Connectivity measures suggest a sub-cortical generator of myoclonus in Angelman syndrome

ObjectiveThe clinical and neurophysiological characteristics of myoclonus in Angelman syndrome (AS) have been evaluated in single case or small cohorts, with contrasting results. We evaluated the features of myoclonus in a wide cohort of AS patients.MethodsWe performed polygraphic EEG-EMG recording in 24 patients with genetically confirmed AS and myoclonus. Neurophysiological investigations included jerk-locked back-averaging (JLBA), cortico-muscular coherence (CMC) and generalised partial directed coherence (GPDC). CMC and GPDC analyses were compared to those obtained from 10 healthy controls (HC).ResultsTwenty-four patients (aged 3–35 years, median 20) were evaluated. Sequences of quasi-continuous rhythmic jerks mostly occurred at alpha frequency or just below (mean 8.4 ± 1.4 Hz), without EEG correlate. JLBA did not show any clear transient preceding the jerks. CMC showed bilateral over-threshold CMC in alpha band that was prominent on the contralateral hemisphere in the patient group as compared to HC group. GPDC showed a significantly higher alpha outflow from both hemispheres toward activated muscles in the patient group, and a significantly higher beta outflow from contralateral hemisphere in the HC group.ConclusionsThese neurophysiological findings suggest a subcortical generator of myoclonus in AS.SignificanceMyoclonus in AS has not a cortical origin as previously hypothesised.     

Sensory evoked potentials in patients with Rett syndrome through the lens of animal studies: Systematic review

ObjectiveSystematically review the abnormalities in event related potential (ERP) recorded in Rett Syndrome (RTT) patients and animals in search of translational biomarkers of deficits related to the particular neurophysiological processes of known genetic origin (MECP2 mutations).MethodsPubmed, ISI Web of Knowledge and BIORXIV were searched for the relevant articles according to PRISMA standards.ResultsERP components are generally delayed across all sensory modalities both in RTT patients and its animal model, while findings on ERPs amplitude strongly depend on stimulus properties and presentation rate. Studies on RTT animal models uncovered the abnormalities in the excitatory and inhibitory transmission as critical mechanisms underlying the ERPs changes, but showed that even similar ERP alterations in auditory and visual domains have a diverse neural basis. A range of novel approaches has been developed in animal studies bringing along the meaningful neurophysiological interpretation of ERP measures in RTT patients.ConclusionsWhile there is a clear evidence for sensory ERPs abnormalities in RTT, to further advance the field there is a need in a large-scale ERP studies with the functionally-relevant experimental paradigms.SignificanceThe review provides insights into domain-specific neural basis of the ERP abnormalities and promotes clinical application of the ERP measures as the non-invasive functional biomarkers of RTT pathophysiology.     

植物神经癫痫52例脑电图分析

报道52倒6个类型植物神经癫痫,重点分析了脑电图的变化,脑电图异常率100％,69.4％的病例出现两侧阵发性4～7Hzθ节律及6和14Hz阳性棘波,提示中线深部有功能紊乱,亦支持丘脑及脑干部有病变。此外蝶骨电极能提高脑电图对本病的阳性诊断率,值得采用。本文证明脑电图对植物神经性癫痫与非癫痫的鉴别诊断,具有肯定的价值。     

动态脑电图与视频脑电图对癫癎诊断的对照研究

目的:观察24小时动态脑电图与视频脑电图对癫发作类型鉴别的价值。方法:242例患者进行24小时视频脑电监测,陪同家属做记录。结果由两名医师判定,一人关闭视频进行分析作为动态脑电组。一人打开视频分析作为视频脑电组。结果:242例患者中63例临床发作伴癫样放电,视频脑电监测后符合例数53例(84%),动态脑电监测后符合例数39例(61·9%),有显著性差异。结论:视频脑电监测与动态脑电图对癫发作类型的鉴别有显著的差异,视频脑电图更准确。     

Electroencephalographic changes in patients with herpes zoster

EEGs of 42 patients with herpes zoster and 6 with herpes zoster-associated encephalitis were studied to characterize the nature and prevalence of EEG abnormalities in apparently uncomplicated herpes zoster. Thirty-one percent of herpes zoster patients had EEG changes with reduced rhythm frequency ranging from 7 to 2 Hz activity. Frontotemporal localization was observed in 54% of the abnormal EEGs. When compared to EEG in herpes zoster associated encephalitis, the findings were qualitatively the same, but tended to be more severe in the encephalitis cases. No effect of acyclovir on the EEG could be demonstrated.     

