Table 1. Summary of the findings related to PrP levels, PrPsc deposition, spongiform degeneration, neuron loss, astrogliosis and microgliosis in sCJD cases in the present series.
sCJD MM1 | sCJD VV2 | |||
---|---|---|---|---|
Frontal cortex | Cerebellum | Frontal cortex | Cerebellum | |
PrP levels | ||||
PrP mRNA | Decreased | Unchanged | Unchanged | Decreased |
PrPc + PrPsc | Decreased | Unchanged | Unchanged | Increased |
PrPsc pattern | Synaptic | Synaptic | Synaptic + Perineuronal + Plaque-like | Synaptic + Plaque-like |
PrPsc | 1 | 1 | 2 | 2 |
Neuropathological hallmarks | ||||
Spongiform degeneration | 1–2 small vacuoles | 1–2 small vacuoles | 2 small vacuoles | 2–3 medium vacuoles |
Purkinje loss | - | -1 | - | 1–2 |
Granule cell loss | - | 0–1 | - | 2 |
Neuronal loss (frontal cortex) | 1–2 | - | 1–2 | - |
Astrogliosis | 2–3 | 1–2 | 2 | 2 |
Microgliosis | 2–3 | 2 | 2 | 2–3 |
Parameters were scored as 0 = absent, 1 = mild, 2 = moderate, and 3 = severe. Mild was considered when at least a 200-fold magnification was needed to observe the alterations, moderate when a 100× magnification was needed, and severe when they were already seen at 20x .