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Article: A functional null mutation of SCN1B in a patient with Dravet syndrome.

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Patino GA; Claes LR; Lopez-Santiago LF; Slat EA; Dondeti RS; Chen C; O'Malley HA; Gray CB; Miyazaki H; Nukina N; Oyama F; De Jonghe P; Isom LL
J. Neurosci., 2009


Table 4.

Seizure parameters in Scn1b+/+ and Scn1b+/− mice

Parameter Pentylenetetrazole dose (mg/kg) Scn1b+/+ Scn1b+/−
Time to myoclonic jerk or seizure of higher severity (min)a 60 2.52 ± 0.31 5.79 ± 1.36
Highest seizure levelbc 60 6 (3.68–6.56) 6 (3.03–6.46)
Time to death (min) 80 4.92 ± 1.67 11.36 ± 3.26
nd 8 8

  • ↵aData are mean ± SEM.

  • ↵bData are median (95% confidence interval).

  • ↵cAs measured using the modified Racine scale: 0, no seizure; 1, staring/unresponsive; 2, focal clonic convulsion (including head nod, twitch, myoclonic jerk, backing); 3, forelimb clonus (tonic–clonic seizure); 4, rearing; 5, loss of posture (including jumping, rearing, and falling); 6, status epilepticus and death.

  • ↵dEight mice of each genotype used for each dose.


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