Febrile infection-related epilepsy syndrome (FIRES), also known as acute encephalitis with refractory, repetitive seizures (AERRPS), is a rare and severe neurological disorder that primarily affects children and young adults. It is characterized by the sudden onset of prolonged and frequent seizures following a febrile illness, such as an infection.
FIRES is a devastating condition that can have long-lasting effects on the affected individuals and their families. The exact cause of FIRES is still unknown, but it is believed to be triggered by an abnormal immune response to an infection, leading to inflammation in the brain. This inflammation disrupts the normal functioning of the brain, resulting in the development of seizures.
The hallmark feature of FIRES is the refractory nature of the seizures, meaning they do not respond well to standard anti-seizure medications. These seizures can be prolonged, frequent, and difficult to control, often requiring intensive care and continuous administration of multiple anti-seizure drugs. The seizures can cause significant neurological damage and cognitive impairment.
Management of FIRES involves a multidisciplinary approach, including neurologists, intensivists, and other specialists. Treatment aims to control seizures, manage complications, and provide supportive care. Various anti-seizure medications, immunotherapies, and ketogenic diet may be utilized in an attempt to gain seizure control.
Prognosis for individuals with FIRES is variable, with some experiencing a gradual improvement in seizures over time, while others may continue to have seizures and face long-term cognitive and behavioral challenges. Research into the underlying mechanisms and potential treatments for FIRES is ongoing, as the condition remains poorly understood.
FIRES is a rare and severe neurological disorder characterized by the sudden onset of prolonged and frequent seizures following a febrile illness. It is a challenging condition to manage, often requiring intensive care and multiple anti-seizure medications. The long-term prognosis varies, and individuals affected by FIRES may experience ongoing cognitive and behavioral difficulties. Ongoing research is crucial to better understand the condition and develop more effective treatments.