Which are the symptoms of Febrile infection-related epilepsy syndrome - FIRES?

Symptoms of Febrile Infection-Related Epilepsy Syndrome (FIRES)

Febrile Infection-Related Epilepsy Syndrome (FIRES) is a rare and severe form of epilepsy that typically affects previously healthy children and young adults. It is characterized by the sudden onset of prolonged 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. Understanding the symptoms of FIRES is crucial for early diagnosis and appropriate management.

1. Febrile Illness:

The first symptom of FIRES is the presence of a febrile illness, which is a fever caused by an infection. The infection can be viral, bacterial, or even an unknown source. The fever usually precedes the onset of seizures and can be accompanied by other symptoms such as cough, sore throat, runny nose, or gastrointestinal disturbances.

2. Acute Seizures:

The hallmark symptom of FIRES is the sudden onset of acute seizures. These seizures are often prolonged and difficult to control, lasting for hours or even days. They can be focal or generalized, meaning they can affect only one part of the brain or involve the entire brain. The seizures may manifest as convulsions, loss of consciousness, repetitive movements, or altered behavior.

3. Status Epilepticus:

FIRES is associated with a condition called status epilepticus, which refers to continuous or recurrent seizures without recovery in between. Status epilepticus is a medical emergency and requires immediate intervention to prevent further complications. In FIRES, status epilepticus can persist for an extended period, leading to significant neurological damage.

4. Cognitive Decline:

Following the acute phase of seizures, individuals with FIRES often experience a rapid and severe decline in cognitive function. This decline can manifest as memory problems, difficulties with attention and concentration, language impairments, and overall intellectual regression. The cognitive decline can be profound and may result in long-term disabilities.

5. Behavioral Changes:

FIRES can also cause significant behavioral changes in affected individuals. They may become irritable, agitated, or exhibit aggressive behavior. Some individuals may develop psychiatric symptoms such as anxiety, depression, or psychosis. These behavioral changes can further impact the quality of life for both the affected individual and their caregivers.

6. Motor Abnormalities:

In addition to seizures, FIRES can lead to various motor abnormalities. These may include muscle weakness, abnormal movements, coordination difficulties, or even paralysis in severe cases. The motor abnormalities can significantly impair mobility and independence.

7. Autonomic Dysfunction:

Autonomic dysfunction refers to the impairment of the autonomic nervous system, which controls involuntary bodily functions such as heart rate, blood pressure, and digestion. In FIRES, autonomic dysfunction can manifest as fluctuations in heart rate, blood pressure instability, abnormal sweating, gastrointestinal disturbances, or temperature dysregulation.

8. Electroencephalogram (EEG) Findings:

EEG is a diagnostic test that measures the electrical activity of the brain. In FIRES, EEG findings often show a characteristic pattern called "burst suppression." This pattern is characterized by bursts of electrical activity followed by periods of electrical silence. The burst suppression pattern on EEG is highly suggestive of FIRES and helps differentiate it from other forms of epilepsy.

9. Neurological Deficits:

As a result of the prolonged seizures and associated brain damage, individuals with FIRES may develop various neurological deficits. These deficits can include motor impairments, speech and language difficulties, sensory abnormalities, or cognitive disabilities. The extent and severity of neurological deficits can vary among individuals.

Early recognition and prompt treatment of FIRES are crucial to improve outcomes and minimize long-term complications. If you suspect FIRES in yourself or a loved one, it is important to seek immediate medical attention. FIRES is a complex condition that requires a multidisciplinary approach involving neurologists, epileptologists, infectious disease specialists, and other healthcare professionals.