ObjectiveAccording to the diagnosis and treatment analysis of 5 children with Febrile infection-related epilepsy syndrome (FIRES) and literature review, to explore the therapeutic effect of Ketogenic diet (KD).MethodsA retrospective analysis of the clinical status of 5 children with KD treatment of FIRES admitted to the Department of Pediatric Neurology, West China Second Hospital of Sichuan University from August 2016 to September 2019, combined with literature data, summarized their disease characteristics, prognosis and KD treatment effects.ResultsThe 5 cases of FIRES children were (5.8±2.0) years old and had a male to female ratio of 2∶3. They were all induced by fever followed by a status epilepticus that was difficult to control with drugs. The interval between fever and first seizure was 4 to 7 days, and the prodromal symptoms were higher respiratory tract infections, dizziness, vomiting, fatigue, listlessness, loss of appetite, etc., convulsions manifested as focal or focal secondary systemic or general seizures, EEG showed slowing background rhythm, changes in multifocal epileptic discharge, early course of disease The cranial imaging examination was basically normal. As the course of the disease progressed, changes in brain atrophy gradually appeared, and abnormal signal shadows were seen in the forehead, parietal, occipital lobe, and periventricular. A variety of anti-epileptic drugs, hormones, gamma globulin, plasma exchange and other treatments have poor therapeutic effects and severe cognitive impairment. The KD treatment started to take effect within 2 weeks, and the convulsions were reduced. One case was completely controlled, and the cognitive function basically returned to normal with only mild learning disabilities; the convulsions were reduced by more than 50% in 2 cases, leaving mild to moderate cognitive impairment, The other 2 cases had poor long-term treatment effects, left intractable seizures and severe mental retardation.ConclusionFIRES is a serious epileptic encephalopathy, most of which leave severe cognitive impairment and refractory seizures. Drug therapy and prognosis are poor. KD treatment is beneficial to control seizures in children with FIRES in the acute stage.
ObjectiveTo improve the clinicians’ understanding and treatment level of electrical status epilepticus during sleep by analyzing the clinical data of patients with ESES retrospectively.MethodsWe collected 20 cases from 2018 to 2019 diagnosed in our hospital by clinical and 24-hour video EEG confirmed and analyzed the clinical manifestations, EEG features, seizure type, classification of epilepsy syndrome as well as the imaging findings.ResultsAmong the 20 patients, there were 12 males and 8 females, the mean age was (10.96±2.68) years old, and the first onset age was (8.90±1.93) years old. Epilepsy seizure as the first symptom is most common, the EEG showed a broad or localized sustained discharge, generalized tonic clonic seizure is the main form of seizure type in most patients, ECSWS is the most prominent in the epilepsy syndrome, for small lesion through MR can be found early.ConclusionMost patients have partial or generalized seizures, which have a range of cognitive impairment, mental decline or other issues. It is important to improve the cognitive function, behavior, neuropsychology of patients by long term video EEG monitoring in the early stage.