Features of patients with corticobasal degeneration confirmed at postmortem examination
| Features | First visit (n=14) | Last visit (n=14) | Rinne et al1*** (n=30) |
|---|---|---|---|
| Extrapyramidal features: | |||
| Gait disorder | 6 (43) | 14 (100) | 29 (97) |
| Postural instability | 5 (45)‡ | 12 (92)3-150 | NA |
| Falls | 6 (43) | 9 (69)3-150 | NA |
| Bradykinesia | 10 (71) | 11 (92)† | NA |
| Axial rigidity | 2 (17)† | 6 (67)¶ | NA |
| Limb rigidity | 11 (79) | 13 (93) | 24 (80) |
| Tremor: | |||
| Rest | 4 (29) | 3 (21) | NA |
| Action | 1 (7) | 1 (7) | NA |
| Dysarthria (slurred) | 4 (29) | 9 (75)† | 21 (70) |
| Aphonia/anarthia | 1 (7) | 4 (33)† | NA |
| Unilateral limb dystonia | 6 (43) | 6 (46)3-150 | 25 (83) |
| Neck dystonia | 1 (7) | 1 (8)† | NA |
| Focal myoclonus | 3 (25)† | 3 (23)3-150 | 17 (57)†† |
| Cortical signs: | |||
| Constructive apraxia | 7 (64)‡ | 9 (75)† | NA¶¶ |
| Ideomotor apraxia | 7 (64)‡ | 9 (75)† | NA¶¶ |
| Eyelid apraxia | 2 (20)§ | 4 (36)‡ | NA |
| Dysphasia | 4 (29) | 7 (50) | NA |
| Extensor plantar response | 3 (21) | 5 (42)‡ | 22 (73) |
| Hyperreflexia | 5 (36) | 7 (58)† | 22 (73) |
| Alien hand/limb phenomenon | 4 (29) | 6 (46)3-150 | 15 (50) |
| Sensory cortical signs | 4 (31)3-150 | 4 (40)§ | 11 (37) |
| Cortical dementia | 4 (36) | 5 (42)† | 9 (30)*** §§ |
| Other features: | |||
| Limited voluntary vertical gaze | 5 (36) | 8 (62)3-150 | 27 (90)‡‡ |
| Limited vertical pursuit movements | 4 (31)3-150 | 7 (58)† | 27 (90)‡‡ |
| Frontal lobe release signs | 5 (42)† | 8 (73)‡ | NA |
| Frontal lobe behaviour | 4 (29) | 7 (58)† | NA |
| Depression | 4 (31)3-150 | 3 (38)** | NA |
| Cerebellar signs | 4 (31)3-150 | 2 (33)** | NA |
| Visual neglect | 1 (7) | 2 (15)3-150 | NA |
↵3-150 Data missing in one patient; † data missing in two patients;‡ data missing in three patients; § data missing in four patients;¶ data missing in five patients; ** data missing in six patients. ††myoclonus of upper extremity, ‡‡ eye-movement problems (including vertical gaze abnormalities), §§ cognitive deficits (including dementia), ¶¶ apraxia present in 30 cases (100%), not specified if constructive or ideomotor, *** only six patients had pathologically proved corticobasal degeneration.