Table 1.
Baseline demographic and clinical features for the PAOS-CBD and PAOS-PSP cases.
| Baseline data | Annualized rates of change | ||||||
|---|---|---|---|---|---|---|---|
| PAOS-CBD (N = 17) |
PAOS-PSP (N = 10) |
p value | AUROC (95% CI) |
PAOS-CBD (N = 12) |
PAOS-PSP (N = 9) |
p value | |
| Demographics | |||||||
| Female sex, N % | 8 (47%) | 4 (40%) | >0.999 | 0.54 (0.32/0.73) | NA | NA | NA |
| Education, years | 16 (12, 16) | 16 (15, 20) | 0.165 | 0.66 (0.43/0.83) | NA | NA | NA |
| Handedness (L/R/A), N | 2/15/0 | 2/7/1 | 0.385 | 0.59 (0.37/0.77) | NA | NA | NA |
| Age onset, years | 60 (51, 64) | 72 (65, 74) | 0.003 | 0.85 (0.63/0.95) | NA | NA | NA |
| Age death, years | 69 (63, 73) | 83 (75, 84) | 0.001 | 0.88 (0.67/0.96) | NA | NA | NA |
| Duration (Onset to death), years | 9 (7, 10) | 11 (10, 13) | 0.021 | 0.77 (0.54/0.90) | NA | NA | NA |
| Age at baseline, years | 64 (60, 69) | 75 (71, 78) | 0.002 | 0.87 (0.65/0.96) | NA | NA | NA |
| Onset to baseline, years | 5 (3, 6) | 5 (3, 6) | 0.960 | 0.51 (0.30/0.71) | NA | NA | NA |
| Baseline to last evaluation, years | NA | NA | NA | NA | 2.7 (1.8, 3.5) | 5.3 (3.5, 5.7) | 0.009 |
| Number of serial visits | NA | NA | NA | NA | 2 (1, 4) | 5 (3, 6) | NA |
| Number of serial MRI | NA | NA | NA | NA | 2 (1, 4) | 4 (3, 5) | NA |
| Number of serial PET | NA | NA | NA | NA | 2 (1, 3) | 4 (2, 5) | NA |
| Genetic data | |||||||
| Family history in 1st or 2nd degree relative, N % | 1 (6%) | 2 (20%) | 0.535 | 0.57 (0.35/0/76) | NA | NA | NA |
| TMEM106B CC/CG/GG | 6/10/1 | 2/7/1 | 0.836 | 0.58 (0.36/0.77) | NA | NA | NA |
| GRN/MAPT/C9ORF72, N | 0/0/0 | 0/0/0 | NA | NA | NA | NA | NA |
| APOE ε4, N % | 4 (24%) | 1 (10%) | 0.621 | 0.57 (0.35/0/76) | NA | NA | NA |
| H1H1 haplotype, N % | 15 (88%) | 4 (40%) | 0.024 | 0.74 (0.51/0.88) | NA | NA | NA |
| Speech and language data | |||||||
| Phonetic/prosodic AOS type, N | 15/2 | 3/7 | 0.004 | 0.79 (0.56/0.91) | NA | NA | NA |
| Aphasia present, N % | 9 (53%) | 4 (40%) | 0.695 | 0.56 (0.35/0.76) | NA | NA | NA |
| Aphasia severity/4 | 0 (0, 1) | 0 (0, 1) | 0.851 | 0.52 (0.31/0.72) | 0.6 (0.4, 0.6) | 0.3 (0.2, 0.4) | 0.006 |
| WAB Aphasia Quotient, /100 | 95 (91, 96) | 97 (87, 100) | 0.225 | 0.64 (0.42/0.82) | −6.7 (−13.4, −5.5) | −1.4 (−2.9, −1.1) | <0.001 |
