performance | ||||
| Identification (% correct) | ||||
| Overall | 91.6 (4.8) | 90.2 (6.3) | 92.0 (3.9) | 81.9 (5.0)** |
| Localisation error (deg) | ||||
| Overall | 4.4 (1.3) | 4.5 (1.3) | 4.6 (1.1) | 7.8 (1.8)** |
| Swap error (%) | ||||
| Overall | 10.6 (5.3) | 11.7 (4.7) | 10.2 (5.9) | 22.6 (8.1)** |
| Block 1, 1s delay | 12.0 (8.4) | 12.4 (9.2) | 9.9 (5.0) | 21.2 (12.6) |
| Block 1, 4s delay | 13.2 (8.7) | 18.7 (9.2) | 15.0 (10.8) | 23.2 (18.0)* |
Unadjusted mean values are given with SD unless otherwise stated. SD = standard deviation; NA = not applicable; PMC = presymptomatic mutation carrier; EYO = estimated years to/from symptom onset (a negative value indicates a younger age than their estimated age at symptom onset); AYO = actual years to/from onset (positive values indicate years post onset); Anxiety and depression scores from HADS = hospital anxiety and depression scale; IQ = intelligence quotient; MMSE = mini mental state examination; CDR = clinical dementia rating scale; RMT = recognition memory test; GNT = graded naming test; VOSP OD = object decision from the visual object and space perception battery. Digit spans forwards and backwards are taken from the WMS-R = Wechsler Memory Scale. Neuropsychology data were available at baseline for: 64 participants for performance IQ, verbal IQ; 98 for arithmetic total, GNT, NART, VOSP; 99 for RMT faces, RMT words, digit span forwards, digit span backwards; 71 for BPVS; and 78 for Stroop (s). Bold = significant; *: the difference between the patient group and controls for that variable was significant at p < 0.05; **: the difference between the patient group and controls for that variable was significant at p < 0.01.