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OBJECTIVE: To investigate aquaporin-4 antibody (AQP4-IgG) dynamics and relapse risk in patients with seropositive neuromyelitis optica spectrum disorder treated with immunosuppressants. METHODS: This observational cohort study with prospectively collected data included 400 neuromyelitis optica spectrum disorder patients seropositive for AQP4-IgG and treated with immunosuppressants. Serum AQP4-IgG was detected by fixed cell-based assay every 6 months. RESULTS: After treatment with immunosuppressants, 128 patients became AQP4-IgG seronegative. The median time to become seronegative for 400 patients was 76.4 months (61.4 months, NA). Among those patients with negative change of AQP4-IgG, the mean annualized relapse rate significantly decreased after patients became seronegative (0.20 vs 0.77, p < 0.001), and a positive correlation was observed between time to become seronegative and relapse (OR 1.018, 95% CI 1.001-1.035, p < 0.05). Independent risk factors for AQP4-IgG becoming seronegative were older age at onset, initiation of immunosuppressants at onset, and shorter disease duration before maintenance therapy. Independent risk factors for relapse included younger age (≤46.4 years) at onset, poly-system involvement in the first attack, and unchanged or increased AQP4-IgG titer. The relapse risk was not associated with sex, combination with connective tissue disease, seropositivity for systemic autoimmune antibodies, or incomplete recovery from the first attack. INTERPRETATION: Patients with younger age at onset, poly-system involvement in the first attack, and unchanged or increased titer of AQP4-IgG are most likely to experience relapse under treatment with immunosuppressants. Time to AQP4-IgG becoming seronegative and change of AQP4-IgG titer may become the surrogate efficacy biomarkers in clinical trials. ANN NEUROL 2023;93:1069-1081.
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Neuromielitis Óptica , Humanos , Persona de Mediana Edad , Inmunosupresores/uso terapéutico , Acuaporina 4 , Autoanticuerpos , Enfermedad Crónica , Biomarcadores , Recurrencia , Inmunoglobulina GRESUMEN
Photocatalytic technology is a popular research area for converting solar energy into environmentally friendly chemicals and is considered the greenest approach for producing H2O2. However, the corresponding reactive oxygen species (ROS) and pathway involved in the photocatalytic generation of H2O2 by the Bi2.15WO6-glucose system are still not clear. Quenching experiments have established that neither â¢OH nor h+ contribute to the formation of H2O2, and show that the formed surface superoxo (≡Bi-OOâ¢) and peroxo (≡Bi-OOH) species are the predominant ROS in H2O2 generation. In addition, various characterizations indicate the enhanced electron-transfer on the surface of Bi2.15WO6 with increasing contents of glucose via the ligand-to-metal charge transfer pathway, confirming H-transfer from glucose to ≡Bi-OO⢠or ≡Bi-OOH. The increased production of H2O2 with decreasing bond dissociation energy (BDEO-H) values of various phenolic compounds again supports the H-transfer mechanism from phenolic compounds to ≡Bi-OO⢠and then to ≡Bi-OOH. DFT calculations further reveal that on the Bi2.15WO6 surface, oxygen is sequentially reduced to ≡Bi-OO⢠and ≡Bi-OOH, while H-transfer from H2O or glucose to ≡Bi-OO⢠and ≡Bi-OOH, resulting in the production of H2O2. The lower energy barrier of H-transfer from adsorbed glucose (0.636 eV) than that from H2O (1.157 eV) indicates that H-transfer is more favorable from adsorbed glucose. This work gives new insight into the photocatalytic generation of H2O2 by Bi2.15WO6 in the presence of glucose/phenolic compounds via the H-abstraction pathway.
