Table 1.
Patient number | ||||||||||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
2 | 3 | 4 | 12 | 13 | 14 | 16 | 17 | 18 | 19 | 20 | 21 | 1 | 5 | 6 | 8 | 10 | 7 | 9 | 11 | |
PPA variant | NFV | NFV | NFV | NFV | NFV | NFV | NFV | NFV | NFV | NFV | NFV | NFV | SV | SV | SV | SV | SV | LV | LV | LV |
Age (years) | 80 | 57 | 62 | 68 | 76 | 66 | 70 | 65 | 49 | 63 | 70 | 76 | 73 | 71 | 63 | 52 | 55 | 77 | 63 | 74 |
Gender | M | F | F | F | M | F | M | M | F | M | M | F | F | F | F | F | M | M | F | M |
Education (years) | 17 | 16 | 12 | 10 | 12 | 15 | 12 | 15 | 12 | 10 | 10 | 12 | 14 | 8 | 13 | 13 | 14 | 10 | 12 | 18 |
Handedness | R | R | L | R | R | R | R | R | R | R | R | R | R | R | R | R | R | R | R | R |
Symptom duration (months) | 33 | 43 | 16 | 60 | 37 | 74 | 39 | 40 | 53 | 29 | 45 | 24 | 19 | 44 | 11 | 131 | 6 | 59 | 95 | 48 |
[11C]PIB SUVR | NC | NC | 1.26 | NC | 2.1 | 1.16 | 1.35 | 1.57 | NC | 1.37 | NC | NC | NC | NC | 1.12 | 1.14 | 1.2 | NC | 1.81 | NC |
CSF Aβ42 (pg/ml) | 816 | 1,057 | NC | 832 | 477 | NC | NC | 759 | 1,077 | NC | 887 | 1,144 | 1,558 | 733 | NC | NC | NC | 564 | 664 | 321 |
CSF t-tau (pg/ml) | 195 | 247 | NC | 320 | 442 | NC | NC | 744 | 231 | NC | 270 | 265 | 428 | 262 | NC | NC | NC | 407 | NC | 858 |
CSF Aβ42/t-tau | 4.18 | 4.28 | NC | 2.60 | 1.08 | NC | NC | 1.02 | 4.66 | NC | 3.29 | 4.32 | 3.64 | 2.80 | NC | NC | NC | 1.39 | NC | 0.37 |
CSF p181-tau (pg/ml) | 42 | 34 | NC | 43 | 59.6 | NC | NC | 87.1 | 31 | NC | 48 | 39.9 | 52 | 36.3 | NC | NC | NC | 65.7 | NC | 95 |
Amyloid-positivity | − | − | − | − | + | − | − | + | − | − | − | − | − | − | − | − | − | + | + | + |
CDR | 1 | 0.5 | 0.5 | 0.5 | 0.5 | 0 | 0.5 | 1 | NC | 0.5 | 0.5 | 0.50 | 1 | 0.5 | 0.5 | 0.5 | 0.5 | 1 | 0 | 0.5 |
MMSE score (/30) | 28 | 24 | 28 | 18 | 24 | 30 | 23 | 5 | NC | 26 | 26 | 18 | 26 | 25 | 29 | 23 | 30 | 26 | 28 | 24 |
CPM score (/36) | 26 | NC | 31 | 29 | 29 | 26 | 29 | 24 | NC | 25 | 26 | 17 | 28 | 27 | 34 | 36 | 34 | 32 | 32 | 12 |
BNT score (/60) | 52 | 7 | 46 | 30 | 43 | 47 | 41 | 4 | 17 | 53 | 53 | 48 | 11 | 14 | 17 | 9 | 33 | 46 | 57 | 24 |
AVF score (1 min) | 8 | 7 | 12 | 2 | 7 | 13 | 9 | 2 | NC | 16 | 4 | 5 | 7 | 11 | 13 | 6 | 16 | 7 | 23 | 8 |
AAT sum single-word comprehension score (/60) | 41 | 56 | 49 | 55 | 41 | 51 | 42 | 37 | 27 | 53 | 51 | 47 | 31 | 32 | 39 | 35 | 58 | 53 | 54 | 50 |
PALPA auditory word–picture matching score (/40) | 40 | 37 | 39 | 39 | 39 | 38 | 39 | 38 | NC | 40 | 40 | 36 | 26 | 27 | 26 | 21 | 39 | 38 | 39 | 38 |
PALPA verbal associative-semantic high imageability (/15) | 11 | 15 | 15 | 10 | 12 | 14 | 14 | 11 | NC | 13 | 10 | 14 | 9 | 15 | 12 | 5 | 14 | 15 | 15 | 15 |
