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.     

Inactivated influenza vaccine formulated with single-stranded RNA-based adjuvant confers mucosal immunity and cross-protection against influenza virus infection

Influenza vaccination is considered the most valuable means to prevent and control seasonal influenza infections, which causes various clinical symptoms, ranging from mild cough and fever to even death. Among various influenza vaccine types, the inactivated subunit type is known to provide improved safety with reduced reactogenicity. However, there are some drawbacks associated with inactivated subunit type vaccines, with the main ones being its low immunogenicity and the induction of Th2-biased immune responses. In this study, we investigated the role of a single-stranded RNA (ssRNA) derived from the intergenic region in the internal ribosome entry site of the Cricket paralysis virus as an adjuvant rather than the universal vaccine for a seasonal inactivated subunit influenza vaccine. The ssRNA adjuvant stimulated not only well-balanced cellular (indicated by IgG2a, IFN-γ, IL-2, and TNF-α) and humoral (indicated by IgG1 and IL-4) immune responses but also a mucosal immune response (indicated by IgA), a key protector against respiratory virus infections. It also increases the HI titer, the surrogate marker of influenza vaccine efficacy. Furthermore, ssRNA adjuvant confers cross-protective immune responses against heterologous influenza virus infection while promoting enhanced viral clearance. Moreover, ssRNA adjuvant increases the number of memory CD4⁺ and CD8⁺ T cells, which can be expected to induce long-term immune responses. Therefore, this ssRNA-adjuvanted seasonal inactivated subunit influenza vaccine might be the best influenza vaccine generating robust humoral and cellular immune responses and conferring cross-protective and long-term immunity.     

Female pelvic floor. Descriptive anatomy and clinical exploration by transvaginal ultrasound

In order to assess the state and pathology of the woman's pelvis minor, a number of methods are commonly used among practitioners, encompassing clinical exploration, radiology, MRN, urodynamics, endoscopy and echography.

Echography has been poorly used in clinical pelvic exploration and its reliability is actually a matter of controversy 1. However, echographic surveys can provide us with valuable gynecological data on the state and pathologies of the soft pelvis, within the genital regions or even going beyond them, i.e. the rectal channel, bladder, urethra, anus, vascular plexuses, and all of their supporting tissues.

At our research unit, we have been employing Transvaginal Ultrasound echography (TVU) for a long time in conjunction with other pelvis-focused methods in order to study different kinds of pelvic alterations. TVU has proven to be friendly to use, fast, harmless and inexpensive, allowing serial explorations and producing high-quality dynamic images (loop-cinema, video-tape). Furthermore, this method is fairly aseptic in that the occurrence of faeces in the rectal ampolla is not a nuisance but a bonus in tracking the contours of the rectum walls and other topographical features which would be otherwise difficult to survey.

A complete pelvic floor TVU may add no longer than 5-8 minutes to a routine gynecological examination, can be implemented by the general gynecologist and generates data that can be further studied by the appropriate specialist for a more insightful evaluation 2.     

中药肾区渗透并常规治疗高血压性肾损害40例疗效观察

目的：观察中药肾区皮肤外敷渗透加西药常规治疗高血压肾损害结果。方法：将部分中药粉碎微细化处理，将另部分中药液化处理．粉液混合装袋醋浸后外敷在肾区皮肤上，用药物导入仪肾区靶点式导人人体。并用一般西医常规治疗。结果：平均治疗3周血肌酐下降23％，BUN下降26％，24h蛋白尿下降23％，尿量增加16％，收缩压下降9％，舒张压下降7％。结论：中药的肾区皮肤外敷渗透加西医常规治疗效果明显高于一般常规治疗。     

回盲部恶性肿瘤的诊治

目的：分析回盲部恶性肿瘤的诊断和治疗过程，总结经验．以期提高早期确诊率和患者生存率。方法：回顾性分析近5年53例住院治疗的回盲部恶性肿瘤患者的临床资料。结果：53例患者中腺癌、黏液腺癌50例，恶性淋巴瘤3例。首次确诊率为75．5％。误诊或延迟诊断的疾病主要为阑尾炎、阑尾脓肿，胆囊炎、胆石症、下消化道出血、贫血待查等。结论：60岁以上的老年患者为回盲部恶性肿瘤的主要人群，肿瘤类型主要为腺癌。尽管首发症状缺乏特异性，但提高警觉性，详细询问病史，通过大便隐血试验、钡灌肠或气钡双重造影、纤维结肠镜检查仍能有效的提高首次确诊率，积极手术冶疗，可显著改善患者的预后。     

腰神经通道卡压综合征病因的探讨

为了探讨腰神经通道狭窄的病因，我们对２２４例腰椎间盘突出及腰椎管狭窄症患者进行了腰神经通道探查手术，结果发现其病因以腰部组织变性为主。除外伤及反复慢性腰部损伤引起腰部病变外，活动少，或很少进行腰部锻炼，体形肥胖也是引起腰神经通道狭窄日趋加重的致病因素。在治疗时强调不只满足于切除突出的椎间盘，应沿神经通道探查，清除一切卡压神经根的病变组织才能获得较好的治疗效果。我们随访１９７例，平均随访３ａ，优良率９３５％。     

The normally expressed κ immunoglobulin light chain gene repertoire and somatic mutations studied by single-sided specific polymerase chain reaction (PCR); frequent occurrence of features often assigned to autoimmunity

The expressed human κ light chain gene repertoire utilized by healthy individuals was studied by two different single-sided specific PCR techniques to avoid bias for certain V genes. A total of 103 rearranged κ sequences from peripheral blood mononuclear cells from healthy individuals were cloned from cDNA and assigned to the Vκ and Jκ germ-line genes with the closest overall homology. The use of cDNA rather than genomic DNA focused the analysis on activated B cells rich in mRNA. Accordingly, the sequences represented the applied repertoire and almost all were somatically mutated. V genes from the Jκ-proximal duplication unit of the κ locus were almost exclusively used. A total of 65% of the sequences could be assigned to four or five genes: A27 (humkv325), L6 (Vg), L2 (humkv328), and A3 and/or A19. N additions and P nucleotides were quite common and found in 32% and 21% of the sequences, respectively. Extended CDR3s more than nine residues in length were found in 18% of the sequences, and in 71% of cases this was due to insertion of an extra proline residue. This proline was usually explained from the germ-line sequences involved. These results are in good agreement with those of previous repertoire studies using potentially V-gene-biased techniques. Thus, it is clear that restricted V-gene usage, common N and P additions, and extended CDR3 regions are normal features and not, as has been claimed, characteristics of pathological autoantibodies.     

神经导航辅助显微手术治疗脑功能区肿瘤

目的探讨神经导航系统辅助下脑重要功能区肿瘤显微手术的治疗效果和应用价值。方法1999年12月～2002年6月应用StealthStation神经导航系统辅助切除邻近脑重要功能区肿瘤10例,对神经导航系统术中应用的优越性、精确性等进行分析。结果本组平均注册误差为(2.8±0.9)mm,肿瘤和重要解剖功能结构定位准确,肿瘤全切除率77.8%。术后神经功能未受明显影响,无手术并发症及死亡。结论神经导航系统对于切除邻近脑重要功能区肿瘤具有定位准确,动态示踪和实时导航,侵袭性小,安全、可靠等特点,有助于提高肿瘤全切率及降低手术并发症。