A Shape Memory Hydrogel with Excellent Mechanical Properties, Water Retention Capacity, and Tunable Fluorescence for Dual Encryption.

Information encryption platforms with reliable encryption performance, excellent mechanical performance, and high water retention capacity are highly desired. In this study, a tough double-network hydrogel is designed using the first network of a polyion complex containing lanthanide complexes via one-pot polymerization and the second network of a poly (N-hydroxyethyl acrylamide) (PHEAA) obtained by deep eutectic solvent (DES)-assisted introduction and subsequent photopolymerization. In this system, the pH-induced shape memory function and pH-/wavelength-dependent fluorescence allow the use of the prepared hydrogel as a dual-encryption platform. Owing to its high response reversibility, the hydrogel-based platform exhibits both a high security level and the advantages of rewritability, reprogrammability, and reusability. Additionally, the excellent mechanical properties and water retention capacity owing to the solvent exchange process involving the low-volatility solvent DES and the resulting introduction of the second network of PHEAA offer high practical application value for the hydrogel-based dual encryption platform, demonstrating its potential for information security protection.

Single-Electrode Electrochemical System for the Visual and High-Throughput Electrochemiluminescence Immunoassay.

The electrochemiluminescence (ECL) immunoassay with its visual and high-throughput detection has received considerable attention in the past decade. However, the development of a facile and cost-effective ECL device is still a great challenge. Herein, a single-electrode electrochemical system (SEES) for the visual and high-throughput ECL immunoassay was developed. The SEES was designed by attaching a plastic sticker with multiple holes onto a single carbon ink screen-printed electrode based on a resistance-induced potential difference. Due to its excellent properties of adsorption and bioaffinity, the carbon ink screen-printed electrode is applied to immobilize antibodies. When cardiac troponin I (cTnI), a specific biomarker of acute myocardial infarction, is present, it will be captured by the immobilized cTnI antibodies on the electrode surface, inhibiting electron transfer, resulting in a decrease of the ECL intensity of the luminol-H2O2 system. Using a smartphone as the detector, cTnI could be determined, ranging from 1 to 1000 ng mL-1, with a detection limit of 0.94 ng mL-1. The SEES based on the carbon ink screen-printed electrode is characterized by its high simplicity, cost effectiveness, and user-friendliness compared with conventional three-electrode systems and bipolar electrochemical systems using electrode arrays and shows superior advantages over other immunoassay strategies, with the elimination of multistep assembling and labeling processes. What is more, the fabricated SEES holds great potential in the point-of-care testing due to its tiny size and the combination of a smartphone detector.

Myelopathy associated with mixed connective tissue disease: clinical manifestation, diagnosis, treatment, and prognosis.

Mixed connective tissue disease (MCTD) is a chronic autoimmune disease, which has a broad range of clinical manifestations shared by systemic lupus erythematosus, systemic sclerosis, polymyositis/dermatomyositis, and rheumatoid arthritis. MCTD is featured with high serum titers of anti-ribonucleoprotein antibodies and multiple system involvement. Its spinal cord involvement mainly manifests as transverse myelopathy (TM) and longitudinal extensive transverse myelopathy (LETM). Myelopathy in MCTD is extremely rare, and is usually characterized by serious neurological complications, such as paralysis or muscular paresis, sensory impairment, and smooth muscle dysfunction. Progressive clinical manifestations combined with laboratory examinations and magnetic resonance imaging examinations play important roles in the diagnosis of this disease. In order to prevent permanent neurological damage to the spinal cord, plasmapheresis and intravenous immunoglobulin can be performed in patients at the early disease stage. Early high-dose corticosteroids combined with cyclophosphamide, followed by low doses of immunosuppressors, can improve the long-term prognosis of patients. There are only nine global cases reported on MCTD associated with myelopathy at present. The death rate and disability rate of myelopathy in MCTD are extremely high. In this review, the pathomechanisms, clinical manifestations, auxiliary examination, diagnosis, differential diagnosis, treatment, and prognosis of myelopathy in MCTD were systematically elucidated.

Potential Mechanism of Venous System for Leukoaraiosis: From post-mortem to in vivo Research.