| ASRS Total Score V3 | 19 (14, 26) | 16 (11, 22) | 0.526 | 0.57 (0.35/0.77) | 4.5 (2.0, 4.9) | 4.2 (3.0, 4.4) | 0.691 |
| ASRS Phonetic subscore | 6 (5, 11) | 4 (3, 4) | 0.013 | 0.79 (0.56/0.91) | 1.5 (0.0, 2.5) | 1.4 (0.9, 1.7) | 0.965 |
| ASRS Prosodic subscore | 6 (4, 8) | 8 (3, 10) | 0.916 | 0.51 (0.30/0.72) | 1.1 (0.9, 1.2) | 0.8 (0.6, 2.2) | 0.895 |
| MSD Severity Scale, /10 | 6 (5, 7) | 7 (6, 8) | 0.085 | 0.70 (0.47/0.85) | −1.2 (−1.5, −1.0) | −0.6 (−0.9, −0.5) | 0.004 |
| BNT, /15 | 13 (12, 14) | 15 (14, 15) | 0.010 | 0.80 (0.57/0.92) | −1.2 (−2.3, -0.9) | −0.3 (−0.7, −0.1) | 0.027 |
| WAB fluency, /10 | 9 (8, 10) | 9 (9, 10) | 0.779 | 0.53 (0.32/0.73) | −1.5 (−2.0, −1.0) | −0.5 (−0.9, −0.1) | 0.025 |
| Letter fluency (FAS) | 17 (10, 25) | 18 (18, 27) | 0.413 | 0.60 (0.37/0.78) | −4.2 (−4.9, −0.6) | −2.9 (−5.0, −1.6) | 0.958 |
| WAB animal fluency | 14 (11, 15) | 16 (11, 20) | 0.278 | 0.63 (0.40/0.81) | −3.0 (−4.0, −2.5) | −2.4 (−2.8, −1.6) | 0.070 |
| Token Test, /22 | 20 (12, 21) | 20 (19, 22) | 0.129 | 0.69 (0.44/0.86) | −1.7 (−3.0, −0.7) | −1.7 (−3.0, −0.3) | 0.501 |
| NAT, /10 | 8 (6, 10) | 9 (9, 10) | 0.413 | 0.61 (0.35/0.82) | −0.8 (−2.8, −0.0) | −0.5 (−1.0, −0.1) | 0.700 |
| PPT word-word, /52 | 50 (48, 51) | 50 (49, 51) | 0.268 | 0.63 (0.40/0.81) | −0.8 (−1.7, −0.1) | −0.1 (−0.6, 0.0) | 0.138 |
| Dysarthria present, N % | 4 (24%) | 2 (20%) | >0.999 | 0.52 (0.31/0.72) | NA | NA | NA |
| Dysarthria severity, /4 | 0 (0, 0) | 0 (0, 0) | 0.974 | 0.50 (0.30/0.71) | 0.4 (0.2, 0.7) | 0.3 (0.2, 0.4) | 0.676 |
| NVOA, /32, 32 = normal | 22 (15, 30) | 29 (26, 32) | 0.277 | 0.63 (0.40/0.81) | −4.4 (−6.5, −3.5) | −5.6 (−6.5, −5.3) | 0.522 |
| Neurological/neuropsychological data | |||||||
| MMSE, /30 | 29 (29, 29) | 30 (28, 30) | 0.345 | 0.61 (0.38/0.79) | −2.9 (−4.8, −1.8) | −0.6 (−0.8, −0.3) | 0.005 |
| MoCA, /30 | 25 (24, 26) | 28 (25, 28) | 0.287 | 0.62 (0.40/0.80) | −3.2 (−5.6, −2.6) | −1.7 (−2.0, −1.2) | 0.011 |
| FAB, /18 | 15 (14, 17) | 16 (14, 17) | 0.503 | 0.58 (0.36/0.77) | −2.3 (−4.3, −1.7) | −1.9 (−2.7, −0.9) | 0.394 |
| FBI, /72, 0 = best | 12 (5, 18) | 4 (2, 5) | 0.001 | 0.88 (0.66/0.96) | 5.9 (3.0, 8.9) | 3.7 (1.8, 5.3) | 0.283 |
| NPI-Q, /36, 0 = best | 2 (1, 6) | 2 (0, 3) | 0.259 | 0.63 (0.40/0.81) | 1.2 (0.3, 4.6) | 0.5 (0.3, 0.8) | 0.186 |