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Although precise regulation of the crystalline structures of metal oxides is an effective method to improve their antibacterial activities, the corresponding mechanisms involved in this process are still unclear. In this study, three kinds of cuprous oxide (Cu2O) samples with different structures of cubes, octahedra, and rhombic dodecahedra (c-Cu2O, o-Cu2O, and r-Cu2O) have been successfully synthesized and their antibacterial activities are compared. The antibacterial activities follow the order of r-Cu2O > o-Cu2O > c-Cu2O, revealing the significant dependence of the antibacterial activities on the crystalline structures of Cu2O. Quenching experiments, as well as the NBT and DPD experiments indicate that ≡CuIIâOO⢠superoxo and ≡CuIIâOOH peroxo, instead of â¢OH, O2â¢-, and H2O2, are the primary oxidizing species in the oxidative damage to E. coli. Raman analysis further confirms the presence of both ≡CuIIâOO⢠superoxo and ≡CuIIâOOH peroxo on the surface of r-Cu2O. On the other hand, the NCP experiment reveals that Cu+, instead of Cu2+, also contributes to the antibacterial process. This study provides new insight into the antibacterial mechanisms of Cu2O.
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Bismaleimide (BMI) is often used as the cross-linking reagent in Diels-Alder (D-A)-type intrinsic self-healing materials (DISMs) to promote the connectivity of damaged surfaces based on reversible D-A bond formation on the molecular scale. Until now, although DISMs have exhibited great potential in the applications of various sensors, electronic skin, and artificial muscles, it is still difficult to prepare DISMs with satisfactory self-healing abilities and high tensile strengths and strains at the same time, thus largely limiting their applications in self-healing anticorrosive coatings. Herein, symmetrical trimaleimide (TMI) was successfully synthesized, and trimaleimide-structured D-A self-healing polyurethane (TMI-DA-PU) was prepared via the reversible D-A reaction (cycloaddition of furan and maleimide). As a DISM, TMI-DA-PU exhibits apparently higher self-healing efficiency (98.7%), tensile strength (25.4 MPa), and strain (1378%) compared to bismaleimide-structured D-A self-healing polyurethane (BMI-DA-PU) (self-healing efficiency, 90.2%; tensile strength, 19.3 MPa; strain, 1174%). In addition, TMI-DA-PU shows a high recycling efficiency (>95%) after 4 cycles of recycling. A series of characterizations indicate that TMI provides more monoene rings as the self-healing sites, forms denser cross-linked structures compared to BMI, and is, thus, more appropriate to be used for DISM applications. Moreover, the barrier abilities of coatings can be semi-quantitatively expressed by the impedance value at 0.01 Hz (|Z|0.01 Hz). The |Z|0.01â¯Hz value of the TMI-DA-PU coating is 3.93 × 109 Ω cm2 on day 0, which is significantly higher than that of the BMI-DA-PU coating (6.76 × 108 Ω cm2 on day 0), indicating that the denser rigid cross-linked structure of TMI results in the small porosity in the TMI-DA-PU coating, thus effectively improving the anticorrosion performance. The construction of DISMs with the structure of TMI demonstrates immense potential in self-healing anticorrosive coatings.
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Although the photocatalytic reduction of Cr(VI) to Cr(III) by traditional powder photocatalysts is a promising method, the difficulty and poor recovery of photocatalysts from water hinder their wide practical applications. Herein, we present that FeC2O4/Bi2.15WO6 (FeC2O4/BWO) composites were tightly bonded to modified polyvinylidene fluoride (PVDF) membranes by chemical grafting with the aid of polyvinyl alcohol (PVA) to form photocatalytic composite membranes (PVDF@PVA-FeC2O4/BWO). The contact angle of PVDF@PVA-FeC2O4/BWO (0.06 wt % of FeC2O4/BWO) is 48.0°, which is much lower than that of the pure PVDF membrane (80.5°). Meanwhile, the permeate flux of 61.43 g m-2 h-1 and water flux of 250.60 L m-2 h-1 were observed for PVDF@PVA-FeC2O4/BWO composite membranes. The tensile strength of composite membranes reached 48.84 MPa, which was 9.8 times higher than that of PVDF membrane. It was found that the PVDF@PVA-FeC2O4/BWO membrane exhibited excellent photocatalytic Cr(VI) reduction performance under both simulated and real sunlight irradiation. The adsorption for Cr(VI) by PVDF@PVA-FeC2O4/BWO can reach 47.6% in the dark process within 30 min, and the removal percentage of Cr(VI) could reach 100% with a rate constant k value of 0.2651 min-1 after 10 min of light exposure, indicating a synergistic effect of adsorption and photoreduction for Cr(VI) removal by the composite membrane. The PVDF@PVA-FeC2O4/BWO membrane had good stability and reusability after seven consecutive cycles. Most importantly, the influences of foreign ions on Cr(VI) reduction were investigated to mimic real sewage, which revealed that no obvious adverse effects can be found with the presence of common foreign ions in sewage. The photocatalytic membrane material developed in this study provides a new idea for treating Cr(VI)-containing wastewater and has a more significant application prospect.