BACKGROUND: Leukoaraiosis (LA), widely accepted as a feature of cerebral small vessel disease, significantly increases the incidence of stroke, dementia, and death. Cerebral small artery disease has been considered as one of the main causes of LA. However, since the term "venous collagenosis" (VC) was proposed in an atrophy research in 1995, there have been pathological and neuroimaging studies proving the association between the venous system and LA in aging, Alz-heimer's disease (AD), and Parkinson's disease. SUMMARY: Autopsy studies confirmed that thickening of the lumen wall in venules, which results from the deposition of collagen I and III, leading to vessel stenosis or occlusion, is closely associated with LA. Susceptibility-weighted imaging research revealed a controversial association of deep medullary veins and LA in vivo, regarding which there are no standard criteria currently. Nevertheless, retinal venous changes had been reported to increase the risk of LA development, providing a novel way for in vivo evaluation. As for the internal jugular vein, jugular venous reflux could double the LA score in aging and modulate circulation of cerebral spinal fluids. Key Messages: Disruption of the venous system was notably associated with LA in aging, AD, and Parkinson's disease post-mortem and in in vivo models. The venous pathological changes may induce cerebral hypoperfusion, drainage system disruption, and vasogenic oedema in the veins around the periventricular white matter. The clarification of VC in LA may provide an early prevention and early treatment strategy for LA patients.

Astrocytic gap junction inhibition by carbenoxolone enhances the protective effects of ischemic preconditioning following cerebral ischemia.

BACKGROUND: Stroke is the second leading cause of death worldwide and the most common cause of adult-acquired disability in many nations. Thus, attenuating the damage after ischemic injury and improving patient prognosis are of great importance. We have indicated that ischemic preconditioning (IP) can effectively reduce the damage of ischemia reperfusion and that inhibition of gap junctions may further reduce this damage. Although we confirmed that the function of gap junctions is closely associated with glutamate, we did not investigate the mechanism. In the present study, we aimed to clarify whether the blockade of cellular communication at gap junctions leads to significant reductions in the levels of glutamate released by astrocytes following cerebral ischemia. METHODS: To explore this hypothesis, we utilized the specific blocking agent carbenoxolone (CBX) to inhibit the opening and internalization of connexin 43 channels in an in vitro model of oxygen-glucose deprivation/re-oxygenation (OGD/R), following IP. RESULTS: OGD/R resulted in extensive astrocytic glutamate release following upregulation of hemichannel activity, thus increasing reactive oxygen species (ROS) generation and subsequent cell death. However, we observed significant increases in neuronal survival in neuron-astrocyte co-cultures that were subjected to IP prior to OGD/R. Moreover, the addition of CBX enhanced the protective effects of IP during the re-oxygenation period following OGD, by means of blocking the release of glutamate, increasing the level of the excitatory amino acid transporter 1, and downregulating glutamine expression. CONCLUSIONS: Our results suggest that combined use of IP and CBX represents a novel therapeutic strategy to attenuate damage from cerebral ischemia with minimal adverse side effects.

Roles of astrocytic connexin-43, hemichannels, and gap junctions in oxygen-glucose deprivation/reperfusion injury induced neuroinflammation and the possible regulatory mechanisms of salvianolic acid B and carbenoxolone.

