Table 3.
Prion-only Cases
| Diagnosis | Sex | Age (y) | Codon 1291 | PrPSc Accumulation in CA1 Region and other neuropathologies | Aβ42 (%) | APOE-4 load | Neuron cell counts | |
|---|---|---|---|---|---|---|---|---|
| 1 | sCJD | F | 57 | MM2 | Prox CA1: Little finely granular PrPSc staining. Distal CA1: Moderate amount of finely granular PrPSc staining. | 90 | 28.0 | 131 |
| 2 | GSS | M | 49 | n.d. | CA4 to CA1: A background of dense finely granular PrPSc staining surrounds neurons and GSS plaques. | 80 | 21.0 | 217 |
| 3 | sCJD | M | 60 | MM2 | Prox CA1: Little or no PrPSc staining. Distal CA1: Moderate degree of PrPSc course deposits. | 80 | 68.0 | 200 |
| 4 | sCJD | M | 68 | MV2 | CA4 to CA1: dense finely granular PrPSc staining surrounding neurons. | 70 | 9.4 | 169 |
| 5 | sCJD | M | 57 | MV2 | Prox CA1: Very little PrPSc. Distal CA1: Moderate amount of finely granular PrPSc surrounding neurons. | 40 | 24.6 | 223 |
| 6 | sCJD | M | 74 | MV1-2 | CA1: Dense finely granular PrPSc; PrP plaques in the SR2 and SO2 and less in the SP2 surrounds nerve cells; a few nerve cell bodies and dendrites contain intracellular PrPSc. | <5 | 60.0 | 122 |
| 7 | sCJD | M | Anon. | MV1-2 | Same as case 6 with some exceptions: Dense, finely granular PrPSc and plaque-like deposits surround dendrites; PrPSc accumulation in nerve cell bodies. Distal: Severe vacuolation CA1 to the EC. | <5 | n.d. | 155 |
| 8 | sCJD | F | 66 | VV2 | All 3 cortical layers of the CA1 stain strongly for PrPSc with focally dense, finely granular PrPSc associated with neuronal cell membranes. | <5 | 35.4 | 105 |
| 9 | sCJD | M | 66 | MV1-2 | Prox CA1: No PrPSc. Distal: Moderate PrPSc in all layers if the CA1. | <5 | 55.2 | 113 |
| 10 | sCJD | F | 60 | MM2 | Prox CA1: Moderate amount of coarse PrPSc filling all 3 layers. | <5 | 56.4 | 179 |
| 11 | sCJD | F | 67 | MV1-2 | CA4 to CA1: Dense, finely granular PrPSc in all 3 layers. | <5 | 40.0 | 115 |
| 12 | sCJD | M | 72 | n.d. | CA1: Sparse amount of finely granular PrPSc in all 3 layers. | <5 | 60.0 | 100 |
| 13 | sCJD | M | 42 | n.d. | Prox CA1: No PrPSc. Distal CA1: ~15% of nerve cell bodies are filled with PrPSc. | <1 | 0.5 | 210 |
| 14 | sCJD | F | 56 | MM1 | CA4 to CA1: Little finely granular PrPSc in the SR; ~50% of CA1 neurons contain intracellular PrPSc; coarsely granular PrPSc is also scattered around dendrites; sparse in the SP. | <1 | 2.3 | 244 |
Comparison of Aβ42, APOE-4 and nerve cell loss in five 10x regions of the CA1 region of the hippocampus. The APOE-4 load is the number of APOE-4-positive neurons in the CA1 multiplied by the APOE-4 staining intensity estimated as 0-none, 1-mild, 2-moderate, and 3-severe. The t-test value for the APOE-4 loads in cases 1–5 and cases 6–12 is 0.07.
1
PRNP genotype and PrPSc phenotype
2
EC, entorhinal cortex; F, female; fCJD, familial Creutzfeldt-Jakob disease; GSS, Gerstmann-Sträussler-Scheinker disease; M, male; n.d., not determined; Prox, proximal; sCJD, sporadic Creutzfeldt-Jakob disease SO, stratum oriens; SP, stratum pyramidale; SR, stratum radiatum.