Incidence of different symptoms in patients with FCCMs (n=64)
Genotype | Yes | No | IR (95%CI) | Mean age to onset (year) | Median age of onset (year) |
Initial symptom appearance | |||||
Overall | 30 | 34 | 67.27 (51.55 to 82.99) | 46.67 (40.56–52.78) | -- |
Unknown | 5 | 14 | 41.18 (10.72 to 71.64) | 57.74 (46.87–68.61) | 40.85 (34.74–46.96) |
CCM1 | 15 | 9 | 81.35 (60.69 to 102.01) | 39.15 (31.43–46.87) | 42.50 (37.24–47.76) |
CCM2 | 7 | 10 | 71.43 (40.68 to 102.18) | 46.44 (36.12–56.75) | 23.32 (15.90–30.74) |
CCM3 | 3 | 1 | 75.00 (32.57 to 117.43) | 28.56 (15.19–41.93) | 45.98 (38.90–53.06) |
ICH | |||||
Overall | 14 | 50 | 44.37 (21.32 to 67.42) | 58.19 (51.87–64.52) | -- |
Unknown | 4 | 15 | 41.18 (6.37 to 75.99) | 61.06 (51.05–71.07) | -- |
CCM1 | 7 | 17 | 48.87 (20.35 to 77.39) | 51.84 (43.52–60.16) | -- |
CCM2 | 1 | 16 | 8.33 (-7.31 to 23.97) | 63.97 (56.16–71.79) | -- |
CCM3 | 2 | 2 | 66.67 (13.32 to 120.02) | 32.98 (18.09–47.87) | 23.90 (23.18–24.62) |
Epilepsy | |||||
Overall | 9 | 55 | 25.63 (5.32 to 45.94) | -- | -- |
Unknown | 0 | 19 | -- | -- | -- |
CCM1 | 5 | 19 | 24.77 (5.84 to 43.70) | -- | -- |
CCM2 | 2 | 15 | 54.17 (-9.82 to 118.16) | -- | -- |
CCM3 | 2 | 2 | 50.00 (1.00 to 99.00) | -- | -- |
‘--’ indicates that the space cannot be calculated by Kaplan-Meier method.
FCCMs, familial cerebral cavernous malformations; ICH, intracranial haemorrhage; IR, incidence rate.