Clinical features and surgical treatment of epilepsy after viral encephalitis

Encephalitis is an acute inflammatory process of the brain parenchyma,which is often caused by viral infection.It is an vital cause of acute symptomatic seizures and subsequent epilepsy.The incidence of unprovoked and recurrent seizures after previous infections of the central nervous system is high and accounts for1%~5%of the cases of epilepsy.Viral encephalitis(VE)is directly caused by viral infection.The occurrence of seizures after VE is associated with poor prognosis.In survivors of VE,among other neurological sequelae,the risk of developing epilepsy is increased10-fold.The risk of severe neurological sequelae after VE is particularly high in very young children.Studies on seizure occurrence,possible underlying mechanisms,clinical characteristics,and clinical treatment(especially surgical treatment)of VE have yielded only limited detailed data.We reviewed the most recent literature on the clinical features and surgical treatment of post-VE epilepsy.

Long-term follow-up of vagus nerve stimulation in drug-resistant KCNT1-related epilepsy: a case presentation

Background The KCNT1 gene encodes a Na+-activated K+channel.Gain-of-function mutations of KCNT1 lead to autosomal dominant sleep-related hypermotor epilepsy,early-onset epileptic encephalopathy,focal epilepsy and other epileptic encephalopathies.In this paper,we report a boy carrying a KCNT1 gene mutation,who presented with drug-resistant focal-onset seizures.He had decreased seizure frequency and improvement of background changes in electroencephalography(EEG)after vagus nerve stimulation(VNS).Case presentation The case was a nonverbal 9-year-old male who presented with drug-resistant focal-onset seizures since age 3 and had underwent VNS therapy for 2 years.He had hypermotor symptoms,automatism and bilateral asymmetric tonic seizures with cognitive decline and aphasis from age 3.The patient had a variety of seizure types that only occurred at night.The most common seizure type was automatisms,and ictal video EEG showed high-amplitude delta waves,followed by a fast rhythmic sharp activity in the mesial frontal and bitemporal regions.The patient was diagnosed with KCNT1-related epilepsy,epileptic encephalopathy and cognitive disorder.He was refractory to multiple anti-seizure medicines(ASM)and ketogenic diet.After VNS treatment at age 7,the frequency of seizures was reduced significantly and EEG was improved in background slowing.Conclusions Children with KCNT1-related epilepsy usually have early onset of disease,are nonverbal,and are refractory to ASM.This boy with drug-resistant KCNT1-related epilepsy showed significantly reduced seizure frequency after VNS.This report may provide reference for management of cases of KCNT1-related epilepsy.

Consensus on pediatric epilepsy Che up surgery for young children:an investigation by the China Association Against Epilepsy task force on epilepsy surgery

Researchers have widely acknowledged the therapeutic value of epilepsy surgery for drug-resistant epilepsy.None-theless,there is a substantial gap in the surgical treatment for appropriate candidates owing to several factors,particularly in the population of young children.To standardize the protocols of preoperative evaluation and sur-gery of young children for epilepsy surgery,the China Association Against Epilepsy has appointed an expert task force to standardize the protocols of preoperative evaluation and surgery in pediatric epilepsy patients.It adopted the modifed Delphi method and performed two rounds of surveys through an anonymous inquiry among 75 experts from four subgroups including pediatric neurologists,epileptologists,pediatric epilepsy surgeons,and functional neurosurgeons.The survey contents contained:(1)the participants,comprising children aged≤6 years;(2)adopted DRE definition proposed by the International League Against Epilepsy in 2010;and(3)investigated epilepsy surgery,principally referring to curative epilepsy surgeries.The neuromodulation therapies were excluded because of the differences in treatment mechanisms from the above-mentioned surgeries.According to the Delphi process,a con-sensus was achieved for most aspects by incorporating two rounds of surveys including preoperative assessment,sur-gical strategies and techniques,and perioperative and long-term postoperative management,despite controversial opinions on certain items.We hope the results of this consensus will improve the level of surgical treatment and man-agement of intractable epilepsy in young children.

MPDZ variants associated with epilepsies and/or febrile seizures and the individualized genotype-phenotype correlation

The multiple PDZ domain crumbs cell polarity complex component gene(MPDZ;MIM:603785),is highly expressed in the brain across the whole lifespan.It encodes the multiple PDZ domain protein,which is a member of the NMDAR signaling complex that may play a role in the control of AMPAR potentiation and synaptic plasticity in excitatory synapses."Previously,MPDZ variants have been demonstrated to be associated with autosomal recessive congenital hydrocephalus-2(HYC2;MIM:615219)which is commonly complicated by brain abnormalities and developmental delay.Seizures were reported in only one case.The association between MPDz and epilepsy requires clarification.

Preoperative evaluation and surgical strategy for epileptic spasms in children

Epileptic spasm(ES)is one of the most common types of seizures in children.It is primarily characterized by brief axial contractions lasting less than 2 s and recurring in short clusters.It usually occurs in children of 3 to 12 months of age,although it can also occur after the age of 1 year.In general,children with ES develop other symptoms of epilepsy,such as tonic,tonic-clonic,or focal seizures,after 3 to 5 years of age.ES in children is often damaging and usually results in developmental regression.First-line treatments for spasm seizures include adrenocorticotropic hormone(ACTH)and vigabatrin.However,many patients fail to respond to these medications,and continued to have spasms associated with progressive neurodevelopmental degeneration.Therefore,it is important to consider whether children with drug resistance meet surgical indications to consider surgical treatment in such conditions.In this study,we reviewed and summarized the importance of preoperative evaluation in order to provide surgical options for treatment of children with ES.