Table 1.
Summary of clinicopathological data. ABC score indicates the score provide for Aβ deposition (A), for Braak stage of neurofibrillary degeneration (B) and neuritic plaque score (C) 28. Abbreviations: PSP, progressive supranuclear palsy; MCI, mild cognitive impairment.
| Case 1 | Case 2 | Case 3 | |
|---|---|---|---|
| Age at death (years) | 83 | 72 | 91 |
| Sex | Male | Female | Female |
| Duration of illness (years) | 20 | 5 | 20 |
| Clinical diagnosis | PSP+ MCI | PSP+ dementia | Unclassified dementia |
| Symptoms at early stage | Imbalance and gait disorder | Incoordination | Not available |
| Symptoms at late stage | Cognitive decline | Cognitive decline | Cognitive decline |
| Supranuclear gaze palsy | Supranuclear gaze palsy | ||
| 2 grand mal seizures | Parkinsonism | ||
| Motor–dementia interval | 15 years | 0 years | 0 years |
| Family history | No | No | Yes |
| ApoE | E2/E3 | E3/E3 | E2/E3 |
| H1/H2 haplotype | H1/H1 | H1/H1 | H2/H2 |
| MAPT mutation | None | None | None |
| Brain weight | 1378 | 1147 | 1066 |
| ABC score | A0B2C0 | A1B1C0 | A3B3C3 |
| pTDP‐43 | No | No | Yes |
| α‐Synuclein | No | No | No |
| Hippocampal sclerosis | no | yes | Yes |
| Others | – | Focal encephalitis in brainstem | – |
| Lacunar infarcts in Globus pallidus and brainstem |