Table 1.
Summary of clinicopathological data
| 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 |
| Other | - | Focal encephalitis in brainstem Lacunar infarcts in Globus pallidus and brainstem |
- |
ABC score indicates the score provide for Aβ deposition (A), for Braak stage of neurofibrillary degeneration (B), and neuritic plaque score (C)(28).
PSP: progressive supranuclear palsy; MCI: mild cognitive impairment.