Circadian variation of absence seizures in an animal model of human leukodystrophy (h-abc)

Abstract Introduction 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. Methods We used 16 male taiep rats at 6 and 9 months of age. All rats were kept in standard conditions with a 12/12 light-dark cycle (lights on at 0700) and free access to rodent food pellets and purified water and were implanted for EEG, EMG and EOG recordings to characterize the frequency and duration of SWD. All procedures followed the NIH rules, and the protocol was approved by BUAP-IACUC. Results The number of SWD are higher in male rats at 9 with respect to 6 months of age (P≤0.05), and also had higher incidence during the light phase with respect to the dark phase (P≤0.05). The duration of SWD had also a greater duration during the light period than the dark phase (P≤0.05). Conclusion Our results showed that SWD has higher incidence of SWD and of longer duration during the light period when the rats had more sleep suggesting that the neurotransmitters that are released during sleep facilitate the discharge in the thalamo-cortical circuit that support SWD. Support (If Any) Partially supported by PRONACES-CONACYT grant 194171, and VIEP-BUAP 2021 to CA in Neuroendocrinología. JMI is fellowship from CONACYT No. 735813.