Neuronal Necrosis Due to a Lethal Epileptic Syndrome Affecting FVB Strain β-Actin Luciferase Transgenic Mice

Abstract

The FVB is the most often inbred mouse strain used in transgenic research. This strain is recognised to be affected with a disease known as FVB Lethal Epileptic Syndrome (FLES). A case series of FVB mouse lethal epileptic syndrome in a cohort of commercially procured β actin luciferase transgenic FVB mice was diagnosed based on the clinical signs, post-mortem and histopathologic findings and immunohistochemical analysis of glutamate receptors. Observed clinical signs included non-responsiveness, hunched posture, piloerection. Histopathologic lesions included moderate to severe acute neuronal necrosis affecting predominantly the cerebral cortex, thalamus and hippocampus with hepatic coagulative necrosis. Glutamate receptors showed mild to marked positive expression within the affected neurons. Glutamate receptor may have a potential role in the pathogenesis of FLES.