More supporting evidence of the interneuron hypothesis of Dravet syndrome arose this summer after researchers found decreased sodium currents and action potential firing in inhibitory patient-derived Dravet iPSC neurons, but normal function in excitatory Dravet neurons (Sun, et. al. 2016). (What is the inhibitory interneuron hypothesis? It is the prevailing belief that many SCN1A mutations render inhibitory interneurons defective compared to those without SCN1A mutations, and this decrease in inhibition results in the seizures seen in Dravet syndrome. This hypothesis helps explain why sodium channel blockers such as lamotrigine are found to exacerbate seizures in Dravet syndrome despite the fact that one would think that less active sodium ion channels would help reduce seizures.)


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