Table 1.
Demographic data, symptoms, and auxiliary examinations of sCJD patients
Demographic data, symptoms, and auxiliary examinations | Our cases |
---|---|
Gender (male:female) | 1.21:1 |
Median age at onset (range), years | 60 (29–82) |
Duration of disease (range), months | 9 (1–36) |
Rapid progressive dementia | 100% (104/104) |
Cerebellar ataxia | 51.9% (54/104) |
Akinetic mutism | 45.2% (47/104) |
Pyramidal | 43.3% (45/104) |
Extrapyramidal | 32.7% (34/104) |
Visual disturbance | 31.7% (33/104) |
Psychiatric | 7.7% (8/104) |
Sleep disorders | 4.8% (5/104) |
DWI changes (hyperintensity) | 88.2% (90/102) |
Cortex | 82.4% (84/102) |
Basal ganglia | 38.2% (39/102) |
Thalamus | 8.8% (9/102) |
14-3-3 protein in the CSF (positive) | 34.1% (15/44) |
EEG changes (PSWCs) | 38.2% (39/102) |
PET changes (hypometabolism) | 96% (48/50) |
Biopsy | 10/104 |
Autopsy | 1/104 |
sCJD, sporadic Creutzfeldt-Jakob disease; DWI, diffusion-weighted imaging; CSF, cerebrospinal fluid; EEG, electroencephalogram; PSWCs, periodic sharp wave complexes; PET, positron emission tomography.