Table 3 Factors associated with shorter survival
Phenotypic characteristic variable versus reference category | Unadjusted HR (95% CI) | P-value | Adjusted HR (95% CI) | P-value |
|---|---|---|---|---|
Geographic origin | Â | Â | Â | Â |
Asian versus European | 3.5 (1.3–9.2) | 0.011 | 5.0 (1.8–13.4) | 0.001 |
African versus European | 3.4 (0.4–25.3) | 0.24 | 3.6 (0.5–24.9) | 0.20 |
American versus European | 2.4 (0.8–7.3) | 0.11 | 3.2 (1.03–9.6) | 0.044 |
Sex | Â | Â | Â | Â |
Male versus female | 1.4 (0.7–2.6) | 0.27 |  |  |
Age at onset | Â | Â | Â | Â |
 ≥ 18 versus < 18 years | 0.44 (0.23–0.85) | 0.014 | 0.21 (0.06–0.79) | 0.021 |
Consanguinity | Â | Â | Â | Â |
Present vs Absent | 1.5 (0.7–3.0) | 0.31 |  |  |
Mutated gene | Â | Â | Â | Â |
EPM2B versus EPM2A | 1.1 (0.5–2.5) | 0.75 | 1.3 (0.5–3.0) | 0.59 |
Compound heterozygosity | Â | Â | Â | Â |
Present vs Absent | 0.8 (0.4–1.7) | 0.63 |  |  |
Type of onset | Â | Â | Â | Â |
Motor versus Epileptic | 1.9 (0.9–4.1) | 0.091 | 1.4 (0.7–3.0) | 0.36 |
Cognitive versus Epileptic | 1.0 (0.4–4.0) | 0.96 | 0.8 (0.3–2.0) | 0.59 |