BACKGROUND: Glia-mediated neuroinflammation is related to brain injury exacerbation after cerebral ischemia/reperfusion (I/R) injury. Astrocytic hemichannels or gap junctions, which were mainly formed by connexin-43, have been implicated in I/R damage. However, the exact roles of astrocytic hemichannels and gap junction in neuroinflammatory responses induced by I/R injury remain unknown. METHODS: Primary cultured astrocytes were subjected to OGD/R injury, an in vitro model of I/R injury. Salvianolic acid B (SalB) or carbenoxolone (CBX) were applied for those astrocytes. Besides, Cx43 mimetic peptides Gap19 or Gap26 were also applied during OGD/R injury; Cx43 protein levels were determined by western blot and cytoimmunofluorescene staining, hemichannel activities by Ethidium bromide uptake and ATP concentration detection, and gap junction intercellular communication (GJIC) permeability by parachute assay. Further, astrocyte-conditioned medium (ACM) was collected and incubated with microglia. Meanwhile, ATP or apyrase were applied to explore the role of ATP during OGD/R injury. Microglial activation, M1/M2 phenotypes, and M1/M2-related cytokines were detected. Also, microglia-conditioned medium (MEM) was collected and incubated with astrocytes to further investigate its influence on astrocytic hemichannel activity and GJIC permeability. Lastly, effects of ACM and MCM on neuronal viability were detected by flow cytometry. RESULTS: We found that OGD/R induced abnormally opened hemichannels with increased ATP release and EtBr uptake but reduced GJIC permeability. WB tests showed decreased astrocytic plasma membrane's Cx43, while showing an increase in cytoplasma. Treating OGD/R-injured microglia with ATP or OGD/R-ACM induced further microglial activation and secondary pro-inflammatory cytokine release, with the M1 phenotype predominating. Conversely, astrocytes incubated with OGD/R-MCM exhibited increased hemichannel opening but reduced GJIC coupling. Both SalB and CBX inhibited abnormal astrocytic hemichannel opening and ATP release and switched the activated microglial phenotype from M1 to M2, thus providing effective neuroprotection. Application of Gap19 or Gap26 showed similar results with CBX. We also found that OGD/R injury caused both plasma membrane p-Cx43(Ser265) and p-Src(Tyr416) significantly upregulated; application of SalB may be inhibiting Src kinase and attenuating Cx43 internalization. Meanwhile, CBX treatment induced obviously downregulation of p-Cx43(Ser368) and p-PKC(Ser729) protein levels in plasma membrane. CONCLUSIONS: We propose a vicious cycle exists between astrocytic hemichannel and microglial activation after OGD/R injury, which would aggravate neuroinflammatory responses and neuronal damage. Astrocytic Cx43, hemichannels, and GJIC play critical roles in OGD/R injury-induced neuroinflammatory responses; treatment differentially targeting astrocytic Cx43, hemichannels, and GJIC may provide novel avenues for therapeutics during cerebral I/R injury.

Transcranial Doppler combined with quantitative EEG brain function monitoring and outcome prediction in patients with severe acute intracerebral hemorrhage.

BACKGROUND: Neurological deterioration after intracerebral hemorrhage (ICH) is thought to be closely related to increased intracranial pressure (ICP), decreased cerebral blood flow (CBF), and brain metabolism. Transcranial Doppler (TCD) is increasingly used as an indirect measure of ICP, and quantitative EEG (QEEG) can reflect the coupling of CBF and metabolism. We aimed to combine TCD and QEEG to comprehensively assess brain function after ICH and provide prognostic diagnosis. METHODS: We prospectively enrolled patients with severe acute supratentorial (SAS)-ICH from June 2015 to December 2016. Mortality was assessed at 90-day follow-up. We collected demographic data, serological data, and clinical factors, and performed neurophysiological tests at study entry. Quantitative brain function monitoring was performed using a TCD-QEEG recording system at the patient's bedside (NSD-8100; Delica, China). Univariate and multivariable analyses and receiver operating characteristic (ROC) curves were employed to assess the relationships between variables and outcome. RESULTS: Forty-seven patients (67.3 ± 12.6 years; 23 men) were studied. Mortality at 90 days was 55.3%. Statistical results showed there were no significant differences in brain symmetry index between survivors and nonsurvivors, nor between patients and controls (all p > 0.05). Only TCD indicators of the pulsatility index from unaffected hemispheres (UPI) (OR 2.373, CI 1.299-4.335, p = 0.005) and QEEG indicators of the delta/alpha ratio (DAR) (OR 5.306, CI 1.533-18.360, p = 0.008) were independent predictors for clinical outcome. The area under the ROC curve after the combination of UPI and DAR was 0.949, which showed better predictive accuracy compared to individual variables. CONCLUSIONS: In patients with SAS-ICH, multimodal neuromonitoring with TCD combined with QEEG indicated that brain damage caused diffuse changes, and the predictive accuracy after combined use of TCD-QEEG was statistically superior in performance to any single variable, whether clinical or neurophysiological.

