The Neurocritical Care Society′s Guidelines for the Evaluation and Management of Status Epilepti-cus defines status epilepticus as the epilepsy lasts for 5 minutes or longer,with continuous clinical and/or electrographic seizure activity or recurrent seizure activity without recovery (returning to baseline) between seizures.Nonconvulsive status epilepticus (NCSE) can be classified into with or without coma/stupor types.The diagnosis of NCSE needs continuous video electroencephalogram monitoring and the indications include:(1) altered mental status appear after seizures,acute brain injury or other unknown causes;(2)efficacy evaluation of seizure therapy is performed to confirm whether nonconvulsive seizures or NCSE is controlled monitoring for 24 to 48 hours;(3)identification of cerebral ischemia.Modified Salzburg consensus criteria for NCSE suggests that the diagnosis of NCSE needs the combination of clinical features and electroencephalograms.Suspected clinical features and signs of NCSE should last at least 10 minutes,and altered EEG of suspected NCSE should last at least 10 seconds.Benzodiazepines is the first choice of treatment in NCSE and antiepileptic drugs are selected if necessary.If the NCSE lasts longer than 60 minutes,anesthetics or other therapies may be administered.

Objective To evaluate clinical efficacy and electroencephalogram(EEG)changes quantitatively after the ketogenic diet (KD) by single sample entropy (SampEn) in the treatment of infantile spasms (IS),and to learn the quantitative relationship between the clinical efficacy and EEG.Methods Patients diagnosed as IS were enrolled and started KD in Shenzhen Children's Hospital from April 2010 to December 2013.The SampEn of EEG data in these patients before and after treatment with KD were analyzed.Patients were classified as seizure-free group and non-seizure-free group according to the therapeutic responsiveness to KD.The SampEn findings from two groups were compared to explore the effect of KD on EEG and its related factors.Results Among 35 patients,more than 2 months of treatment,10 cases were seizure free,25 cases still had seizures.SampEn was 0.377 ± 0.246 before treatment,and 0.725 ± 0.405 after treatment in all patients,there was significant difference (Z =-4.351,P =0.000).SampEn was 0.342 ± 0.277 before treatment,and 0.929 ± 0.379 after treatment in seizure free group,there was significant difference between 2 groups(Z =-3.371,P =0.001).While SampEn was 0.391 ± 0.237 before treatment,and 0.643 ± 0.393 after treatment in non-seizure free group,there was a significant difference between 2 groups(Z =-3.371,P =0.001).The mental and motor development was improved after KD with improvement rate were 56％ (14/25 cases) and 70％ (7/10 cases),respectively,but there was no statistical difference(P =0.704).Conclusions No matter seizures are controlled or not,KD can increase the complexity of electrical activity in the brain,which was more obvious in the seizure-free group.Intellectual and movement development can be improved in patients with KD.

In the use of antiepileptic drugs in patients with epilepsy,1/3 of the patients with epilepsy can not get effective control,showing resistance to antiepileptic drugs.Now the mechanism of drug resistance is considered to be caused by multiple factors work together.The concept and connotation between drug-resistant epilepsy and refractory epilepsy are different.Since 2010,the international union of antiepileptic proposeddrug-resistant epilepsy expert consensus,different medical centers use the new defition to study different populations and confirm that the new definition is effective and reliable.However,there are some controversier about the drug dosage,drug kinds,judge for determining evaluation index and the treatment judgement in that definition.

Although the ketogenic diet(KD) has originally been used to treat epilepsies,plenty of evidence about the efficacy to intractable epilepsy has not accumulated until recently.These epilepsy or epilepsy syndrome include:Dravet syndrome,Doose syndrome,infantile spasms,Angelman syndrome with epilepsy,tuberous sclerosis complex and focal cerebral dysplasia or other structural epilepsy.It is worth paying more attention to the efficacy of KD on cognition,language development and behavior problems.

High-frequency oscillations are used more and more widely on localizing epileptogenic zone and evaluating outcome as the surgery on epilepsy cases are increasing and the technique of electroencephalogram (EEG) are being improved.High-frequency oscillations are recorded by intracranial electrodes on most previous studies.However,recent reports describing high-frequency oscillations on scalp EEG recordings have created significant interest.Scalp high-frequency EEG provides a safe,non-invasive and simple method for us to study the special brain electrical activity.This paper summary the clinical applications and some questions on scalp high-frequency EEG.

West syndrome is one of the most common refractory epileptic syndromes with onsets mostly in infancy.The prognosis is generally poor.The morbidity rate reported in the literature is 0.2‰ to 0.5‰.Hormonal therapies(Adrenocorticotropic hormone and glucocorticosteroid)are recommended as preferred treatment options for West syndrome.It can effectively control spasms, ameliorate recognition impairment, and improve developmental outcomes.The incidence of side effects after hormonal therapy are high, and irritation, hypertension and infection are frequently reported.In this paper, advances in therapeutic mechanism, usage and dosage, short-term effectiveness, long-term effectiveness, prognosis and adverse effects of hormonal therapies are reviewed.

