Anti-seizure medications (ASMs) are the most important and basic treatment for epilepsy, and are also the first choice for epilepsy treatment, but about one-third of patients have drug resistance. Perampanel (PER), as a novel third generation ASMs, inhibits the α-amino-3-hydroxy-5-methyl-4-isoxazole-propionic acid receptor (AMPAR) through non-competitive inhibition. AMPA plays an anti-seizure role. Since its approval in China in 2021, it has been mainly used in the treatment of focal epilepsy (with or without general seizure) as a single drug or addition, and has good safety, effectiveness and tolerability. Self-limited epilepsy with centrotemporal spikes (SeLECTS) is a common childhood focal epilepsy syndrome, accounting for 15% ~ 25% of various childhood epilepsies, PER has important advantages in clinical studies and has shown certain curative effect. At the same time, the overall effect of PER on cognition was neutral, with no systemic cognitive deterioration or improvement. In view of the relatively short application time of PER, which is still a new drug, this article will review the mechanism of action, dose, add-on (single drug) treatment, adverse events and, in order to provide clinicians with more drug choices and facilitate the individualized diagnosis and treatment of epilepsy.
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.