Simultaneously recorded intracranial and scalp high frequency oscillations help identify patients with poor postsurgical seizure outcome

ObjectiveHigh frequency oscillations (HFO) between 80–500 Hz are markers of epileptic areas in intracranial and maybe also scalp EEG. We investigate simultaneous recordings of scalp and intracranial EEG and hypothesize that scalp HFOs provide important additional clinical information in the presurgical setting.MethodsSpikes and HFOs were visually identified in all intracranial scalp EEG channels. Analysis of correlation of event location between intracranial and scalp EEG as well as relationship between events and the SOZ and zone of surgical removal was performed.Results24 patients could be included, 23 showed spikes and 19 HFOs on scalp recordings. In 15/19 patients highest scalp HFO rate was located over the implantation side, with 13 patients having the highest scalp and intracranial HFO rate over the same region. 17 patients underwent surgery, 7 became seizure free. Patients with poor post-operative outcome showed significantly more regions with HFO than those with seizure free outcome.ConclusionsScalp HFOs are mostly located over the SOZ. Widespread scalp HFOs are indicative of a larger epileptic network and associated with poor postsurgical outcome.SignificanceAnalysis of scalp HFO add clinically important information about the extent of epileptic areas during presurgical simultaneous scalp and intracranial EEG recordings.     

药物联合心理治疗在难治性精神分裂症患者中的应用效果观察

目的探讨药物联合心理治疗对难治性精神分裂症患者的应用效果。方法随机选取2015年9月～2017年8月78例难治性精神分裂症患者为研究对象。根据随机数字表法将其随机分为心理组和对照组,每组39例。对照组采用利培酮联合喹硫平治疗,心理组联合心理治疗。比较两组治疗效果。结果心理组治疗后的BPRS评分明显低于对照组,治疗总有效率明显高于对照组,复发率及不良反应发生率均显著低于对照组,组间比较,差异均有统计学意义(P0.05)。结论药物联合心理治疗可提高难治性精神分裂症患者的疗效,降低复发率及不良反应发生率,值得临床推广。     

难治性癫痫外科治疗的长期效果:一项6年随访研究结果

目的总结分析难治性癫痫外科治疗的方法及效果,为进一步的临床实践提供指导。方法综合临床症状学、神经电生理和神经影像学等方法,对66例难治性癫痫患者进行致痫灶定位和外科手术治疗,术后进行长期随访,平均随访时间为6年。结果 1例患者在随访期间因意外窒息死亡,余结果如下:谭启富标准:满意22例(33.8%),显著改善33例(50.8%),良好6例(9.2%),较差2例(3.1%),无改善2例(3.1%);Engel标准:Ⅰ级21例(32.3%),Ⅱ级13例(20.0%),Ⅲ级24例(36.9%),Ⅳ级7例(7.8%)。术后出现的并发症有偏瘫、失语、感染等,但均在短期内恢复。结论综合临床症状、神经电生理和神经影像学检查,可以精确定位癫痫患者的致痫灶,进一步选择合适的手术方法可以使难治性癫痫的外科治疗获得良好的效果。     

Ketogenic diet for refractory epilepsy with MEHMO syndrome: Caution for acute necrotizing pancreatitis

BackgroundThe MEHMO (mental retardation, epileptic seizures, hypogonadism and hypogenitalism, microcephaly, and obesity) syndrome, which is caused by a hemizygous variant in the EIF2S3 gene on chromosome Xp22, is associated with significant morbidity and mortality. Refractory epileptic seizures and glucose dysregulation are characteristic manifestations of the MEHMO syndrome, which can often diminish patients’ quality of life.CaseA 5-year-old boy was referred to our hospital because of profound intellectual disability, micropenis, cryptorchidism, central hypothyroidism, and microcephaly. He had neonatal hypoglycemia at birth and later experienced refractory epileptic seizures and developed obesity and insulin-dependent diabetes. A diagnosis of MEHMO syndrome was established on the basis of the patient’s clinical manifestations and de novo novel missense variant in the EIF2S3 gene (NM_001415.3:c.805 T > G) that was detected through whole-exome analysis. Although the patient’s refractory seizures and diabetes had been well controlled with a combination of ketogenic diet (KD) therapy and insulin therapy, acute fatal necrotizing pancreatitis occurred at the age of 68 months. Moreover, despite intensive care, his condition rapidly deteriorated to multiple organ failure and acute respiratory distress syndrome, resulting in death.ConclusionThe pathophysiology of glucose intolerance in MEHMO syndrome remains to be elucidated; however, recent studies have suggested that EIF2S3 gene variants could lead to glucose dysregulation and β-cell damage in the pancreas. We suspect that in the present case, KD therapy led to an abnormal load on the beta cells that were damaged owing to eIF2γ dysfunction. Therefore, the adverse effects of KD in patients with MEHMO syndrome should be considered.     

