Table 1. .
Characteristics of NFV patients.
| Case | 6 | 13 | 15 | 20* | 21* | 22* | 23* | 25 | 31* | 36 | 37 | 38 | Cut-off |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Age | 52 | 79 | 71 | 78 | 72 | 63 | 62 | 57 | 69 | 58 | 80 | 65 | / |
| Sex | F | F | M | M | F | F | F | F | M | F | F | M | / |
| Education | 17 | 8 | 15 | 17 | 12 | 15 | 12 | 10 | 18 | 12 | 10 | 16 | / |
| CPM (/36) | 31 | 24 | 24 | 30 | 12 | 32 | 31 | / | 29 | 21 | 4 | 35 | <28 |
| Dis. Dur. | 2 | 5 | 1.5 | 2.5 | 2.5 | 5 | 2.5 | 3.5 | 1.5 | 4 | 4 | 3 | / |
| BNT (/60) | 58 | 48 | 55 | 48 | 30 | 41 | 46 | 7 | 29 | 52 | 27 | 57 | <50 |
| AAT rep 1 (/30) | 28 | 29 | 30 | 29 | 26 | 30 | 28 | 27 | 20 | 28 | 14 | 30 | <28 |
| AAT rep 2 (/30) | 26 | 24 | 21 | 30 | 28 | 30 | 30 | 29 | 30 | 28 | 25 | 30 | <29 |
| AAT rep 3 (/30) | 28 | 22 | 29 | 28 | 24 | 30 | 30 | 28 | 29 | 28 | 27 | 29 | <29 |
| AAT rep 4 (/30) | 26 | 23 | 30 | 29 | 24 | 30 | 29 | 15 | 29 | 28 | 14 | 30 | <28 |
| AAT rep 5 (/30) | 26 | 27 | 30 | 30 | 15 | 30 | 28 | 17 | 28 | 23 | 10 | 30 | <28 |
| DS | 6 | 3 | 4 | 6 | 2 | 4 | 5 | 3 | 7 | 5 | 3 | 4 | <4 |
| DIAS cons (/15) | / | / | / | 13 | / | 15 | 10 | 14 | 13 | 3 | 0 | 15 | <14 |
| DIAS vow (/15) | / | / | / | 15 | / | 15 | 14 | 14 | 14 | 15 | 6 | 15 | <15 |
| DIAS dia | / | / | / | 103 | / | 77 | 50 | 24 | 115 | 48 | 6 | 47 | <56 |
| WEZT (/40) | / | / | / | 36 | / | 35 | 36 | 29 | 31 | 20 | 11 | 38 | <38 |
| Dysarthria | − | − | S | − | − | − | S | − | H | − | SH | H | / |
| Extrapyr | − | − | + | − | − | + | + | + | + | − | − | − | / |
| CIT Spect | / | / | / | / | / | / | / | + | / | / | / | / | / |
| FDG-PET | SMA | L IFG | L Frontal | L IFG | Nl | / | Nl | / | Nl | L SMA | L Frontal | L SMA | / |
| Tau CSF | / | 312 | / | 195 | 424 | 183 | / | 247 | / | 298 | 273 | 530 | >367 |
| Aβ42 CSF | / | 1028 | / | 816 | 1060 | 865 | / | 1057 | / | 1320 | 1264 | 1139 | <500 |
| Neuropath | / | CBD | CBD | / | / | / | / | / | / | / | / | / | / |
Note. Norms were calculated as 2 standard deviations below the mean in the age- and education-matched group of healthy controls (n = 29). CPM: Raven’s progressive matrices. Dis. Dur.: disease duration (years). BNT: Boston Naming test. AAT: Akense Afasie test repetition scores parts 1 to 5. DS: digit span forward. DIAS cons: Diagnostic Instrument for AOS (DIAS) repetition of consonants. DIAS vow: DIAS repetition of vowels. DIAS dia: DIAS diadochokinesis score. WEZT: grammaticality score on Verbs and Sentences Test. Dysarthria: if present, the type of dysarthria is indicated (S: spastic, H: hypokinetic, SH: mixed spastic-hypokinetic). Extrapyr: extrapyramidal signs upon examination. CIT Spect: [123I]ß-CIT (2ß-carbomethoxy-3ß-(4-iodophenyl)tropane) SPECT to evaluate the presynaptic dopaminergic system. FDG-PET: [18F]fluorodeoxyglucose PET, hypometabolism was observed in the SMA, IFG, or whole frontal lobe, either bilateral or on the left side (L). Cerebrospinal fluid (CSF) analysis of Aβ1-42 and total tau using ELISA. Neuropath: anatomopathological findings. *Also meets criteria for the mixed variant of PPA. NFV: non-fluent variant of primary progressive aphasia. Values outside the normal range are in bold.