Subacute (days/weeks) onset of severe dystonia in the context of bilateral striatal necrosis (symmetrical signal change in the caudate and putamen – but not thalami - associated with swelling and later shrinkage), manifesting at a few months of age or in later childhood, often proceeded (possibly triggered) by a notable - but not major - infectious episode, almost exclusively due to ADAR1 mutations. Here, ADAR is linked to Dystonia.