0134 Circadian variation of absence seizures in an animal model of human leukodystrophy (H-ABC)

Hypomyelination with atrophy of the basal ganglia and cerebellum (H-ABC) is a human leukodystrophy is due to a mutation in the tubulin b 4a (TUBB4A) and the taiep rats is the only available model of this human disease with similar signs in the magnetic resonance imaging and a mutation in the TUBB4 that induced an accumulation of microtubules in the cytoplasm and its processes in the oligodendrocytes. Taiep rats had spike-wave discharges (SWD) that are similar to absence epilepsy with a progressive increase with the age of the subjects. The aim of this study is to analyze the circadian distribution of SWD on male taiep.

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