Qianzheng powder for the treatment of primary Hemifacial spasm: A protocol for systematic reviews and meta-analysis

Background:Hemifacial spasm (HFS) is a clinical common neurological disease, its main performance for 1 side or 2 sides muscles (the orbicularis oculi muscle, expression, orbicularisoris muscles) recurrent paroxysmal, involuntary twitching, aggravating when excited or nervous, more severe cases of the disease may include difficulty in opening the eyes, crooked corners of the mouth, and twitching noises in the ears, etc.^[1] Early manifestations of the disease are intermittent mild convulsions of the orbicularis oculi muscle, and then gradually spread to 1 side of the facial muscles, such as frowning muscles, nasal muscles, buccinalis muscles, etc, especially the most obvious spasms of the oral muscles, which can involve the ipsilateral platysma muscle in severe cases, with each twitch for a few seconds to a few minutes. The disease will affect the quality of life such as speaking, eating, seeing and so on, and even cause psychological effects such as inferiority, anxiety and depression. At present, the incidence of the disease in China is 11 per 1.1 million, females are more common than males. There are many ways to treat HFS, but the Qianzheng powder has a unique advantage in treating this disease. Therefore, our systematic review aims to evaluate the efficacy and safety of Qianzheng powder in the treatment of Primary Hemifacial spasm, and to provide a reliable basis for clinical decision makers.Methods:From its inception until April 2021, we will search electronic databases, including PubMed, Embase, Cochrane Library, China Biomedical Literature Database, China Knowledge Infrastructure, Wanfang Database, and China Scientific Journals Database. The authors will independently sift through studies, extract data information, and assess methodological quality using the Cochrane Risk of Bias tool. The RevManV. 5.3 software will be used for statistical analysis.Results:The results of this study, which will be published in a peerreviewed journal, will evaluate the efficacy and safety of Qianzheng powder in the treatment of primary Hemifacial spasm.Conclusion:This systematic review will provide reliable evidence-based basis for treating primary Hemifacial spasm with Qianzheng powder.INPLASY Registration number:INPLASY202130037.     

Clinical practice guidelines for the interventional treatment of advanced pancreatic cancer (5th edition)

Pancreatic cancer has become a major disease affecting people's health because of its insidiousness, rapid progression and poor prognosis. Based on the practical needs of clinical work, combined with domestic multi-center research and experience, this guideline provides constructive suggestions for the interventional treatment of pancreatic cancer.     

Motor dysfunction in mild cognitive impairment as tested by kinematic analysis and transcranial magnetic stimulation

ObjectivePrevious studies have demonstrated voluntary movement alterations as well as motor cortex excitability and plasticity changes in patients with mild cognitive impairment (MCI). To investigate the pathophysiology of movement abnormalities in MCI, we tested possible relationships between movement abnormalities and primary motor cortex alterations in patients.MethodsFourteen amnestic MCI (aMCI) patients and 16 healthy controls were studied. Cognitive assessment was performed using clinical scales. Finger tapping was recorded by a motion analysis system. Transcranial magnetic stimulation was used to test the input/output curve of motor evoked potentials, intracortical inhibition, and short-latency afferent inhibition. Primary motor cortex plasticity was probed by theta burst stimulation. We investigated correlations between movement abnormalities, clinical scores, and cortical neurophysiological parameters.ResultsMCI patients showed less rhythmic movement but no other movement abnormalities. Cortical excitability measures were normal in patients, whereas plasticity was reduced. Movement rhythm abnormalities correlated with frontal dysfunction scores.ConclusionOur study in MCI patients demonstrated abnormal voluntary movement and plasticity changes, with no correlation between the two. Altered rhythm correlated with frontal dysfunction.SignificanceOur results contribute to the understanding of pathophysiological mechanisms of motor impairment in MCI.     