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In harsh environments, it is crucial to design personal protective materials that possess both puncture/cut resistance and chemical resistance. In order to fulfill these requirements, this study introduces an innovative approach that combines hydrophobically modified rigid nanoparticles with thermoplastic polyurethane elastomers. These materials are then laminated with high-performance aramid fabrics through a scraping process, resulting in a multifunctional composite with puncture/cut resistance, superhydrophobicity, self-cleaning properties, and acid/alkali resistance. The quasi-static puncture tests conducted reveal the remarkable performance of the composite. The maximum spike puncture resistance reaches 267.62 N, which is 17.14 times higher than that of the pure fabric (15.61 N). Similarly, the maximum knife puncture resistance reaches 115.02 N, exhibiting a 5.01 times increase compared to that of the pure aramid fabric (22.97 N). Furthermore, the results obtained from the yarn pull-out, fabric burst strength, and tearing experiments demonstrate that the incorporation of rigid nanoparticles significantly enhances the friction between the yarns, enabling a greater number of yarns to participate in the dissipation of impact energy. As a result, the puncture resistance of the fabric is greatly improved. Significantly, the composite exhibits sustained superhydrophobicity even after exposure to harsh chemicals such as concentrated sulfuric acid and sodium hydroxide as well as undergoing cyclic mechanical wear. These findings highlight the composite's exceptional durability and resistance to corrosion. Overall, this study offers insights and methods for the development of multifunctional flexible puncture-resistant equipment for individuals.
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Photooxidation of As(III) by TiO2 is a complicated process in which the oxidation mechanisms are always controversial. In this study, the enhanced photooxidation rates of As(III) with increasing pH values from 8.0 to 11.0 indicate the high photocatalytic reactivity of TiO2 under alkaline conditions. Moreover, As(III) improves the production of H2O2, indicating H-abstraction from As(III) (soluble or adsorbed) for H2O2 production. Although O2Ë-, h+, ËOH and -OOH are always regarded as the reactive oxygen species in the UV-TiO2 system, the superoxo and peroxo species formed on the surface of TiO2 also contribute to As(III) oxidation. The As(III)-O-Ti(IV) surface complexes formed on TiO2, as well as the decreased bandgaps of TiO2 with increasing concentrations of As(III) indicate that the ligand-to-metal charge transfer (LMCT) pathway also contributes to the oxidation of As(III) under alkaline conditions. Electrochemical analyses further reveal that As(III) enhances the electron density on the surface of TiO2, thereby improving the catalytic reactivity of TiO2. We therefore suggest that H-abstraction from As(III) or H2O to the formed superoxo and peroxo species results in the formation of H2O2, accompanied by the oxidation of As(III). This enriches our knowledge on the oxidation of As(III), as well as other contaminants rich in -OH groups during the photocatalytic oxidation processes.
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Arsenitos , Arsenitos/química , Peróxido de Hidrógeno/química , Ligandos , Metales , Titanio/químicaRESUMEN
Biotic and abiotic oxidation of Mn(II) in aqueous environments is an important process for the cycling of many elements. However, the mechanism involved in photocatalytic oxidation of Mn(II) has not been clearly elucidated yet. In this study, the photocatalytic oxidation of Mn(II) on the surface of self-doped Bi2+xWO6 (Bi2.15WO6) under visible light was conducted. Kinetics results show that visible light apparently accelerates the oxidation of Mn(II) to Mn(III, IV) oxides on Bi2.15WO6. The average oxidation states (AOS) of manganese reach 2.18 after 80 min of reaction under visible light at pH 8.50. Characterizations indicate the formation of Bi(III)-O-Mn(II) surface complexes between Mn(II) and surface Bi(III) on Bi2.15WO6, which then decreases the bandgap of [Bi2.15WO6 + Mn(II)]light (2.53 eV) compared with those of [Bi2.15WO6 + Mn(II)]dark (2.72 eV) and pure Bi2.15WO6 (2.86 eV), suggesting the contribution of the ligand-to-metal charge transfer (LMCT) pathway to the photocatalytic oxidation of Mn(II). Moreover, the addition of inorganic oxidants with strong oxidizing capacities (such as Cr2O72-, NO3- or NO2-) significantly increases the oxidation rate of Mn(II), further verifying the contribution of the LMCT pathway to Mn(II) oxidation. We therefore suggest that the LMCT pathway is one of the important oxidation routes for Mn(II) oxidation on Bi2.15WO6 under visible light.
