Lee HF, et al. - An exploration was carried out of the long-term neurological outcomes and chronological changes of brain magnetic resonance imaging (MRI) in patients with febrile infection-related epilepsy syndrome (FIRES). At the acute stage of disease course, liver function impairment, skin rash, and arrhythmia were often noted during hospitalization for FIRES. Data hinted towards poorer neurological outcomes, via the higher grade of periventricular white matter lesions.

Methods

• Researchers enrolled 29 patients diagnosed with FIRES from December 2000 to May 2016.
• They elucidated the demographic distribution, clinical manifestations, neuroimaging findings, and treatment methodology.
• Data analysis was carried out of the follow-up clinical outcomes and chronological evolution of neuroimaging findings.

Results

• It was determined that the median age of disease onset was 8.9 years.
• The median duration of hospitalization was reported to be 87 days.
• Complications such as skin rash, liver function impairment, and arrhythmia were experienced by more than 50% of patients during the period of hospitalization.
• In the initial study, abnormal findings were revealed in 38% of neuroimaging studies and in 87% of the follow-up brain MRI.
• More extensive lesions possibly related to a poorer clinical outcome as indicated by the focal abnormal signal changes over the periventricular white matter.
• In 23 patients, the median duration of follow-up was discovered to be 5 years.
• Residual and/or refractory epilepsy was disclosed among 87% of the patients.
• Herein, 26% of patients had normal intelligence quotient, 26% had learning disability and mild to moderate mental retardation, and 48% had severe mental retardation or vegetative status in terms of the cognitive function.
• It was discovered that the mortality rate at acute stage was 10%, and that at chronic stage was 13%.