Table 3.
Distribution and severity of tau pathology in the PNS as well as co-pathology in 15 cases of progressive supranuclear palsy
| PSP Case | 1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 | 9 | 10 | 11 | 12 | 13 | 14 | 15 | Tau (+)/available number of cases (%) | |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Cranial | I (Olf. bulb) | na | na | na | − | na | ++ | na | na | na | na | ++ | ++ | na | na | na | 3/4 (75) |
| II | na | na | na | na | + | + | na | na | na | na | + | na | na | na | na | 3/3 (100) | |
| III | ++ | na | − | + | ++ | na | + | na | na | na | na | ++ | na | ++ | +++ | 7/8 (88) | |
| IV, intraa | na | na | na | na | na | na | na | na | na | na | ++++ | na | ++ | na | na | 2/2 (100) | |
| V | na | na | na | na | na | na | na | na | na | +++ | na | na | na | na | na | 1/1 (100) | |
| VI, intraa | na | na | na | na | na | +++ | na | ++ | na | na | na | na | na | na | na | 2/2 (100) | |
| VII, intraa | na | na | na | na | na | +++ | na | ++ | na | na | na | na | na | na | na | 2/2 (100) | |
| IX/X | +++ | ++ | − | na | +++ | ++ | na | na | + | ++ | ++ | ++ | ++ | na | +++ | 10/11 (91) | |
| XI | na | na | na | na | na | na | ++ | na | na | +++ | +++ | +++ | na | na | na | 4/4 (100) | |
| XII | na | ++ | na | na | na | ++++ | ++ | na | + | na | ++++ | +++ | na | na | na | 6/6 (100) | |
| Spinal (A/P) | Cervical | ++++/+ | +/na | +/+ | +/− | ++++/na | na | na | ++/− | ++/++ | ++++/na | na | +++/na | +++/na | na | na | A:10/10(100), P: 3/5 (60) |
| Thoracic | na | na | na | +/− | na | na | na | +/− | na | na | na | na | na | na | na | A: 2/2 (100), P: 0/2 (0) | |
| Lumbar | na | na | na | +/− | na | na | na | +/− | na | na | na | na | na | na | na | A: 2/2 (100), P: 0/2 (0) | |
| Cauda equina | na | na | na | − | na | na | na | − | na | na | na | na | na | na | na | 0/2 (0) | |
| Tau-positive/available number of areas (%) |
3/3 (100) |
3/3 (100) |
1/3 (33) |
4/6 (66) |
4/4 (100) |
6/6 (100) |
3/3 (100) |
5/6 (83) |
3/3 (100) |
4/4 (100) |
6/6 (100) |
6/6 (100) |
3/3 (100) |
1/1 (100) |
2/2 (100) |
||
| Other features | TDP-43 (+)/available number of cases (%) | ||||||||||||||||
| pTDP-43 (CNS) | Motor area | − | − | − | + | − | − | − | − | − | − | − | − | − | − | − | 1/15 (6.7) |
| Spinal cord | − | na | − | + | − | na | na | + | − | − | na | − | na | na | na | 2/8 (25) | |
| Co-pathology | AD (15) | Not | Not | Int. | Not | Not | Not | Not | Int. | Low | Not | Not | Low | Low | Not | Low | |
| AGD (18) | 0 | 0 | 0 | 3 | 0 | 3 | 0 | 3 | 1 | 0 | 1 | 1 | 0 | 0 | 0 | ||
| LATE (20) | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 1 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | ||
| LBD (19) | 0 | 1 | 4 | 0 | 0 | 0 | 0 | 0 | 0 | 2 | 0 | 0 | 1 | 0 | 0 | ||
A = anterior spinal roots; AD = Alzheimer’s disease; AGD = argyrophilic grain disease; Int. = intermediate; intra = intramedullary; LATE = limbic predominant age-related TDP-43 encephalopathy; LBD = Lewy body disease; na = not available; Olf = olfactory; P = posterior spinal roots. Semiquantitative assessment: − = absent; + = minimal; ++ = mild; +++ = moderate; ++++ = severe.
aA part of cranial nerves (IV, VI and VII) was only evaluated in the intramedullary nerve fibres.