Cortical spreading depression preconditioning mediates neuroprotection against ischemic stroke by inducing AMP-activated protein kinase-dependent autophagy in a rat cerebral ischemic/reperfusion injury model.

Cortical spreading depression (CSD), based on its similarities with peri-infarct depolarization, is an ideal model for investigating transformation from the ischemic penumbra to infarct core. However, the underlying mechanisms remain unclear. To our knowledge, this is the first study to use a middle cerebral artery occlusion ischemic-reperfusion (I/R) injury model to determine whether AMP-activated protein kinase (AMPK)-dependent autophagy contributes to the neuroprotection of CSD preconditioning in rat cortex. In this study, we topically applied a pledget soaked in 1 mol/L KCl solution on rat cortex for 2 h to elicite CSD or 1 mol/L NaCl solution as a control. The results demonstrated that CSD preconditioning significantly decreased the infarct volume, neurological deficits and neuronal apoptosis in the cortical penumbra of middle cerebral artery occlusion rats, which was inhibited by the autophagy inhibitor 3-methyladenine (3-MA, 200 nmol). Furthermore, CSD increased the protein levels of the autophagy markers LC3-II, Beclin-1 and the p-AMPK (Thr172 )/AMPK ratio at 12 h and decreased P62 and p-P70S6K (Thr389 ). Moreover, the AMPK inhibitor Compound C (20 mg/kg) down-regulated the LC3-II, p-AMPK (Thr172 )/AMPK and ULK1 levels, up-regulated the P62 and p-P70S6K (Thr389 ) levels induced by CSD. The neuroprotection of CSD is likely a result of AMPK-mediated autophagy activity and autophagy-induced neuronal cells apoptosis inhibition. These novel findings support a central role for AMPK and autophagy in CSD-induced ischemic tolerance. AMPK-mediated autophagy may represent a new target for stroke.

A case report: Becker muscular dystrophy presenting with epilepsy and dysgnosia induced by duplication mutation of Dystrophin gene.

BACKGROUND: Becker muscular dystrophy (BMD), a genetic disorder of X-linked recessive inheritance, typically presents with gradually progressive muscle weakness. The condition is caused by mutations of Dystrophin gene located at Xp21.2. Epilepsy is an infrequent manifestation of BMD, while cases of BMD with dysgnosia are extremely rare. CASE PRESENTATION: We describe a 9-year-old boy with BMD, who presented with epilepsy and dysgnosia. Serum creatine kinase level was markedly elevated (3665 U/L). Wechsler intelligence tests showed a low intelligence quotient (IQ = 65). Electromyogram showed slight myogenic changes and skeletal muscle biopsy revealed muscular dystrophy. Immunohistochemical staining showed partial positivity of sarcolemma for dystrophin-N. Multiplex ligation-dependent probe amplification revealed a duplication mutation in exons 37-44 in the Dystrophin gene. CONCLUSIONS: The present case report helps to better understand the clinical and genetic features of BMD.

A Facile Strategy to Fabricate Multishape Memory Polymers with Controllable Mechanical Properties.

A facile blending strategy to fabricate multishape memory polymers (SMPs) with only one sort of phase transition material has been reported. In this work, olefin block copolymer (OBC) and styrene-b-(ethylene-co-butylene)-b-styrene (SEBS), which are both physically crosslinked, are blended with crystalline paraffin together. Due to the different interactions between polymer matrices and paraffin, the paraffin penetrated in OBC and SEBS exhibit separated melting transitions. It is quite interesting that merely paraffin distributed in OBC also shows two distinct melting transitions with enough OBC content in composites. Therefore, excellent quadruple shape memory effect can be achieved with a maximum of three melting transitions. Furthermore, through adjusting the polymer species and content, the mechanical and rheological properties can be conveniently tuned to a great extent. Compared with the reported strategies, this simple and controllable method sheds light on rapid design of multi-SMPs using inexpensive raw materials, which greatly paves the way for multi-SMPs from laboratory to factory.

Relaxation behavior of shear-induced crystallization precursors in isotactic polypropylene containing sorbitol-based nucleating agents with different nucleating abilities.