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Although synthesis of oligoaniline (OANI) by persulfate and aniline has been investigated in the recent years, the impact of phenol on the synthesized soluble OANI is still not clear. In this study, our results indicate that phenol and pH mediate the production of the blue water-soluble OANI (OANIblue) in the reaction between sodium persulfate (SPS) and aniline under alkaline conditions, and the yields of OANIblue increase with increasing concentrations of phenol and pH values. Quenching experiments rule out the contributions of SO4Ë- and ËOH to aniline oxidation and imply that the non-radical activation of SPS is an important pathway in the formation of OANIblue. MALDI-TOF-MS analysis indicates that phenol apparently inhibits the polymerization degree of aniline in that the molecular weights of OANIblue gradually decrease from 1586.4 to 684.6 when phenol is increased from 0 to 2.0 mM. FTIR and Raman analyses confirm the structure of aniline oligomers in OANIblue and indicate that phenol inhibits the phenazine-like structure in OANIblue and facilitates the transformation of benzenoid rings to quinoid rings in the oxidation products. However, simultaneous activation of SPS by phenol and aniline is likely to occur in the reaction system with the formation of PhNHË, as indicated by DFT calculations. The high scavenging reactivity of phenol towards both PhNH2Ë+ and PhNHË implies that PhNH2Ë+ and PhNHË are not the intermediates in the formation of OANIblue. DFT calculations also reveal that apart from the one-electron transfer pathway between aniline and SPS, the two-electron transfer pathway is also likely to occur in the presence of phenol, resulting in the formation of PhNH+/PhNËË without producing PhNH2Ë+ and PhNHË. The produced PhNH+/PhNËË intermediates then couple with aniline, PhNH+, aminophenyl sulfate and its hydrolysate to form dimers, trimers, oligomers, and eventually OANIblue. This study not only describes a novel method to prepare water-soluble OANI, but also gives new insight on the importance of phenol in the production of OANIblue.
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Contaminantes Químicos del Agua , Purificación del Agua , Compuestos de Anilina/química , Oxidación-Reducción , Fenol/química , Fenoles , Sulfatos/química , Agua , Contaminantes Químicos del Agua/química , Purificación del Agua/métodosRESUMEN
Removal of organic solvents and heavy metals in effluents is of great significance to environmental pollution control and ecological civilization construction. pH-responsive materials have unique advantages in treating complicated oily wastewater. In this work, an intelligent pH-responsive nonwoven fabric with excellent reversible wettability was prepared. The pH-sensitive polymer was synthesized by free radical polymerization (FRP) technique, then dipped with SiO2 on PP fabric. The particular molecular structure of poly (dimethylaminoethyl methacrylate) (PDMAEMA) enabled the fabric surface to switch wettability rapidly between hydrophilic/underwater oleophobic and oleophobic/hydrophobic under pH stimulus and exhibit controllable selective separation of various oil/water mixtures. Furthermore, the fabric removed Pb2+ efficiently under a wide pH range. The experimetal results showed that the separation flux reached 19,229 ± 1656.43 L-h-1-m-2 for water and 19,342 ± 1796.77 L-m-2-h-1 for n-hexane. Besides, the obtained fabric effectively realized the separation and collection process of complex ternary mixtures. The fabric removed Pb2+ in solutions with efficiency up to 90.83%. After immersing in acid and alkali solutions for 24 h, no significant damage to the surface wettability. This economical and intelligent fabric is able to meet the different separation purposes of industrial wastewaters with complex compositions.
