RT Journal Article SR Electronic T1 Autoinflammatory syndromes in neurology: when our first line of defence misbehaves JF Practical Neurology JO Pract Neurol FD BMJ Publishing Group Ltd SP 145 OP 153 DO 10.1136/practneurol-2021-003031 VO 22 IS 2 A1 William K Diprose A1 Anthony Jordan A1 Neil E Anderson YR 2022 UL http://pn.bmj.com/content/22/2/145.abstract AB Autoinflammatory syndromes result from 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.