The nature of shear-induced crystallization precursors, especially their relaxation behaviour, is an important issue in polymer chemical physics. In our work, relaxation behavior of shear-induced crystallization precursors in isotactic polypropylene containing various sorbitol-based nucleating agents (NAs) with different nucleating abilities was investigated by using both rheological and in situ small angle X-ray scattering (SAXS) methods. Rheological crystallization kinetics results showed that the amount of shear-induced precursors, calculated separately from the total nuclei, decayed exponentially with relaxation time in both pure and nucleated iPP. By fitting the decay of shear-induced precursors with relaxation time, the relaxation rate of precursors in nucleated iPP was found to be slower than that in pure iPP. Interestingly, it further decreased with the increase in the nucleating ability of sorbitol-based NAs. Meanwhile, the life-time of precursors was prolonged in nucleated iPP with increasing nucleating ability. Similar results were also testified by in situ SAXS measurements. By investigating the life-times at different temperatures, the activation energy for the relaxation of precursors was calculated and found to increase with stronger nucleating abilities. Our results demonstrated that sorbitol-based NAs could stabilize the iPP precursors and the effect of stabilization enhanced with the increase in nucleating ability. We believe that our work can not only help better reveal the relaxation behavior of shear-induced precursors but also provides a new perspective for understanding the role of NAs in real processing.

Neuroprotective Effect of Salvianolic Acids against Cerebral Ischemia/Reperfusion Injury.

This study investigated the neuroprotective effect of salvianolic acids (SA) against ischemia/reperfusion (I/R) injury, and explored whether the neuroprotection was dependent on mitochondrial connexin43 (mtCx43) via the phosphatidylinositol 3-kinase/protein kinase B (PI3K/AKT) pathway. In vitro, we measured astrocyte apoptosis, mitochondrial membrane potential, and also evaluated the morphology of astrocyte mitochondria with transmission electron microscopy. In vivo, we determined the cerebral infarction volume and measured superoxide dismutase (SOD) activity and malondialdehyde (MDA) content. Additionally, mtCx43, p-mtCx43, AKT, and p-AKT levels were determined. In vitro, we found that I/R injury induced apoptosis, decreased cell mitochondrial membrane potential (MMP), and damaged mitochondrial morphology in astrocytes. In vivo, we found that I/R injury resulted in a large cerebral infarction, decreased SOD activity, and increased MDA expression. Additionally, I/R injury reduced both the p-mtCx43/mtCx43 and p-AKT/AKT ratios. We reported that both in vivo and in vitro, SA ameliorated the detrimental outcomes of the I/R. Interestingly, co-administering an inhibitor of the PI3K/AKT pathway blunted the effects of SA. SA represents a potential treatment option for cerebral infarction by up-regulating mtCx43 through the PI3K/AKT pathway.

Mechanism of Mitochondrial Connexin43's Protection of the Neurovascular Unit under Acute Cerebral Ischemia-Reperfusion Injury.

We observed mitochondrial connexin43 (mtCx43) expression under cerebral ischemia-reperfusion (I/R) injury, analyzed its regulation, and explored its protective mechanisms. Wistar rats were divided into groups based on injections received before middle cerebral artery occlusion (MCAO). Cerebral infarction volume was detected by 2,3,5-triphenyltetrazolim chloride staining, and cell apoptosis was observed by transferase dUTP nick end labeling. We used transmission electron microscopy to observe mitochondrial morphology and determined superoxide dismutase (SOD) activity and malondialdehyde (MDA) content. MtCx43, p-mtCx43, protein kinase C (PKC), and p-PKC expression were detected by Western blot. Compared with those in the IR group, cerebral infarction volumes in the carbenoxolone (CBX) and diazoxide (DZX) groups were obviously smaller, and the apoptosis indices were down-regulated. Mitochondrial morphology was damaged after I/R, especially in the IR and 5-hydroxydecanoic acid (5-HD) groups. Similarly, decreased SOD activity and increased MDA were observed after MCAO; CBX, DZX, and phorbol-12-myristate-13-acetate (PMA) reduced mitochondrial functional injury. Expression of mtCx43 and p-mtCx43 and the p-Cx43/Cx43 ratio were significantly lower in the IR group than in the sham group. These abnormalities were ameliorated by CBX, DZX, and PMA. MtCx43 may protect the neurovascular unit from acute cerebral IR injury via PKC activation induced by mitoKATP channel agonists.

