Table 1 Clinicopathological features of CJD cases with Lewy body disease in the literature
Author (published year) | Ezrin-Waters et al. (1985) [15] | Iida et al. (2001) [16] | Vital et al. (2007) [17] | Haraguchi et al. (2009) [18] | Fernández-Vega et al. (2015) [19] | Vita et al. (2017) [20] | Present case |
|---|---|---|---|---|---|---|---|
Clinical diagnosis | PD + CJD | AD (or LBD) + CJD | CJD | CJD | CJD | CJD | PD + CJD |
Pathological diagnosis | PD + CJD | DLB + CJD | CJD + AD + LBD | CJD + LBD + AD | CJD + LBD + AGD | CJD + LBD | PD + CJD |
Sex | Female | Female | Male | Male | Male | Female | Female |
Age at onset, years | 68 | 64 | 76 | 77 | 77 | 77 | 68 |
Age at the diagnosis of CJD | 71 | 67 | 78 | 77 | 78 | 78 | 84 |
Age at death, years | 71 | 69 | 79 | 77 | 80 | 78 | 85 |
Total disease duration, years (duration from onset to mutism) | 3 (3) | 4 to 5 (2 to 3) | 3 (ND) | 0.4 (0.25) | 2.3 (ND) | > 1 (> 1) | 17 (17) |
Initial symptoms | Akinesia, Shuffling gait, Resting tremor | Insomnia, Restlessness | Fluctuations in mood | Memory disturbance, Visual disturbance | Memory disturbance | Visual hallucination/delusion | Resting tremor, Rigidity, Bradykinesia, Difficulty with ambulation |
Parkinsonism prior to diagnosis of CJD (age at onset of symptoms) | + (68) | + (65) | - | - | - | - | + (68) |
Neuropsychiatric symptoms prior to diagnosis of CJD (age at onset of symptoms) | - | + (64) | ± (76) | - | + (77) | ± (77) | - |
Myoclonus | + | + | + | + | + | ND | + |
Cerebellar signs | ND | ND | + | ND | ND | ND | - |
Visual symptoms | + | ND | ND | + | ND | + | + |
Pyramidal sign | + (hyperreflexia/pathological reflex) | ND | ND | ND | + (pathological reflex*) | - | - |
EEG periodic synchronous discharges | + | + | + | + | + | + | + |
CSF 14-3-3 protein | ND | + | + | + | - | + | + |
MR-DWI hyperintensity of cerebral cortices and/or basal ganglia | ND | ND | ND | + | + | + | + |
PRNP gene | ND | 129MV; 219GG | 129MV | 129MM; 219GG | 129MM | 129MM | 129MM; 219GG |
PrP Western blotting | ND | ND | Type 1 | Type 1 | Type 2 | Type 1+2 | Type 1 |