Clinical features of the dura mater graft-associated Creutzfeldt-Jakob disease cases for all cases, the supratentorial group and the infratentorial group
| Total (n=84)* | Supratentorial group (n=36) | Infratentorial group (n=39) | p Value† | |
|---|---|---|---|---|
| Type classification‡ | ||||
| Non-plaque type (%) | 53 (63) | 20 (56) | 28 (72) | ns |
| Plaque type (%) | 18 (21) | 9 (25) | 8 (21) | ns |
| Male/female | 35/49 | 19/17 | 10/29 | 0.015 |
| Age at dural grafting§ (years) | 45 (1–65) | 45 (1–60) | 51 (7–65) | ns |
| Incubation period§ (years) | 15 (6–30) | 15 (8–30) | 14 (6–25) | ns |
| Age at onset§ (years) | 61 (15–80) | 61 (15–79) | 66 (24–80) | ns |
| Initial manifestations¶ (%) | (n=63) | (n=30) | (n=26) | |
| Unsteady gait | 30 (48) | 16 (53) | 11 (42) | ns |
| Dementia | 16 (25) | 8 (27) | 6 (23) | ns |
| Vertigo | 9 (14) | 1 (3) | 8 (31) | 0.007 |
| Behavioural abnormality | 7 (11) | 5 (17) | 2 (8) | ns |
| Ataxia | 7 (11) | 4 (13) | 1 (4) | ns |
| Diplopia | 4 (6) | 0 (0) | 4 (15) | 0.041 |
| Sensory disturbance | 4 (6) | 2 (7) | 2 (8) | ns |
| Visual disturbance | 3 (5) | 0 (0) | 1 (4) | ns |
| Extrapyramidal signs | 2 (3) | 1 (3) | 1 (4) | ns |
| Others** | 5 (8) | 5 (17) | 0 (0) | — |
| Manifestations during clinical course (%) | ||||
| Cerebellar signs | 62/82 (76) | 23/36 (64) | 32/37 (87) | 0.024 |
| Psychiatric feature | 51/79 (65) | 20/32 (63) | 27/38 (71) | ns |
| Dementia | 82/84 (98) | 35/36 (97) | 38/39 (97) | ns |
| Visual disturbance | 36/81 (44) | 16/35 (46) | 18/37 (49) | ns |
| Myoclonus | 71/82 (87) | 31/36 (86) | 34/37 (92) | ns |
| Extrapyramidal signs | 53/82 (65) | 25/36 (69) | 26/37 (70) | ns |
| Pyramidal signs | 58/81 (72) | 28/35 (80) | 25/37 (68) | ns |
| PrP gene polymorphisms | (n=58) | (n=25) | (n=27) | |
| Codon 129 | MM 56, MV 2 | MM 25 | MM 25, MV 2 | ns |
| Codon 219 | EE 52, EK 3 | EE 21, EK 3 | EE 26 | ns |
*Total includes two cases with spinal cord regions and seven cases with uncertain grafting regions.
†p Value was assessed between the supratentorial group and the infratentorial group.
‡Thirteen cases of type classification were unclear.
§Median.
¶Twenty-two cases of the total, 9 cases of the supratentorial group and 8 cases of the infratentorial group developed more than one initial manifestation.
**Others include individual cases of hemiparesis, dysarthria, incontinence, hearing disturbance and nystagmus.
EE, glutamine homozygote; EK, glutamine/lysine heterozygote; MM, methionine homozygote; MV, methionine/valine heterozygote; ns, not significant; PrP, prion protein.