Table 2.
Prevalence of Movement Disorders in Human Prionopathies
N | sCJD |
vCJD |
iCJD |
gCJD |
FFI |
GSS |
p-value |
---|---|---|---|---|---|---|---|
N(%) |
N(%) |
N(%) |
N(%) |
N(%) |
N(%) |
||
164 | 23 | 12 | 45 | 30 | 50 | ||
Gait ataxia | 103 (62.8%) | 21 (91.3%) | 9 (75%) | 30 (66.7%) | 17 (56.7%) | 37 (74%) | 0.051 |
Limb ataxia | 66 (40.2%) | 9 (39.1%) | 7 (58.3%) | 24 (53.3%) | 8 (26.7%) | 10 (20%) | 0.012 |
Myoclonus | 96 (58.5%) | 13 (56.5%) | 8 (66.7%) | 32 (71.1%) | 20 (66.7%) | 12 (24%) | <0.001 |
Tremor | 8 (4.9%) | 2 (8.7%) | 1 (8.3%) | 3 (6.7%) | 4 (13.3%) | 4 (8%) | 0.47 |
Parkinsonism | 12 (7.3%) | 2 (8.7%) | 1 (8.3%) | 7 (15.6%) | 4 (13.3%) | 9 (18%) | 0.24 |
Rigidity | 72 (43.9%) | 7 (30.4%) | 3 (25%) | 26 (57.8%) | 7 (23.3%) | 20 (40%) | 0.037 |
Chorea | 6 (3.7%) | 7 (30.4%) | 0 (0%) | 0 (0%) | 0 (0%) | 2 (4%) | <0.001 |
Dystonia | 15 (9.1%) | 2 (8.7%) | 0 (0%) | 1 (2.2%) | 0 (0%) | 2 (4%) | 0.28 |
Gaze palsy | 18 (11%) | 3 (13%) | 1 (8.3%) | 1 (2.2%) | 0 (0%) | 0 (0%) | 0.011 |
Abbreviations: FFI, Fatal Familial Insomnia; gCJD, Genetic Creutzfeldt–Jakob Disease; GSS, Gerstmann–Sträussler–Scheinker Disease; iCJD, Iatrogenic Creutzfeldt–Jakob Disease; p-value, Fisher’s exact test; sCJD, Sporadic Creutzfeldt–Jakob Disease; vCJD, Variant Creutzfeldt–Jakob Disease.