Preparation of Lanthanide-Polymer Composite Material via Click Chemistry.

Covalently attaching lanthanide complexes to the polymer backbone can effectively reduce the clustering of lanthanides and thus become an important strategy to fully unleash their potential. In this Communication, a metal-free click reaction is used for the first time to link a lanthanide complex to the polymer matrix. A diene-bearing copolymer with anthracenylmethyl methacrylate as a monomer and a dienophile-bearing lanthanide complex with 5-maleimido-1,10-phenanthroline as the second ligand are synthesized and coupled together through a Diels-Alder cycloaddition (DA). A comparative investigation demonstrates that the composite material prepared by DA click reaction shows the highest quantum yields in the same lanthanide concentration as compared to materials prepared by widely used "directly doping" and "in situ coordinating lanthanide ions with macromolecular ligand" approaches. This work suggests that the "metal-free" DA click reaction can be a promising tool in the synthesis of high efficient lanthanide functionalized polymeric materials.

Tuning the crystalline and mesophase structure of olefin block copolymer through self-nucleation and annealing treatments.

As a type of novel semi-crystalline block copolymers, the final properties of olefin block copolymers (OBCs) greatly depend on the crystalline and phase-separated structures. In the present work, we systematically investigated the influence of self-nucleation and annealing on the lamellar and mesophase-separated structure of OBCs. According to the crystalline and melting behavior after self-nucleation and annealing treatments, four different regimes can be recognized with the self-seeding temperature Ts varying from 125 to 109 °C. In regime A, only self-nucleation occurs, while it coexists with lamellar thickening in regime B. In regime C, there is only lamellar thickening behavior. The lamellar thickening induced inconsecutive lamellar crystals observed revealed that the rearrangement of the hard blocks, which are next to the soft blocks and trapped in the intermediate regime between crystalline and amorphous phases, into neighboring lamellar crystals should be the mechanism for the lamellar thickening of the OBCs. Surprisingly, no lamellar thickening occurs and a new small melting peak appears at lower temperatures in regime D. Considering the block dispersity of OBCs, the emergence of a small melting peak at lower temperatures can be attributed to the crystallization of the ethylene sequence with relatively weaker crystallization abilities, which are not able to crystallize in a standard crystalline state. Based on these findings, we gained some new understandings on lamellar thickening behavior of OBCs and established the self-nucleation and annealing process as a powerful tool for tuning the crystalline and phase-separated structures of OBCs.

Elevated blood pressure and headache disorders in China--associations, under-treatment and implications for public health.

BACKGROUND: Both hypertension (HTN) and headache disorders are highly prevalent worldwide. Our purpose, in a nationwide study of the Chinese general population, was to evaluate any association between primary headache disorders and elevated blood pressure (eBP). We could not collect data on antihypertensive therapy, but took the view that, whatever such therapy might be taken, eBP was a sign that it was failing to meet treatment needs. Therefore, as a secondary purpose, important from the public-health perspective, we would present the prevalence of eBP (treated or not) as indicative of unmet health-care need in China. METHODS: This was a questionnaire-based nationwide cross-sectional door-to-door survey using cluster random-sampling, selecting one adult (18-65 years) per household. Headache was diagnosed by ICHD-II criteria and eBP as systolic blood pressure ≥140 mmHg and/or diastolic blood pressure ≥90 mmHg. Chi-squared test and multivariate logistic regression analysis were used to assess the strength and significance of associations. We set significance at P ≤ 0.05. RESULTS: Of 5,041 survey participants (participation rate 94.1 %), 154 were excluded because of missing BP data, leaving 4,987 for analysis [mean age: 43.6 ± 12.8 years; male 2,532 (mean age: 43.4 ± 12.9 years); female 2,455 (mean age 43.9 ± 12.8 years)]. There were 466 participants with migraine, 535 with tension type headache (TTH) and 48 with all causes of headache on ≥15 days/month. The prevalence of eBP was 22.1 % (males 22.9 %, females 21.3 %). No associations of eBP with any of the headache disorders survived multivariate adjusted analysis. The demographic and anthropometric variables most strongly associated with eBP were higher age (AOR 3.7) and being overweight (AOR 2.4), seen in both genders. Less strong were male gender, lower educational level and urban habitation. CONCLUSIONS: We found no clear-cut associations between eBP and any headache disorder. The associations with demographic and anthropometric variables may have acted as confounders in past reports to the contrary. We did find an alarmingly high prevalence of eBP, recognizing that this signals substantial under-treatment in China of a serious condition, and therefore a major public-health concern.

