Neurological manifestations of the Mendelian‐inherited autoinflammatory syndromes

摘要: Autoinflammatory syndromes include an expanding list of conditions characterized by unprovoked recurrent attacks systemic inflammation with lack auto-antibodies or autoreactive T cells. Many these are genetic diseases a Mendelian inheritance. Neurological manifestations may be one the major clinical features and, in some cases, presenting symptom syndromes. The purpose this review is to increase recognition among neurologists Mendelian-inherited autoinflammatory highlighting neurological context other symptoms that should lead physicians suspect Most important for cryopyrin-associated periodic familial cold syndrome, Muckle-Wells syndrome and neonatal-onset multisystem inflammatory disease (called chronic infantile cutaneous articular Europe). We also less common involvement, including Mediterranean fever, tumor necrosis factor receptor-associated hyperimmunoglobulinemia D syndrome. Because often treatable irreversible damage prevented if they treated early, it recognize result neurologist, especially early childhood.