Table 4.

Factors associated with shorter time from disease onset to loss of autonomy

Phenotypic characteristic variable versus reference category	Unadjusted HR (95% CI)	P-value	Adjusted HR (95% CI)	P-value
Geographic origin
Asian vs European	2.5 (0.9–7.0)	0.071	4.1 (1.4–12.7)	0.013
African vs European	7.4 (0.3–180)	0.222	7.4 (0.2–284)	0.28
American vs European	1.8 (0.7–4.7)	0.20	2.3 (0.8–6.8)	0.13
Sex
Male versus female	1.0 (0.4–2.8)	0.95
Age at onset
≥ 18 versus < 18 years	0.48 (0.24–0.96)	0.039	0.18 (0.04–0.79)	0.024
Consanguinity
Present versus Absent	1.2 (0.5–2.5)	0.71
Mutated gene
EPM2B versus EPM2A	2.7 (0.9–8.6)	0.084	1.6 (0.4–7.4)	0.53
Compound heterozygosity
Present versus absent	0.8 (0.4–1.6)	0.52
Type of onset
Motor versus Epileptic	0.9 (0.3–3.1)	0.93	0.9 (0.3–3.0)	0.82
Cognitive versus Epileptic	0.5 (0.2–1.6)	0.26	0.7 (0.2–2.0)	0.49

Bold means statistically significant value (P < 0.005)