This is a genetically determined encephalopathy caused by mutations in TREX1 genes encoding RNAs involved in removing RNA, leading to the accumulation of endogenous RNA, triggering Toll-like receptor-dependent interferon-α production in the brain with the resultant activation of neurotoxic lymphocytes and immune system in addition to the inhibition of angiogenesis (102, 103). Here, TREX1 is linked to Encephalopathy.