Neurological manifestations in autoinflammatory diseases

Autoinflammatory diseases (AIDs) are a distinct group of diseases characterised by a dysregulation of the innate immune response leading to systemic inflammation. The clinical spectrum of these conditions is extremely variable and possibly every system and tissue can be involved, including the central nervous system (CNS). Indeed, neurological manifestations may dominate the clinical picture from disease onset in some rare conditions. However, the involvement of the CNS in AIDs is not a disease in itself, but represents a rare complication which is consequent to a systemic or local immune response, mainly involving cells of the innate immunity. This review will describe neurological manifestations associated with AIDs, including: chronic aseptic meningitis and brain atrophy, sensorineural hearing loss, early-onset haemorrhagic and ischaemic strokes, mental retardation, cerebellitis, and ataxia, and severe encephalopathy with brain calcifications.