Table 1.
Summary of sCJD patient information: sCJD PrPres glycotype 1–3, grouped by codon 129 genotype
| Codon 129 genotype | Glycotype | n | Sex (M, F) | Age (years), median (range) | Disease duration (months), median (range) | Typical magnetic resonance imaginga | Positive 14-3-3a | Typical electro encephalograma | PMDb (days), median (range) | Storage (years), median (range) |
|---|---|---|---|---|---|---|---|---|---|---|
| MM | 1 | 6 | 1, 5 | 72 (58–81) | 2.2 (1–3) | 5/5 | 5/5 | 2/5 | 1.5 (0–3) | 11.5 (5–12) |
| MM | 2 | 6 | 2, 4 | 64 (60–82) | 4.6 (1.5–9.6) | 5/5 | 6/6 | 4/5 | 2 | 11.5 (9–13) |
| MM | 3 | 6 | 4, 2 | 56.5 (47–85) | 8.4 (2–21) | 4/4 | 3/5 | 1/4 | 1 (0–2) | 11.5 (8–15) |
| MV | 1 | 2 | 1, 1 | 65.5 (58–73) | 1.8 (1.5–2.1) | 2/2 | 2/2 | 1/2 | 2 (1–3) | 14 (11–17) |
| MV | 2 | 4 | 2, 2 | 67 (63–74) | 2.7 (2–8) | 4/4 | 3/4 | 0/4 | 3 | 11.5 (7–13) |
| MV | 3 | 5 | 1, 4 | 67 (50–75) | 11.5 (6–40.5) | 4/4 | 3/3 | 1/3 | 2.5 (2–6) | 12 (10–15) |
| VV | 3 | 6 | 3, 3 | 71 (59–78) | 4.5 (2–6.5) | 6/6 | 3/3 | 1/5 | 4 (2–6) | 11 (6–15) |
| Total | 35 | 14, 21 | 67 (47–85) | 3.5 (1–40.5) | 30/30 | 25/28 | 10/28 | 2 (0–6) | 12 (5–17) | |
Disease duration and age are represented as median (range). Wherever performed, outcome of ante-mortem clinical testing results suggestive of CJD are provided. Variables relating to tissue handling are included as PMD and storage time of tissues prior to experimental use.
a
Values indicate the number of positive outcomes/total number of results available.
b
Value indicates number reported, where information is available.
PMD, post-mortem delay; F, female; M, male; VV, homozygous valine; PrPres, proteinase K-resistant PrP; MV, heterozygous methionine/valine.