PALPA verbal associative-semantic low imageability (/15) | 12 | 14 | 11 | 7 | 14 | 12 | 11 | 7 | NC | 11 | 9 | 5 | 3 | 7 | 10 | 6 | 14 | 13 | 14 | 12 |
PPT score (/52) | 46 | 47 | 47 | 48 | 47 | 48 | 47 | 47 | NC | 49 | 48 | 45 | 31 | 31 | 38 | 34 | 47 | 49 | 52 | 45 |
BORB easy B score (/32) | 28 | 31 | 25 | 30 | 29 | 28 | 28 | 29 | NC | 30 | 28 | 27 | 22 | 19 | 22 | 18 | 25 | 28 | 30 | 26 |
BORB hard A score (/32) | 25 | 31 | 19 | 22 | 22 | 26 | 24 | 20 | NC | 21 | 30 | 20 | 17 | 19 | 22 | 17 | 26 | 23 | 26 | 21 |
WEZT verb comprehension score (/60) | 56 | 60 | 45 | 40 | 55 | 42 | 52 | 42 | NC | 58 | 55 | 49 | 43 | 32 | 51 | 48 | 58 | 57 | 57 | 53 |
WEZT auditory sentence comprehension score (/40) | 36 | 29 | 36 | 26 | 33 | 35 | 31 | 12 | NC | 33 | 32 | 23 | 37 | 33 | 38 | 38 | 39 | 27 | 40 | 37 |
WEZT active sentence anagram score (/10) | 10 | 10 | 10 | 9 | 9 | 10 | 10 | 5 | NC | 10 | 10 | 10 | 10 | 10 | 10 | 10 | 10 | 10 | 10 | 10 |
WEZT passive sentence anagram score (/10) | 10 | 10 | 9 | 6 | 9 | 10 | 5 | 5 | NC | 10 | 3 | 5 | 10 | 9 | 10 | 9 | 10 | 10 | 10 | 10 |
AAT cognate word repetition score (/30) | 29 | 28 | 30 | 30 | 16 | 30 | 29 | NC | 5 | 29 | 27 | 30 | 30 | 30 | 28 | 30 | 30 | 30 | 29 | 29 |
AAT sentence repetition score (/30) | 27 | 17 | 28 | 24 | 28 | 28 | 28 | NC | 0 | 25 | 23 | 27 | 29 | 29 | 30 | 27 | 30 | 13 | 26 | 24 |
PALPA single word repetition score (/80) | 77 | 76 | 80 | 79 | 55 | 80 | 77 | NC | NC | 49 | 76 | 80 | 79 | 77 | 77 | 80 | 79 | 79 | 80 | 80 |
PALPA pseudoword repetition score (/80) | 21 | 63 | 72 | 72 | 11 | 78 | 57 | NC | NC | 20 | 59 | 53 | 77 | 69 | 67 | 79 | 77 | 66 | 74 | 56 |
DIAS diadochokinesis score | 103 | 24 | 50 | 51 | 42 | 77 | 115 | 75 | NC | 79 | 18 | 32 | 50 | 70 | 147 | 125 | 80 | 77 | 114 | 117 |
Values in bold are significantly different from those in healthy controls based on Crawford and Garthwaite [30] or Crawford and Howell [31] statistical procedures
AAT Akense Afasie Test (Aachen Aphasia Test), Aβ1–42 amyloid-β42, AVF Animal Verbal Fluency, BNT Boston Naming Test, BORB Birmingham Object Recognition Battery, CDR Clinical Dementia Rating scale, [11C]PIB 11C-Pittsburgh Compound B, CPM Coloured Progressive Matrices, CSF cerebrospinal fluid, DIAS Diagnostisch Instrument voor Apraxie van de Spraak (Diagnostic Instrument for Apraxia of Speech), LV logopenic variant, MMSE Mini-Mental State Examination, NFV nonfluent variant, PALPA Psycholinguistic Assessment of Language Processing in Aphasia, PPA primary progressive aphasia, PPT Pyramids and Palm Trees Test, p181-tau phospho181-tau, SUVR standardized uptake value ratio in a composite cortical volume of interest, SV semantic variant, WEZT Werkwoorden En Zinnen Test. NC no data collected