Changes in the Eeg Background Activity of Children with Acute Lymphoblastic Leukemia During Cytotoxic Therapy

Thirteen children with acute lymphoblastic leukemia (ALL) were investigated before and during cytotoxic therapy. EEG findings were correlated with the clinical course and the therapy protocol and compared with normal data obtained from 295 healthy children. Frequency analysis of the background activity of the EEG revealed an initial slowing of the background activity prior to therapy and further slowing each time a combination of vincristine (VCR), daunorubicine (DAU) or adriblastine (ADR), prednisone (FRED), and L-asparaginase (L-ASP) was administered. The slowing of the background activity correlated only with the administration of these drugs. DAU, ADR, and FRED are not known to influence the EEG; therefore, VCR and L-ASP remain the primary candidates responsible for the central nervous system alteration.     

Identifying the affected hemisphere by 1H-MR spectroscopy in patients with temporal lobe epilepsy and no pathological findings in high resolution MRI

Up to 30% of patients with temporal lobe epilepsy (TLE) remain without remarkable changes in MRI. In this study we investigated the role of (1)H-MR spectroscopy ((1)H-MRS) in lateralizing the affected hemisphere in the mentioned patient group. Twenty-two consecutive patients diagnosed with TLE were investigated by high resolution MRI and (1)H-MRS. We examined the incidence and diagnostic accuracy of temporal metabolite alterations determined by Linear Combination of Model Spectra (L C Model) via water reference. Metabolite values of each hemisphere of TLE patients were compared with healthy controls. Results of metabolite alterations were related to intensive video EEG focus localization. Reduction of N-acetylaspartate + N-acetylaspartyl-glutamate (tNAA) in the affected hemisphere revealed identification in six of nine patients (66%) with unilateral TLE. Group comparison revealed a significant reduction of tNAA (6.1+/-0.8*) in the involved temporal lobe compared with controls (6.67+/-0.4*, P=0.026). Choline levels were significantly increased in the affected hemisphere (1.42+/-0.17*) compared with healthy controls (1.22+/-0.17*, P=0.035). The results of our study show that (1)H-MRS is able to identify the affected hemisphere of MRI negative TLE patients and can be used as an additive tool in multimodal focus localization.     

基于LVQ的自发脑电信号的分类研究

为了识别在不同思维状态下的自发脑电(EEG)信号，本文用6阶自回归(AR)模型表示EEG信号，用学习矢量量化(LVQ)神经网络作分类器，分别用LVQl和LVQ2．1算法对网络进行训练，并对分类结果进行测试，比较了网络选择不同参数时对分类正确率的影响。研究表明：竞争层神经元数目直接影响了正确率，当选择最佳参数值时分类正确率为62％-83％，因人而异。     

电针对沙鼠急性脑缺血再灌注后神经原损伤的保护作用

本实验利用沙鼠急性脑缺血再灌注模型，研究电针对脑缺血及再灌注各期脑电活动的影响及组织病理学的改变。结果表明：缺血１０ｍｌｎ，脑电幅度受到严重抑制，甚至变平坦，总功率大大下降，再灌注后总功率难以恢复，在１２０ｍｉｎ时仅恢复到缺血前的２７．３９±１１．３１％，以后即不再进一步恢复，电针组动物缺血１０ｍｉｎ再灌注后，脑电的恢复明显比对照组快，１２０ｍｉｎ时恢复至缺血前的７１．４５±１６．４６％（Ｐ＜０．０１），２４０ｍｉｎ时继续恢复至缺血前的７５．２７±１８．４３％。同时电针能明显减轻缺血１０ｍｉｎ后再灌注２４小时的神经原缺血性损伤。结果提示：电针对急性脑缺血引起的神经原损伤具有保护作用，并能促进脑功能的恢复。     

临床全身性抽搐发作状态下的动态脑电图分析

本文对７２例全身抽搐发作的患者且２４小时脑电图监测期间有临床发作的动态脑电图进行分析，年龄１个月￣６２岁，其中１个月￣１４岁６７例，结果显示：在临床发作状态下，ＡＥＥＧ均显示痫性放电，且有五种波形。其中４２例仅在临床发作时有痫性放电，而发作间歇期背景活动均正常。根据临床及ＡＥＥＧ波形特点，确诊阵挛性发作３８例，强直－阵挛性发作１３例，复杂性部分发作１３例，局限性发作泛化７例，强直发作１例。我们的研