Table 1.
Prion-Alzheimer Disease Patients
| Case No. | Age (y) | Sex | Diagnosis | Codon 129 | PrP mutation | PrP type | CERAD | Brain weight (g) |
|---|---|---|---|---|---|---|---|---|
| Study I: Diagnosis based on Bielschowsky preparation, and PrPSc and Aβ42 histoblots | ||||||||
| 1 | nd | nd | GSS | MV | Q217R | Type 1 | Incipient AD | nd |
| 2 | 71 | M | fCJD | MV | A117V | Type 1 | Def AD | 1120 |
| 3 | 68 | F | fCJD | MM | E200K | Type 1 | Incipient AD | nd |
| 4 | 62 | M | fCJD | MM | R208H | Type 1 | Incipient AD | nd |
| 5 | 69 | M | sCJD | MM | — | Type 1 | Incipient AD | 1190 |
| 6 | 60 | M | sCJD | VV | — | nd | Incipient AD | nd |
| 7 | 66 | F | sCJD | MV | — | Type 1–2 | Incipient AD | 1100 |
| 8 | 81 | F | sCJD | VV | — | Type 2 | Incipient AD | 1290 |
| 9 | 65 | F | sCJD | MM | — | Type 1 | Incipient AD | nd |
| 10 | 75 | F | sCJD | VV | — | Type 2 | Incipient AD | 1100 |
| 11 | 83 | F | sCJD | MM | — | Type 1 | Incipient AD | nd |
| 12 | 73 | F | sCJD | VV | — | Type 2 | Incipient AD | nd |
| 13 | 53 | F | sCJD | nd | nd | nd | Incipient AD | nd |
| 14 | 76 | F | sCJD | MV | silent 117 | Type 1 | Incipient AD | 1290 |
| 15 | 70 | NA | sCJD | MM | — | nd | Def AD | 1160 |
| 16 | 74 | F | sCJD | MM | — | Type 1 | Incipient AD | nd |
| 17 | 62 | F | sCJD | MM | — | Type 1 | Incipient AD | 1250 |
| 18 | 77 | F | sCJD | nd | nd | nd | Incipient AD | nd |
| 19 | 73 | F | sCJD | nd | nd | nd | Incipient AD | 1125 |
| 20 | 77 | M | sCJD | MM | — | Type 1 | Incipient AD | 1375 |
| 21 | 66 | M | sCJD | nd | nd | nd | Def AD | nd |
| 22 | 60 | M | sCJD | nd | nd | nd | Def AD | nd |
| 23 | 66 | F | sCJD | nd | nd | nd | Incipient AD | nd |
| 24 | 54 | M | sCJD | Type 2 | Def AD | 1330 | ||
| 25 | 57 | F | sCJD | nd | nd | nd | Incipient AD | nd |
| 26 | 65 | F | sCJD | MM | — | Type 1 | Def AD | nd |
| 27 | 66 | M | sCJD | VV | — | Type 2 | Incipient AD | 1400 |
| 28 | 69 | F | sCJD | nd | nd | nd | Incipient AD | 1175 |
| 29 | 73 | M | sCJD | MM | — | Type 1 | Def AD | nd |
| 30 | 79 | F | sCJD | MM | — | Type 1 | Incipient AD | 1350 |
| 31 | 60 | M | sCJD | nd | nd | nd | Def AD | nd |
| 32 | 73 | F | sCJD | MM | — | Type 1–2 | Incipient AD | 1300 |
| 33 | 52 | M | sCJD | VV | — | Type 2 | Incipient AD | nd |
| 34 | 52 | F | sCJD | MM | — | Type 1 | Incipient AD | 1280 |
| 35 | 70 | M | sCJD | nd | nd | nd | Def AD | nd |
| 36 | 51 | F | fCJD | MM | 6ORI | Type 1 | Def AD | nd |
| Study II: Diagnosis based on PrPSc, Aβ42, and abnormal tau immunohistochemistry | ||||||||
| 37 | 66 | F | sCJD | MM | — | Type 1 | Def AD | 1290 |
| 38 | 75 | M | sCJD | VV | — | Type 2 | Incipient AD | 1500 |
| 39 | 64 | M | sCJD | MM | — | Type 1 | Def AD | 1675 |
| 40 | 59 | M | sCJD | MM(MV) | — | Type 1–2 | Incipient AD | 1560 |
| 41 | 60 | F | sCJD | MM | — | Type 1–2 | Def AD | 1300 |
| 42 | 75 | M | sCJD | MM | — | Type 1 | Incipient AD | 1200 |
| 43 | 82 | F | sCJD | MV | — | Type 1 | Def AD | 1250 |
| 44 | 66 | F | sCJD | __ | Incipient AD | 1290 | ||
| 45 | 79 | M | sCJD | __ | Def AD | nd | ||
| 46 | 52 | M | sCJD | __ | Def AD | 1330 | ||
Alzheimer disease (AD) was classified according Consortium to Establish a Registry for Alzheimer’s Disease (CERAD) criteria using the density of Aβ plaques, as either definite AD (Def AD; varying) or incipient AD (sparse to moderate).
F, female; fCJD, familial Creutzfeldt-Jakob disease; GSS, Gerstmann-Sträussler-Scheinker disease; M, male; nd, not determined; PrPsc, sCJD, sporadic Creutzfeldt-Jakob disease.