A multicenter retrospective case series of 22 children (16 male, 6 female) aged 3-15 years (median 6.5 yrs) with prolonged or recurrent seizures occurring 2-14 days (median 5 days) after a febrile respiratory (59%) or nonspecific infection is reported from Kiel University, Germany. The early clinical course was biphasic in 68%, the acute period of high seizure activity lasting 1-12 weeks (median 3 weeks). Despite enteral and parenteral anticonvulsant drugs, barbiturate-induced coma (64%), pyridoxine (18%), folinic acid (9%), and adjuvant immunotherapy (46%) for suspected cerebral inflammation, the outcome was uniformly poor. CSF revealed 2-42 cells/mcl (median 5 cells/mcl) and no pathogens. Serological and PCR tests for pathogens were negative. Inborn errors of metabolism, mitochondrial disease, including Alpers syndrome (POLG disease), were excluded. EEG showed diffuse slowing (41%) or multifocal discharges (59%). MRI or CT during acute phase was normal in 41% and showed altered signal intensities in hippocampus or temporal lobe in 41%. Follow-up MRI showed brain atrophy in 10 (50%) of surviving patients. Brain biopsies performed in 7 children (32%) showed gliosis but no evidence of inflammation. The median follow-up was 5 years (range 1-14 years). Two children died, 8 had persistent impaired consciousness, 8 had refractory epilepsy, 2 had behavior disorders, and 2 recovered. The authors propose the term “febrile infection-related epilepsy syndrome” (FIRES). 
COMMENT. Under different terminologies, this syndrome has been described in various case series, and was first reported in Brain  as “acute encephalopathies of obscure origin” by Lyons, Dodge, and Adams. “Lyons-Dodge-Adams syndrome” would be a suitable eponym. Further research concerning the etiology suggested by the current authors includes immune and nonimmune mechanisms (eg.channelopathies, antibodies against ion channels and receptors, and infection-triggered alterations of receptor expression). A special issue on acute encephalopathy/encephalitis in childhood appears in Brain Dev June 2010.