Table 1 Patient demographic and clinical characteristics
Characteristic | Total (n = 51) | FMF (n = 38) | CAPS (n = 6) | TRAPS (n = 5) | DADA2 (n = 2) |
|---|---|---|---|---|---|
Demographics | |||||
Age at onset (years) | 31.3 (11.8) | 29.3 (9.9) | 38.8 (16.6) | 31.2 (9.2) | 46 (28.3) |
Gender distribution (% Female) | 19 (37.2%) | 16 (42.1%) | 1 (16.7%) | 1 (20%) | 1 (50%) |
Neurological signs and symptoms | |||||
Headache | 29 (56.9%) | 19 (50%) | 5 (83.3%) | 5 (100%) | 0 |
Focal deficits | 14 (27.4%) | 12 (31.6%) | 0 | 0 | 2 (100%) |
Seizures | 7 (13.7%) | 3 (7.9%) | 3 (50%) | 0 | 1 (50%) |
Cranial nerve dysfunction | 14 (27.4%) | 9 (23.7%) | 3 (50%) | 0 | 1 (100%) |
Neuroimaging patterns | |||||
Aseptic meningitis | 11 (21.6%) | 8 (21%) | 3 (50%) | - | 0 |
CNS demyelination | 23 (45.1%) | 21 (55.3%) | 2 (33.3%) | - | 0 |
Stroke | 7 (13.7%) | 4 (10.5%) | 1(16.7%) | - | 2 (100%) |
Laboratory findings | |||||
Most frequently associated genes | - | MEFV | NLRP3 | TNFRSF1A | ADA2 (CECR1) |
Elevation of serum CRP or ESR | 14 (73.7%) | 9 (64.3%) | 3 (100%) | - | 2 (100%) |