Sodium selenate treatment improves symptoms and seizure susceptibility in a malin-deficient mouse model of Lafora disease

SummaryObjective To search for new therapies aimed at ameliorating the neurologic symptoms and epilepsy developing in patients with Lafora disease. Methods Lafora disease is caused by loss-of-function mutations in either the EPM2A or EPM2B genes. Epm2a−/− and Epm2b−/− mice display neurologic and behavioral abnormalities similar to those found in patients. Selenium is a potent antioxidant and its deficiency has been related to the development of certain diseases, including epilepsy. In this study, we investigated whether sodium selenate treatment improved the neurologic alterations and the hyperexcitability present in the Epm2b−/− mouse model. Results Sodium selenate ameliorates some of the motor and memory deficits and the sensitivity observed with pentylenetetrazol (PTZ) treatments in Epm2b−/− mice. Neuronal degeneration and gliosis were also diminished after sodium selenate treatment. Significance Sodium selenate could be beneficial for ameliorating some symptoms that present in patients with Lafora disease.