磷酸化肌球蛋白轻链及其激酶在耐药颞叶癫(癎)患者脑组织中的表达

目的探讨耐药性癫癎颞叶和海马脑组织中磷酸化肌球蛋白轻链(phosphorylation of myosin light chain,P-MLC)及其激酶(myosin light chain kinase,MLCK)的表达在癫癎芽生形成中的作用。方法免疫组化检测32例耐药性颞叶癫癎中P-MLC及其激酶MLCK的蛋白质表达水平,并与对照组比较。结果P-MLC免疫组化结果显示,耐药性颞叶癫癎组颞叶光密度值(0.35±0.13)高于对照组(0.19±0.027,P<0.05);耐药组海马光密度值(0.41±0.02)高于对照组(0.10±0.06,P<0.05)。免疫组化结果显示耐药组的MLCK表达水平在颞叶组织和海马组织中较对照组无统计学差异(P>0.05)。结论P-MLC及其激酶MLCK的功能与苔藓纤维芽生关系密切,P-MLC蛋白质产物在耐药性癫中明显升高,但其磷酸化激酶MLCK的表达并不升高,提示MLCK和去磷酸化酶间动态平衡被打破是P-MLC升高的原因,而P-MLC的升高可能在苔藓纤维芽生的形成中发挥重要作用。     

成人顽固性癫痫持续状态的临床分析

目的 探讨成人顽固性癫痫持续状态（RSE）的危险因素、临床特点、治疗及预后。方法 54例癫痫持续状态（SE）．58次发作事件，分为RSE组和非顽固性癫痫持续状态（NRSE）组．对病因、诱因、临床表现、辅助检查、预后等进行对比分析。结果 RSE占SE的43．1％，病毒性脑炎是RSE最主要的病因（P=0．001），相反，既往癫痫发作在NRSE中更常见（P=0．000），相应地药物治疗的改变引起的SE多为NRSE（P=-0．003）；RSE组GCS评分及预后较NRSE组均差（均P=0．000）。结论 SE经一、二线抗癫痫药治疗后仍有很大一部分难以控制，病毒性脑炎是导致RSE的一个重要病因，其预后较差．目前对RSE的治疗还缺乏十分合理的方案。     

含安丫啶的预激方案治疗难治／复发急性髓细胞白血病的临床研究

目的探讨安丫啶、小剂量阿糖胞苷和粒细胞集落刺激因子组成的预激方案治疗难治／复发性急性髓细胞白血病（RAML）的疗效及毒副反应。方法难治／复发性急性髓细胞白血病患者随机分成两组：研究组20例,给予安丫啶（Amsacrine）50mg／d静脉滴注,1-7d;阿糖胞苷（Ara—C）10mg／（m^2·d）皮下注射,1次／12h,1-14d;粒细胞集落刺激因子（G—CSF）300μg／d,从化疗前一天开始。对照组18例,应用MAE方案治疗。结果20例研究组患者12例获得CR,CR率60070,3例PR,总有效率（CR＋PR）75％。对照组18例患者中CR7例,CR率38．9％,PR2例,总有效率（CR＋PR）50％。两组CR率及总有效率有统计学差异（P〈0．05）。血液学毒性两组比较有显著差异（P〈0．05）。感染发生率：研究组25％,对照组94．4％,两组比较差异显著（P〈0．01）。结论含安丫啶预激方案治疗难治／复发性急性髓细胞白血病（RAWL）的疗效较MAE方案疗效好,不良反应显著减少,值得进一步临床推广应用研究。     

C-reactive protein and seizures in focal epilepsy: a video-electroencephalographic study

Purpose: C‐reactive protein (CRP) has been studied extensively in many noninflammatory neurologic conditions, but there has been little study of CRP in the context of seizures or epilepsy. The purpose of this study was to examine CRP concentrations in patients with refractory focal epilepsy who were undergoing video‐electroencephalography (EEG) monitoring compared with healthy controls, and CRP change during 24 h after a seizure. Methods: CRP levels were measured in serum at the onset of video‐EEG recording (CRP‐0h) and at 3, 6, 12, and 24 h after index seizure (the first verified localized‐onset seizure) in 31 patients during inpatient video‐EEG monitoring by using high sensitivity measurement of CRP concentration. The patients were categorized into two groups: temporal lobe epilepsy (TLE; n = 15) and extratemporal lobe epilepsy (XLE; n = 16). Eighty healthy volunteers served as controls. Key Findings: CRP‐0h concentration was significantly higher in patients with refractory focal epilepsy than in controls (3.5 vs. 0.7 mg/ml, p < 0.001). All five patients with elevated CRP‐0h (>mean + 2 standard deviations in controls) had TLE (vs. none in XLE; p = 0.018). Index seizure type was associated with CRP increase from baseline to maximum level after index seizure (p = 0.005). The most important predictor of increase in CRP level was secondarily generalized tonic–clonic seizure (SGTCS; p = 0.030). Significance: The higher baseline levels in patients with epilepsy compared with healthy controls demonstrates that CRP concentrations are also affected in refractory epilepsy. Our data suggest that SGTCS stimulates CRP production. These results emphasize the association between inflammation and refractory epilepsy.     

阿立哌唑与小剂量氯氮平治疗难治性精神分裂症对照研究

目的探讨阿立哌唑合并小剂量氯氮平治疗难治性精神分裂症的疗效和安全性。方法将40例难治性精神分裂症患者随机分为A组(阿立哌唑合并小剂量氯氮平,n=20)和B组(单用氯氮平,n=20)。于治疗前和治疗第4、8、12周末采用阳性与阴性症状量表(PANSS)评定临床疗效;使用副反应量表(TESS)评定不良反应,并进行对比分析。结果 2组治疗后PANSS总分较治疗前明显降低,A组显著低于B组,差异有统计学意义。2组治疗12周末有效率分别是80%、50%,差异有统计学意义(χ2=3.95,P<0.05)。2组不良反应比较差异有统计学意义(P<0.05)。结论阿立哌唑合并小剂量氯氮平治疗难治性精神分裂症疗效好,不良反应少且依从性好。