Febrile infectious-related epilepsy syndrome (FIRES)

Clinical history: A 14-year-old girl with normal psychomotor development was admitted to our hospital with convulsive status epilepticus. Focal seizures rapidly evolving into status epilepticus had appeared, without fever, about 10 days after a non-specific upper respiratory tract infection. Progressive spread of seizures arising independently from either side of the body produced secondary generalization. Family history No family history of neuropsychiatric conditions. Neurological examination Patient was intubated and markedly sedated. GCS: 9. Special studies Several seizures starting independently from either the left or right frontal temporal region were recorded during video-EEG. Brain MRI and CSF study were unrevealing. Follow-up: Status epilepticus was refractory to multiple treatments. Burst-suppression coma was achieved using general anesthesia in the intensive care unit. After status epilepticus subsided, the patient experienced a 2 month seizure-free period, after which focal epilepsy developed, with sporadic secondarily generalized seizures. In the following 4 years an average of two seizures per year occurred. Neuropsychological testing revealed impaired executive functions.