| MDS-UPDRS III, /132, 0 = best | 14 (5, 23) | 14 (8, 22) | >0.999 | 0.50 (0.29/0.71) | 12.2 (7.7, 18.4) | 12.3 (8.0, 15.4) | 0.943 |
| WAB Praxis, /60 | 56 (53, 58) | 58 (52, 59) | 0.730 | 0.54 (0.33/0.74) | −7.0 (−10.7, −5.4) | −5.4 (−6.3, −1.5) | 0.177 |
| PSIS, /5, 0=best | 0 (0, 1) | 1 (0, 1) | 0.154 | 0.65 (0.42/0.82) | 0.4 (0.2, 0.5) | 0.3 (0.2, 0.5) | >0.999 |
| TMT A MOANS | 6 (5, 9) | 8 (6, 10) | 0.380 | 0.60 (0.38/0.79) | −0.6 (−2.6, 0.0) | −1.8 (−1.9, −0.4) | 0.817 |
| TMT B MOANS | 7 (5, 8) | 10 (8, 10) | 0.111 | 0.70 (0.45/0.87) | −1.5 (−2.0, −0.8) | −1.3 (−2.1, −1.0) | 0.668 |
| Sorting Test SS | 9 (8, 12) | 12 (11, 16) | 0.042 | 0.75 (0.51/0.89) | −0.3 (−0.8, −0.0) | −0.7 (−1.3, 0.2) | 0.699 |
| WMS-III VR I SS | 8 (6, 11) | 11 (9, 14) | 0.093 | 0.72 (0.46/0.88) | 0.0 (−0.4, 0.4) | 0.3 (−0.4, 0.5) | 0.643 |
| WMS-III VR II SS | 12 (9, 14) | 14 (12, 15) | 0.149 | 0.69 (0.43/0.86) | −1.7 (−3.9, −1.4) | 0.2 (−0.5, 0.7) | 0.021 |
| VOSP letters, /20 | 20 (19, 20) | 20 (20, 20) | 0.162 | 0.64 (0.42/0.82) | 0.0 (−0.0, 0.3) | −0.0 (−0.2, 0.0) | 0.282 |
| VOSP cubes, /10 | 10 (9, 10) | 9 (8, 10) | 0.349 | 0.60 (0.38/0.79) | 0.0 (−1.9, 0.0) | −0.1 (−0.5, 0.0) | 0.690 |
Data shown as median (inter-quartile range) or N (%). Annualized rates of change are calculated as average change overall visits within participant.
MOANS and SS have a mean of 10 with a standard deviation of 3 in normal healthy individuals.
L left, R right, A ambidextrous, APOE apolipoprotein, GRN progranulin, MAPT microtubule associated protein tau, MMSE Mini Mental State Examination, MoCA Montreal Cognitive Assessment, FAB Frontal Assessment Battery, FBI Frontal Behavioral Inventory, NPI-Q short questionnaire version of the Neuropsychiatric Inventory, MDS-UPDRS III Movement Disorder Society sponsored revision of the Unified Parkinson’s Disease Rating Scale part III, WAB Western Aphasia Battery, PSIS Progressive supranuclear palsy Saccadic Impairment Scale, AOS apraxia of speech, ASRS Apraxia of Speech Rating Scale, MSD Motor Speech Disorder, BNT Boston Naming Test, NAT Northwestern Anagram Test, PPT Pyramids and Palm Trees test, NVOA nonverbal oral apraxia, SS scaled score, TMT Trail Making Test, WMS-III VR Wechsler Memory Scale-III Visual Reproduction, MOANS Mayo Older American Normative Studies, VOSP Visual Object and Space Perception Battery.