P0001 / #308: FEBRILE INFECTION-RELATED EPILEPSY SYNDROME (FIRES) TREATED WITH HUMAN RECOMBINAT INTERLEUKIN 1 RECEPTOR ANTAGONIST-ANAKINRA

The expanding spectrum of febrile infection-related epilepsy syndrome (FIRES)

In recent years, a number of phenomena included in the group of probable epileptic encephalopathies of autoimmune origin have been described. Their common denominator is abrupt onset, usually after a self-limited febrile illness, and a clinical picture of repetitive seizures or difficult-to-control status epilepticus. These symptoms occur mostly in children and have received a wide range of nam...

Hippocampal Changes in Febrile Infection-Related Epilepsy Syndrome (FIRES)

BACKGROUND Febrile seizures are the most common seizure disorder in childhood, associated with a significant rise in body temperature. However, post-infectious refractory afebrile form of seizures in previously healthy children is being increasingly recognized in around the world, which evolves into a chronic refractory form of epilepsy. The term 'Febrile infection-related epilepsy syndrome' (F...

Interleukin-1 receptor antagonist (anakinra) for Schnitzler syndrome.

Schnitzler syndrome is a rare autoinflammatory disease, which is defined by the presence of two major criteria: chronic urticaria and monoclonal immunoglobulin M (IgM) or immunoglobulin G gammopathy, in combination with at least two additional minor criteria: recurrent fever, leukocytosis and/or elevated C-reactive protein (CRP), objective signs of abnormal bone remodelling and a neutrophilic i...

Recurrent poliserositis succesfully treated with IL-1 receptor antagonist Anakinra

Background Recurrent pericarditis is known to be a clinical feature in numerous inflammatory and infectious diseases. However, in the majority of cases, recurrent pericarditis appears to be idiopathic. The optimal regimen for preventing recurrence is not established; treatment modalities include non-steroidal anti-inflammatory drugs (NSAIDs), corticosteroids, colchicine and other immunomodulato...

Dermal interleukin-1 expression and effective and long-lasting therapy with interleukin-1 receptor antagonist anakinra in Schnitzler syndrome.