Child ; History, 20th Century ; History, 21st Century ; Humans ; Pediatrics ; history ; Pharmacology, Clinical ; history ; Societies, Medical ; history

Objective To explore the clinical and peripheral blood characteristics of childhood purulent meningitis complicated with subdural effusion. Methods A retrospective cohort chart review was performed. A total of 162 consecutive cases of purulent meningitis were identified in Shenzhen Children′s Hospital from January 2009 to De-cember 2013,all of them were divided into 2 groups according to whether complicating with subdural effusion, and the clinical characteristics of 2 groups were analyzed. The clinical characteristics were analyzed. The statistical data were determined by SPSS 17. 0 software. Results Forty-nine cases(30. 25%)of them were complicated with subdural effu-sion;and 37 cases (75. 5%)of them suffered from the disease in the first 10 days after the onset of the meningitis,and the incidence was 48. 84% (42 cases) in infancy. Fever,lethargy,seizures and bulging fontanelle were its common clinical signs,and there were no significant differences in the incidence between subdural effusion group and the control group(non-subdural effusion group),the white blood cell (WBC) count did not increase markedly and C-reactive pro-tein(CRP) was high in the subdural effusion group on the admission day(Z=1. 74,2. 10,all P<0. 05),but no differ-ence in platelet( PLT) . The PLT was high in the whole meningitis cohort,the ratio of cases whose PLT was higher than 300×109/L reached 93. 75%,but no difference between the 2 groups. There were many factors of WBC on admission, including age, cerebrospinal fluid culture results and the application of antibiotics before admission, but CRP was only affected by antibiotics. Conclusions Subdural effusion is common in infancy,and most occurred in the first 10 days af-ter onset of the meningitis. On admission day the CRP,was high, while WBC did not increase too much. If CRP rises for the second time it might predict the occurrence of subdural effusion in bacterial meningitis. The increased PLT may be considered as clinical feature of infectious diseases. The clinical implication of inflammatory pathophysiology in men-ingitis needs further investigation.

Objectives To investigate the clinical features of Guillain-Barré syndrome (GBS) in children from Shen-zhen. Methods The clinical manifestations, results of electrophysiological tests and prognosis of 28 GBS patients from July 2002 to July 2012 were retrospectively analysed. Results Of 28 children, 16(57.1%) had preceding acute upper respiratory infection for 3-14 days but no patient had acute gastroenteritis. One had received HBV vaccination in 2 weeks before the onset of GBS. The peak season for GBS is spring. According to the clinical presentations and the neurophysiological results 17 patients had demyelinating neuropathy, 5 acute motor axonal neuropathy, 2 acute motor sensory axonal neuropathy, 3 Miller-fisher syndrome, and 1 polyneuritis cranialis. 14 (50.0%) patients suffered from pain in limbs which is the most nota-ble symptom in the early stage. Intravenous immune globulin (IVIG) and steroids were given during the acute phases in the majority of the patients, and assisted ventilation was performed in 2 patients due to respiratory muscle paralysis. No diffe-rence was found in Hughes scores, average hospitalization durations, and the prognosis between patients with GBS variants patients and patients with classic GBS. Conclusions Children with GBS in Shenzhen area have different clinical features.

Objective:To study the clinical features and molecular genetic mechanisms of children with lissencephaly (LIS), as well as to analyze the relationship between genotypes and phenotypes of the disease.Methods:From October 2016 to December 2017, the clinical data and follow-ups of 21 LIS children were collected in the Department of Neurology, Shenzhen Children′s Hospital.Whole genome sequencing (WGS) was performed for genetic testing.Results:Among these 21 cases, 18 cases developed epilepsy (86%), and 3 cases were seizure free (14%). The onset age of children with epilepsy was relatively young, and 16 cases occurred within 1 year old (89%). Among these cases, 16 were pachygyria (76%), 3 cases were agyria combined with pachygyria (14%) and 2 cases were agyria (10%). Epileptic syndromes included 12 cases of West syndrome (67%), 2 cases of Ohtahara syndrome (11%), 2 cases of other epileptic encephalopathy (11%), and 2 cases of focal epilepsy (11%). Brain magnetic resonance imaging(MRI) demonstrated that most cases were pachygyria, among which diffuse pachygyria was more common (56%, 9/16 cases). The results of WGS: 13 pathogenic or likely pathogenic single nucleotide variants (SNV) and copy number variants (CNV) were detected.The total detection rate was 62%, of which 2 cases were frameshift, 1 case was nonsense and 1 case was missense variants of PAFAH1B1, 6 cases were chromosome 17p13.3 deletion syndrome, thus lea-ding to the whole gene deletion of PAFAH1B1, and 1 case was missense variant of DCX, frameshift variant of KIF2A, and missense variant of PIK3R2, respectively.Totally, 48% (10/21 cases) of the cases were variants or deletions of PAFAH1B1, which resulted in lissencephaly in the parietal-occipital region of the brain.Novel variants were PAFAH1B1: c.1067G>A, PAFAH1B1: c.897delT and KIF2A: c.2225delG. Conclusions:Most cases of LIS accompanied with epilepsy, in which West syndrome was relatively more common.Brain MRI showed that most cases were diffuse pachygyria.The variants and deletions of PAFAH1B1 was the main genetic cause of LIS.The identification of the novel variants expanded the genotypical spectrum of LIS.