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Published on: January 29, 2018
Sleep-related epilepsy in the A/J mouse
Kingman P Strohl1, Laura Gallaugher, Audrey Lynn
1Department of Medicine, Case Western Reserve University, Cleveland OH, USA. kps@po.cwru.edu
Sleep
|March 1, 2007
Summary
Epileptic seizures during sleep were identified in A/J JAX mice, a novel animal model. This sleep-related epilepsy shows oligogenic inheritance, linked to chromosome 4 loci.
Area of Science:
- Neuroscience
- Genetics
- Epilepsy Research
Background:
- Seizures during sleep are common in humans but underrepresented in animal epilepsy models.
- Previous observations of spontaneous spike-and-wave activity in A/J JAX mice suggested a potential genetic basis for sleep-related epilepsy.
Purpose of the Study:
- To describe the inheritance pattern of spontaneous epileptic activity during sleep in A/J JAX mice.
- To identify genetic loci associated with this sleep-related epilepsy phenotype.
Main Methods:
- Electroencephalography (EEG) and electromyography (EMG) were recorded in A/J, C57BI/6J (B6), and chromosome-substitution mouse strains.
- Anti-epileptic drugs (ethosuximide, diazepam) were administered to assess seizure inhibition.
- Intercrosses and linkage analysis were performed to map genetic loci.
Main Results:
- Spike-discharge patterns characteristic of epilepsy were observed in A/J JAX mice during slow-wave sleep.
- Ethosuximide and diazepam effectively reduced or eliminated seizure activity.
- Linkage analysis in an intercross population identified significant association with loci on chromosome 4.
Conclusions:
- A distinct sleep-related epilepsy phenotype was characterized in A/J JAX mice.
- The inheritance pattern suggests an oligogenic model.
- Genetic linkage to chromosome 4 provides a basis for further investigation into the underlying genes.
