Neuroinflammation and progressive myoclonus epilepsies: from basic science to therapeutic opportunities

7 páginas, 2 tablas, 1 figuraProgressive myoclonus epilepsies (PMEs) are a group of genetic neurological disorders characterised by the occurrence of epileptic seizures, myoclonus and progressive neurological deterioration including cerebellar involvement and dementia. The primary cause of PMEs is variable and alterations in the corresponding mutated genes determine the progression and severity of the disease. In most cases, they lead to the death of the patient after a period of prolonged disability. PMEs also share poor information on the pathophysiological bases and the lack of a specific treatment. Recent reports suggest that neuroinflammation is a common trait under all these conditions. Here, we review similarities and differences in neuroinflammatory response in several PMEs and discuss the window of opportunity of using anti-inflammatory drugs in the treatment of several of these conditions.This work was supported by grants from the Spanish Ministry of Economy and Competitiveness SAF2017-83151-R (to PS) and SAF2014-59594-R (to JMS), a grant from Fundación Ramón Areces (CIVP18A3935) (to PS) and a grant from the National Institute of Health(NIH-NINDS) P01NS097197, which established the Lafora Epilepsy Cure Initiative (LECI) (to PS and JMS).Peer reviewe