Familial necrotic encephalopathies caused by mutations in RANBP2 and a number of other encephalopathies of cerebral organic acid origin are often precipitated by episodes of febrile and infectious illnesses, which in turn may elicit fasting and catabolic stress, and secondarily, excitotoxic, and proinflammatory mechanisms [33, 87, 88]. This evidence concerns the gene RANBP2 and Encephalopathy.