129MM n=139 | 129MV n=45 | p Value | |
---|---|---|---|
Male/female | 58/81 | 20/25 | 0.862 |
Age at onset (years)* | 77.3±6.8 (78, 44–93, n=139) | 76.7±7.6 (78, 57–92, n=45) | 0.701 |
Period from onset to death (months)* | 23.1±15.1 (19, 5–70, n=75) | 27.8±16.3 (25, 7–64, n=23) | 0.159 |
Myoclonus† | 46/130 (35.4%) | 21/43 (48.8%) | 0.149 |
Period from onset to myoclonus (months)* | 6.4±6.1 (5, 0–36, n=38) | 9.2±7.2 (7, 2–30, n=18) | 0.154 |
Cognitive impairment† | 138/138 (100.0%) | 43/44 (97.7%) | 0.242 |
Period from onset to cognitive impairment (months)* | 0.5±1.4 (0, 0–7, n=121) | 0.6±1.4 (0, 0–5, n=38) | 0.456 |
Pyramidal signs† | 66/132 (50.0%) | 14/42 (33.3%) | 0.075 |
Period from onset to pyramidal signs (months)* | 3.9±5.8 (3, 0–36, n=58) | 5.2±4.2 (5, 0–14, n=11) | 0.136 |
Extrapyramidal signs† | 71/133 (53.4%) | 23/40 (57.5%) | 0.719 |
Period from onset to extrapyramidal signs (months)* | 3.8±3.5 (3, 0–19, n=58) | 3.8±4.5 (2, 0–16, n=18) | 0.460 |
Cerebellar dysfunction† | 40/119 (33.6%) | 12/38 (31.6%) | 1.000 |
Period from onset to cerebellar dysfunction (months)* | 2.9±2.7 (3, 0–9, n=33) | 3.4±4.1 (3, 0–12, n=8) | 0.973 |
Visual disturbance† | 10/109 (9.2%) | 1/34 (2.9%) | 0.460 |
Period from onset to visual disturbance (months)* | 2.2±1.5 (2, 0–4, n=10) | (n=0) | NA |
Psychiatric symptoms† | 68/130 (52.3%) | 16/38 (42.1%) | 0.357 |
Period from onset to psychiatric symptoms (months)* | 1.6±3.0 (0, 0–19, n=62) | 2.0±2.6 (0, 0–7, n=13) | 0.576 |
Akinetic mutism† | 74/137 (54.0%) | 30/44 (68.2%) | 0.116 |
Period from onset to akinetic mutism (months)* | 9.8±6.6 (8, 1–27, n=64) | 13.2±10.9 (9, 0–49, n=23) | 0.190 |
PSWCs on EEG† | 10/131 (7.6%) | 5/39 (12.8%) | 0.339 |
Hyperintensities on MRI† | 135/136 (99.3%) | 44/44 (100.0%) | 1.000 |
Positive rate of 14-3-3 protein in CSF† | 46/53 (86.8%) | 11/18 (61.1%) | 0.035 |
Positive rate of t-τ protein in CSF† | 48/53 (90.6%) | 12/18 (66.7%) | 0.014 |
Amount of t-τ protein in CSF (pg/mL)* | 2965±1712 (2400, 146.0–9940.0, n=53) | 2025±1441 (1689, 170.0–6430.0, n=18) | 0.022 |
Positive rate of PrPSc in CSF† | 36/53 (67.9%) | 7/18 (38.9%) | 0.049 |
*Age of onset, period from disease onset to death or the appearance of each symptom and sign and CSF biomarker level are presented as mean±SD (median, range, case number).
†Frequencies of positive cases are presented as positive cases/total cases (percentage).
Medians are compared using the two-tailed Mann-Whitney U test for age of onset, the period from disease onset to death or the appearance of each symptom and sign and the CSF biomarker level. Frequencies of positive cases are compared using the two-tailed Fisher's exact test.
CSF, cerebrospinal fluid; PSWCs, periodic sharp wave complexes; t-τ, total τ.