Table 1.
Characteristics of 47 participants with sporadic 4R tauopathy
| Characteristics | Total (n = 47) |
|---|---|
| Demographics | |
| Female, n (%) | 22 (47%) |
| Education, year | 16 (14, 16) |
| Age at onset, year | 64 (58, 70) |
| Age at death, year | 73 (67, 78) |
| Disease duration, year | 6 (4, 8) |
| Neuroimaging | |
| Age at MRI, year | 70 (65, 75) |
| Age at DTI, year | 70 (65, 75) |
| MRI to death, year | 2.3 (1.2, 3.1) |
| DTI to death, year | 2.3 (1.3, 3.1) |
| Clinical data | |
| Final clinical diagnosisa | |
| PSP-RS | 18 (39%) |
| PSP-CBS | 2 (4%) |
| PSP-F | 2 (4%) |
| PSP-P | 2 (4%) |
| PSP-PGF | 1 (2%) |
| CBS | 19 (41%) |
| BvFTD ± parkinsonism | 2 (4%) |
| MoCA score/30 | 20 (15, 24) |
| MDS—UPDRS III score/132 | 60 (43, 72) |
| PSP rating scale score/100 | 54 (37, 67) |
| Neuropathology | |
| PSP | 28 (60%) |
| CBD | 19 (40%) |
| NFT positive, n (%) | 45 (96%) |
| Braak NFT stage | |
| I | 9 (20%) |
| II | 7 (16%) |
| III | 19 (42%) |
| IV | 9 (20%) |
| V | 1 (2%) |
| VI | 0 (0%) |
| ARTAG positive, n (%) | 23 (49%) |
| AGD positive, n (%) | 19 (40%) |
Data are shown as median (Q1, Q3) or count (%).
One patient had an ambiguous clinical presentation and was not assigned a clinical diagnosis. Autopsy later revealed PSP pathology.
AGD = argyrophilic grain disease; ARTAG = aging-related tau astrogliopathy; bvFTD = behavioural variant of frontotemporal dementia; CBD = corticobasal degeneration; CBS = corticobasal syndrome; DTI = diffusion tensor imaging; MDS-UPDRS III = Movement Disorders Society-sponsored revision of the Unified Parkinson’s Disease Rating Scale; MoCA = Montreal Cognitive Assessment; NFT = neurofibrillary tangle; PSP = progressive supranuclear palsy; PSP-F = PSP-frontal; PSP-P = PSP-parkinsonism; PSP-PGF = PSP-progressive gait freezing; PSP-RS = PSP-Richardson syndrome.