Cortical mechanisms underlying variability in intermittent theta-burst stimulation-induced plasticity: A TMS-EEG study

ObjectiveTo test the hypothesis that intermittent theta burst stimulation (iTBS) variability depends on the ability to engage specific neurons in the primary motor cortex (M1).MethodsIn a sham-controlled interventional study on 31 healthy volunteers, we used concomitant transcranial magnetic stimulation (TMS) and electroencephalography (EEG). We compared baseline motor evoked potentials (MEPs), M1 iTBS-evoked EEG oscillations, and resting-state EEG (rsEEG) between subjects who did and did not show MEP facilitation following iTBS. We also investigated whether baseline MEP and iTBS-evoked EEG oscillations could explain inter and intraindividual variability in iTBS aftereffects.ResultsThe facilitation group had smaller baseline MEPs than the no-facilitation group and showed more iTBS-evoked EEG oscillation synchronization in the alpha and beta frequency bands. Resting-state EEG power was similar between groups and iTBS had a similar non-significant effect on rsEEG in both groups. Baseline MEP amplitude and beta iTBS-evoked EEG oscillation power explained both inter and intraindividual variability in MEP modulation following iTBS.ConclusionsThe results show that variability in iTBS-associated plasticity depends on baseline corticospinal excitability and on the ability of iTBS to engage M1 beta oscillations.SignificanceThese observations can be used to optimize iTBS investigational and therapeutic applications.     

针对所有高血压患者进行原醛症筛查：势在必行

原发性醛固酮增多症(primary hyperaldosteronism,PA)占所有高血压患者的5％～10％.筛查为诊断PA的关键步骤.本文根据国内外最新证据,提出了目前筛查PA存在的一些主要问题,并给出建议,以期指导临床工作和提高PA检出率.     

Mice with a conditional deletion of Talpid3 (KIAA0586) – a model for Joubert syndrome

Joubert syndrome (JS) is a ciliopathy associated with mutations in numerous genes encoding cilia components. TALPID3 encoded by KIAA0856 in man (2700049A03Rik in mouse) is a centrosomal protein essential for the assembly of primary cilia. Mutations in KIAA0856 have been recently identified in JS patients. Herein, we describe a novel mouse JS model with a conditional deletion of the conserved exons 11–12 of Talpid3 in the central nervous system which recapitulates the complete cerebellar phenotype seen in JS. Talpid3 mutant mice exhibit key hallmarks of JS including progressive ataxia, severely hypoplastic cerebellar hemispheres and vermis, together with abnormal decussation of the superior cerebellar peduncles. The Purkinje cell layer is disorganised with abnormal dendritic arborisation. The external granule layer (EGL) is thinner, lacks primary cilia, and has a reduced level of proliferation. Furthermore, we describe novel cellular defects including ectopic clusters of mature granule neurons, and abnormal parallel fibre-derived synapses and disorientation of cells in the EGL. The defective glial scaffold results in abnormal granule cell migration which manifests as ectopic clusters of granule neurons. In addition, we show a reduction in Wnt7a expression suggesting that defects may arise not only from deficiencies in the Hedgehog (Hh) pathway but also due to the additional roles of Talpid3. The Talpid3 conditional knockout mouse is a novel JS model which fully recapitulates the JS cerebellar phenotype. These findings reveal a role for Talpid3 in granule precursor cell migration in the cerebellum (either direct or indirect) which together with defective Hh signalling underlies the JS phenotype. Our findings also illustrate the utility of creating conditional mouse models to assist in unravelling the molecular and cellular mechanisms underlying JS. © 2019 The Authors. The Journal of Pathology published by John Wiley & Sons Ltd on behalf of Pathological Society of Great Britain and Ireland.     

Neutrophilic Panniculitis in a child with MYSM1 deficiency

Neutrophilic panniculitis (NP) with myelodysplasia has been described in adults but not in children. We report a case of NP associated with myelodysplasia in a child with MYSM1 deficiency, a newly described syndrome with primary immunodeficiency (PI), bone marrow failure, and developmental aberrations.     

表观遗传调控与青光眼发病机制研究进展

表观遗传学主要机制包括DNA甲基化、组蛋白修饰和miRNAs等。这些机制将环境与基因相联系，在基因的特异性表达过程中发挥重要作用，影响疾病的表型。青光眼是全球第一大不可逆致盲眼病，其发病机制复杂，受遗传和环境的共同影响。本文分别对DNA甲基化、组蛋白修饰和miRNAs参与青光眼发病的机制进行综述，以期为临床诊治提供参考。