Figure 4.

Behavioural seizures in homozygous mutant N5 mouse strains recorded by video after weaning A, continuous 12 h daylight monitoring in four knockin homozygous mutant mice illustrating the dependence of seizure frequency and type on genetic background strain, mutation and age. B6-Q2 (B6.129-Kcnq2^A306T/A306T; n = 5; at least one stage 5 seizure per animal), B6-Q3 (B6.129-Kcnq3^G311V/G311V; n = 9; 4 out of 9 animals experienced no seizures during the monitoring period), FVB-Q2 (FVB.129-Kcnq2^A306T/A306T; n = 2, most animals die of presumed seizures between P16 and P30) and FVB-Q3 (FVB.129-Kcnq3^G311V/G311V; n = 9; see Supplemental Table 1 for detailed seizure number). Horizontal black lines correspond to monitoring period for each mouse; black diamond, animals died during or immediately after seizure; arrow, mouse was monitored until P116. B, during the 12 h light cycle, a typical FVB.129-Kcnq3^G311V/G311V knockin mouse experienced spontaneous generalized seizures that went from almost daily grade 5 (forelimb and hindlimb tonic extension) seizures around P30–P45 to primarily grade 3 (forelimb and hindlimb clonus) seizures to primarily grade 2 (bilateral forelimb clonus, orofacial automatisms, jaw chomping, head clonus, occasional falling and rearing) seizures at P65–P75.