Combined occurrence of a novel TOR1A and a THAP1 mutation in primary dystonia.

BACKGROUND: The ΔGAG deletion of the TOR1A gene (DYT1) is responsible for DYT1 dystonia. However, no other TOR1A mutation has been reported in the Chinese population. METHODS: Two hundred one dystonia patients without the ΔGAG deletion were screened for other mutations in TOR1A. Gene function changes were analyzed by subcellular distribution and luciferase reporter assay. RESULTS: A novel TOR1A mutation (c.581A>T, p.Asp194Val) was found in a patient with early-onset segmental dystonia harboring a THAP1 mutation (c.539T>C, p.Leu180Ser). Overexpression of mutant TOR1A Asp194Val protein induces inclusion formation in SK-N-AS cell lines, and the repressive activity of the mutant THAP1 Leu180Ser protein on TOR1A gene expression is decreased compared with wild-type THAP1. CONCLUSIONS: This is the first report about a dystonia patient harboring two distinct dystonia gene mutations. Functional analysis indicated a potential additive effect of these two mutations, which might provoke the occurrence of dystonic symptoms in this patient.

A rare case of limbic encephalitis with anti leucine-rich glioma inactivated-1 (LGI1) antibodies.

We report a case of limbic encephalitis with LGI1 antibodies and cranial MRI abnormality. A 41-year-old woman who presented with confusional state that had started 1 month ago. Brain magnetic resonance imaging revealed hyperintense signal in right basal ganglia followed by hyperintense signals in left hippocampus and bilateral basal ganglia half a month later. This case expands the spectrum of limbic encephalitis to include LGI1 antibodies and cranial MRI changing process.

A rare case of critical illness polyneuropathy and literature review.

A 40- year-old Male was admitted to the first hospital of Jilin University with the complaint of 4 days of fever and headache and aggravation of weakness in his lower extremities accompanied with dysuria and disturbance of consciousness for one day. He had tachycardia, tachypnea and elevated white blood cell counts. General status of the patient got better day by day, while weakness and pain in his lower extremities had developed and gradually quadriplegia arose. When intensive care unit history, weaning difficulty from mechanical ventilator, clinical manifestations in intensive care unit associated with SIRS, symmetrical paresis pronounced in distal lower extremities, absence of deep tendon reflexes, evidence of distal sensory impairment, presence of electrophysiologic results indicating axonal sensorimotor polyneuropathy and muscle and nerve biopsy results were taken into consideration, he was diagnosed as critical illness polyneuropathy.

A rare case of pituitrin-induced delayed encephalopathy.

A 26-year-old girl was admitted to the Neurological Department of The First Teaching Hospital of Jilin University with complaints of rapidly deteriorating speech clumsiness for 18 days. Five Days before her attack she had undergone intramuscular pituitrin therapy on account of recurrent haemoptysis. Cranial MRI revealed multiple abnormal signals in bilateral hemisphere and symmetric abnormal signal in bilateral caudate nucleus and putamen. Serum electrolyte analysis revealed mild hyponatremia. The abnormal signals in bilateral hemisphere almost disappeared after 7 days of cerebral circulation ameliorating and serum electrolyte turbulence correcting therapy, whereas the symmetric abnormal signals in bilateral caudate nucleus and putamen still existed. A diagnosis of delayed encephalopathy was made and we presume the encephalopathy was associated with pituitrin therapy.