Abstracts

Rationale: Focal cortical dysplasia (FCD) is the most common etiology of symptomatic epilepsies in children. Febrile infection related epilepsy syndrome (FIRES) is a catastrophic epileptic encephalopathy with a yet undefined etiology in childhood, which comprises a small minority of all patients with status epilepticus (SE). FCD and FIRSE are different entities in pathophysiology of epileptogenesis, but both of them show medically refractory epilepsies. Here we report that cases exhibited clinical manifestations of FIRSE at the seizure onset, and diagnosed as FCD during the period of follow-up.Methods: Three cases, who manifested clinical features of FIRSE after an episode of upper respiratory tract infection and finally diagnosed either with pathology-proven FCD or an evident FCD showing on the brain MRI, were enrolled. All of them fulfilled the diagnostic criteria of FIRSE with clinical seizure types of mainly partial or secondarily generalized at seizure onset. The partial seizures are often complex partial seizures, at times with facial myoclonia. Prolonged hospitalization and refractory response to medical treatments are the characteristics. We excluded patients with definitive viral encephalitis, and/or immune related encephalitis. Clinical manifestations, seizure types, EEG findings, and brain MRI features were analyzed.Results: Case 1 exhibited clinical features of FIRSE at age of 9 years with the cluster seizure duration of 2 months. Focal spikes became prominent during the period of follow-up. Type IIa FCD was diagnosed after seven years of the first seizure, and he achieved Engel III of seizure outcome after epilepsy surgery. Case 2 had clinical features of FIRSE complicated with arrhythmia at age of 10 years with the cluster seizure duration of one month. He was diagnosed with FCD type Ib after epilepsy surgery and achieved Engel II of seizure outcome. Case 3 showed clinical pictures of FIRSE at age of 2 years with the cluster seizure duration of 14 days. Her brain MRI showed an evident thick cortex over the right frontal area. Her seizures were well control by antiepileptic drugs and she did not receive epilepsy surgery.Conclusions: In cases who were diagnosed with FIRSE, but presented with focal epileptiform discharges on EEG during the period of follow-up, FCD should be considered as one of differential diagnoses.