Table 1.
Clinical, imaging, and EEG characteristics of the cohort
| Clinical variable (n=50) | Value (%) |
|---|---|
| Age (mean±SD) | 59.76±8.17 (37-78) |
| Gender M: F | 31:19 |
| Interval between EEG and clinical onset in days (mean±SD) | 141.94±220.29 (7-1440) |
| Interval between EEG and death in days n=19 (mean±SD) | 261.68±343.70 |
| Median Survival duration in days n=19 | 270 (60-1980) |
| Myoclonus n (%) | 35 (70) |
| Dementia n (%) | 44 (88) |
| Psychiatric disturbances n (%) | 38 (76) |
| Extrapyramidal symptoms/signs n (%) | 28 (56) |
| Ataxia n (%) | 15 (30) |
| Pyramidal symptoms/signs n (%) | 18 (36) |
| Akinetic mutism n (%) | 16 (32) |
| Visual symptoms n (%) | 9 (18) |
| Seizures n (%) | 2 (4) |
| Previous surgery | 2 (4) |
| Abnormal MRI supporting CJD | 48 (96) |
| Bilateral MRI changes n=44 | 44 (100) |
| Symmetric n=45 | 8 (17.8) |
| Right > left n=45 | 22 (48.9) |
| Left > right n=45 | 15 (33.3) |
| Cortical ribbon | 46 (92) |
| Cortical ribbon distribution anteriorly prominent n=41 | 4 (9.8) |
| Cortical ribbon distribution posteriorly prominent n=41 | 28 (70.7) |
| Cortical ribbon distribution uniform n=41 | 8 (19.5) |
| Basal ganglia signal changes | 38 (76) |
| Caudate and lentiform uniform involvement n=37 | 8 (21.6) |
| Caudate more than lentiform involvement n=37 | 25 (67.6) |
| Caudate less than lentiform involvement n=37 | 4 (10.8) |
| Bilateral thalamic signal changes | 17 (34) |
| Overall MRI is suggestive of cortical more than BG involvement n=46 | 25 (54.3) |
| Overall MRI is suggestive of BG more than cortical involvement n=46 | 10 (21.7) |
| Overall MRI is suggestive of BG and cortical uniform involvement n=46 | 11 (23.9) |
| Signal changes only in DWI n=44 | 14 (30.2) |
| Signal changes DWI and T2 FLAIR n=44 | 30/43 (69.8) |
| Abnormal EEG | 44 (88) |
| Background abnormal | 42 (84) |
| PDR absent | 31 (62) |
| Predominant BG frequency | |
| Beta | 1 (2) |
| Alpha | 13 (26) |
| Theta | 27 (54) |
| Delta | 9 (18) |
| Background reactivity | |
| Nonreactive | 5 (10) |
| Poorly reactive | 34 (68) |
| Well reactive | 11 (22) |
| FIRDA | 8 (16) |
| Periodic complexes | 33 (66) |
| Morphology (n=33) | |
| Biphasic | 23 (69.7) |
| Triphasic | 10 (30.3) |
| Abundance of PSWC (n=33) | |
| Infrequent | 11 (33.3) |
| Frequent but not continuous | 9 (27.2) |
| Continuous | 13 (39.4) |
| PSWC peak gradient (n=33) | |
| No gradient | 15 (45.5) |
| Anterior to posterior | 8 (24.2) |
| Posterior to anterior | 2 (6.1) |
| Mixed | 5 (15.2) |
| Central to periphery | 3 (9.1) |
| PSWC amplitude (n=33) | |
| Frontal maximum | 12 (36.4) |
| Occipital maximum | 14 (42.4) |
| Temporal maximum | 3 (9.1) |
| Mixed | 4 (12.1) |
| PSWC symmetry (n=33) | |
| Symmetric | 3 (9.1) |
| Right >left | 19 (57.6) |
| Left >right | 11 (33.3) |
| PSWC synchrony (n=33) | |
| Unilateral/Bilateral independent | 2 (6.1) |
| Bilateral synchronous | 13 (39.4) |
| Independent as well as synchronous | 18 (54.5) |