Table 2.
Summary of PSPr common features
| Mean age at onset (range) |
Mean duration (range) |
Clinical Presentation |
Histopathology | PrP IHC | Abnormal PrP | Family History |
PrP Genetics |
|---|---|---|---|---|---|---|---|
| 62 (48–71)yrs | 20 (10–60)mo1 | Cognitive decline (8/11)2 and mood/ behavioral changes (7/11)2 |
Minimal spongiform degeneration with vacuoles larger than typical CJD; and minimal or no astrogliosis |
Intense staining with distinct target pattern in cerebral gray matter; and dot pattern in cerebellar molecular layer |
Minimal amount of PK-resistant PrP forming a ladder-like pattern on Western Blot |
Dementia (8/10)2. Dementia with onset < 61 (2/4)2 |
Valine homozygosity at codon 129. No mutation in the PrP gene coding region |
1
One patient alive after 23-month duration and one dead 7 months from onset for other causes excluded.
2
positive cases/total number of cases.