Objective To discuss the diagnosis of refractory epilepsy (RE) in children, and to study the association of the single nucleotide polymorphisms (SNPs) of muhidrug-resistance gene (MDR1) C3435T with pharmaco- resistant epilepsy. Methods Four hundred children with epilepsy were retrospectively or prospectively identified from multiple sources in our hospital in Shanghai and were followed-up for the occurrence of refractory epilepsy. The clinical features of RE regarding age at onset, gender, seizure type, electroencephalogram, neuroimaging, development of central nervous system, etiology and prognosis etcetera were investigated. DNA samples were obtained from 132 patients with epilepsy (70 RE and 62 responsive epilepsy) and 62 health children by DNA extraction kit. Genotype of the C3435T polymorphism was determined by DNA sequence analysis after traditional polymerase chain reaction. The frequency of genotypes and alleles among the three groups was compared by Chi-square test. Results Eighty-three (20.8%) out of total 400 patients were RE. Among them 65 (78.3%) patients failed at least 2 drugs in six months. Forty-two (50.6%) were administered at least 3 drugs on the last follow-up. Medical treatment showed remarkable effective in 6 (7.2%) RE patients, effective in 40 RE patients (48.2%). No effectiveness was seen in another 37 (44.6%) RE patients, however 25 out of 37 presented symptomatic alleviation. Significant difference in genotype (CC, CT, Tr) frequency was neither found between RE and responsive epilepsy patients nor between RE patients and healthy controls. No association between the C3435T polymorphism in the human MDR1 gene and refractory epilepsy was found by logistic analysis. Conclusions Refractory epilepsy could be diagnosed in 6 months after being treated with anti-epilepsy drugs (AEDs) in children with average attack once per month at least and failed more than 2 AEDs. Multiple AEDs were necessary for treatment. No association between the C3435T polymorphism in the human MDR1 gene and refractory epilepsy was found by logistic analysis in this study.

Objective To evaluate the diagnostic value of bedside video electroencephalogram (VEEG) in neonatal within 6 h after birth in diagnosing hypoxic-ischemic encephalopathy ( HIE) and the correlation of bedside VEEG results and early neural and behavioral development. Methods Neonatal severe asphyxia cases were collected and bedside VEEG and HIE were recorded and graduated. The sensitivity and specificity of different VEEG degrees within 6 h after birth were calculated in prognosing HIE degree. The sensitivity and specificity of abnormal VEEG performed within 6 h, the third day and the seventh day after birth, respectively, were compared in prognosing HIE. Neonatal behavior neurological assessment (NBNA) was performed at 7 to 14 days of age, EEG and general movements assessment (GMs), developmental screening test for child under six(DST) when patients were 3 months old, and EEG, Bayley scales of infant development(BSID) at 6 months old to analyze the correlation of bedside VEEG results and early neural and behavioral development. Results Forty-eight severe asphyxia neonatal were included, among which 12 severe asphyxia and 36 HIE, including 14 mild, 12 moderate and 10 severe HIE. There were nine normal and 39(81. 3%) abnormal VEEG including 16 mild, 11 moderate, five severe abnormal and seven inactive VEEG within 6 h after birth. There were 32(88. 9%) abnormal VEEG within 6 h after birth in 36 HIE patients. Significant positive correlation was found between VEEG within 6 h after birth and HIE (r= 0.849, P<0. 01). Severe abnormal and inactive EEG within 6 h after birth showed sensitivity of 100%, specificity of 94. 7% in predicting severe HIE. The sensitivity of VEEG testing at 6 h,3 and 7 d in predicting HIE were 88. 9%, 83. 9% and 28. 6% , correspondingly the specificity were 41.7%, 91. 7% and 100%, respectively. Nine patients with continually abnormal VEEG died in hospital. The NBNA scores of patients with moderate and severe abnormal EEG were significantly lower than those with normal EEG (both P<0. 01), the NBNA scores of patients with severe abnormal EEG were significantly lower than those with moderate abnormal EEG (P<0. 05). Thirty-five patients were followed up in the hospital at 3 months old, and 32 patients had DST >85 and three had DST between 70 and 84 with abnormal EEG. GMs assessment of one of the three patients showed absence of fidget movements, cuing a chance of cerebral palsy. Fourteen patients were followed up in hospital at 6 months old, and seven of them had abnormal EEG, four had abnormal BSID with abnormal EEG. Conclusions VEEG within 6 h after birth shows high sensitivity and specificity in prognosing HIE, and much relates to short-term neural and behavior development.

Objective To characterize contemporary electrographic neonatal seizures by video electroencephalogram (VEEG) and to assess the value and the limitations of two-channel (C3-C4/T3-T4) amplitude-integrated electroencephalogram (aEEG) plus original EEG signals used to diagnose neonatal seizure with video EEG as a golden standard.Methods Sixty-six neonates admitted to Children's Hospital of Fudan University from January 2011 to July 2011 with clinical or suspected clinical seizure were investigated and bedside VEEG were recorded for more than 3 hours.VEEG signals were transformed into three kinds of aEEG signals by Galileo NT PMS software:one-channel aEEG (C3-C4),one-channel aEEG (C3-C4) plus original EEG,two-channel aEEG (C3-C4/T3-T4) plus original EEG.Electrical seizure activity on VEEG was signed out with respect to its occurrence,duration and localization of seizure onset; while aEEG seizure was recorded only with its occurrence.The relationship between aEEG and VEEG was analyzed by Spearman analysis.The value and the limitations of aEEG to diagnose neonatal seizure were evaluated by sensitivity,specificity,positive predictive value and negative predictive value.Results A total of 62 traces were suitable for analysis.(1) VEEG showed 39 seizure activities,among which 8 status epilepticus; and the rest 31 neonates had 352 non-status epilepticus electrical seizures,79.3％ (279/352) of which occurred over the centrotemporal region.(2) Eight cases with status epilepticus on VEEG were all diagnosed as status epilepticus on aEEG.For non-status epilepticus electrical seizures,the sensitivity of aEEG for detection of electrical seizures was as followed:49.1％ (173/352) for one-channel aEEG,54.5 ％ (192/352) for one-channel aEEG plus original EEG,81.2％ (286/353) for two-channel aEEG plus original EEG.Results from one-channel aEEG,one-channel aEEG plus original EEG and two-channel aEEG plus original EEG were all related to VEEG (ρ =0.790,0.907 and 0.953,respectively,P＜ 0.01).(3) Sensitivity of seizure detection was 66.7％ (26/39,95％ CI:0.62-0.81) for one-channel aEEG,74.4％(29/39,95％ CI:0.78-0.96) for one-channel aEEG(C3-C4) plus original EEG and 89.7％ (35/39,95％ CI:0.89-1.00) for two-channel aEEG(C3-C4/T3-T4) plus original EEG.Conclusions VEEG might help aEEG in diagnosis of neonatal seizure.two-channel aEEG (C3-C4/T3-T4) plus original EEG could significantly increase the sensitivity of neonatal seizures indentification.