TY - JOUR T1 - Autoinflammatory syndromes in neurology: when our first line of defence misbehaves JF - Practical Neurology JO - Pract Neurol DO - 10.1136/practneurol-2021-003031 SP - practneurol-2021-003031 AU - William K Diprose AU - Anthony Jordan AU - Neil E Anderson Y1 - 2021/10/01 UR - http://pn.bmj.com/content/early/2021/10/01/practneurol-2021-003031.abstract N2 - Autoinflammatory syndromes result in a defective innate immune system. They are characterised by unexplained fever and systemic inflammation involving the skin, muscle, joints, serosa and eyes, along with elevated acute phase reactants. Autoinflammatory syndromes are increasingly recognised as a cause of neurological disease with a diverse range of manifestations. Corticosteroids, colchicine and targeted therapies are effective if started early, and hence the importance of recognising these syndromes. Here, we review the neurological features of specific autoinflammatory syndromes and our approach (as adult neurologists) to their diagnosis. ER -