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Metales Pesados , Aguas Residuales , Álcalis , Concentración de Iones de Hidrógeno , Plomo , Metacrilatos , Polímeros , Dióxido de Silicio , Solventes , Aguas Residuales/química , HumectabilidadRESUMEN
BACKGROUND: Neurocysticercosis (NCC) is the most common helminthic infection of the central nervous system (CNS) caused by the larval stage of Taenia solium. Accurate and early diagnosis of NCC remains challenging due to its heterogeneous clinical manifestations, neuroimaging deficits, variable sensitivity, and specificity of serological tests. Next-generation sequencing (NGS)-based pathogen analysis in patient's cerebrospinal fluid (CSF) with NCC infection has recently been reported indicating its diagnostic efficacy. In this case study, we report the diagnosis of a NCC patient with a symptomatic history of over 20 years using NGS analysis and further confirmation of the pathology by immunological tests. CASE PRESENTATION: This study reports the clinical imaging and immunological features of a patient with a recurrent headache for more than 20 years, which worsened gradually with the symptom of fever for more than 7 years and paroxysmal amaurosis for more than 1 year. By utilizing NGS technique, the pathogen was detected in patient's CSF, and the presence of Taenia solium-DNA was confirmed by a positive immunological reaction to cysticercus IgG antibody in CSF and serum samples. The symptoms of the patient were alleviated, and the CSF condition was improved substantially after the anti-helminthic treatment. CONCLUSIONS: This study suggests that combining CSF NGS with cysticercus IgG testing may be a highly promising approach for diagnosing the challenging cases of NCC. Further studies are needed to evaluate the parasitic DNA load in patients' CSF for the diagnosis of disease severity, stage, and monitoring of therapeutic responses.
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Secuenciación de Nucleótidos de Alto Rendimiento , Neurocisticercosis , Pruebas Serológicas , Taenia solium , Animales , Anticuerpos Antihelmínticos/sangre , ADN de Helmintos/genética , Humanos , Técnicas de Diagnóstico Molecular , Neurocisticercosis/diagnóstico , Neurocisticercosis/inmunología , Neurocisticercosis/parasitología , Taenia solium/genética , Taenia solium/inmunologíaRESUMEN
BACKGROUND: Glial fibrillary acidic protein (GFAP) autoimmune astrocytopathy is characterized by GFAP autoantibody positive encephalitis, meningoencephalitis or meningoencephalomyelitis. The initial clinical presentation may be similar to central nervous system infections making early diagnosis challenging. CASE PRESENTATION: A Chinese female patient presented with subacute meningitis with symptoms of headache, vomiting, and fever. Cerebrospinal fluid (CSF) analysis showed monocytic pleocytosis, elevated protein level, low glucose level, and negative basic microbiological studies including Xpert MTB/RIF. Brain magnetic resonance imaging (MRI) showed bilateral cerebral cortical and white matter hyperintensities on FLAIR sequences. The patient was diagnosed with possible tuberculous meningitis and started on anti-tuberculosis therapy (ATT). Three months later, the patient developed cervical myelopathy and encephalopathy with persistent CSF pleocytosis. Five months later, tissue-based and cell-based assays demonstrated GFAP antibodies in blood and CSF. Her symptoms improved with repeated administration of intravenous immunoglobulin (IVIG) and corticosteroids. One-and-a-half -year follow-up showed neither clinical progression nor relapses. CONCLUSIONS: Anti-GFAP astrocytopathy should be included in the differential diagnosis of patients who present with subacute meningitis with negative microbiological studies and a progressive clinical course including encephalitis and/or myelitis.
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Astrocitos/patología , Enfermedades Autoinmunes del Sistema Nervioso/diagnóstico , Proteína Ácida Fibrilar de la Glía/inmunología , Mielitis/diagnóstico , Pueblo Asiatico , Autoanticuerpos/inmunología , Enfermedades Autoinmunes del Sistema Nervioso/tratamiento farmacológico , Enfermedades Autoinmunes del Sistema Nervioso/inmunología , Diagnóstico Diferencial , Femenino , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Imagen por Resonancia Magnética , Meningoencefalitis/diagnóstico , Meningoencefalitis/etiología , Meningoencefalitis/inmunología , Mielitis/etiología , Mielitis/inmunologíaRESUMEN
The MnII(HCO3-)-H2O2 (MnII-BAP) system shows high reactivity toward oxidation of electron-rich organic substrates; however, the predominant oxidizing species and its formation pathways involved in the MnII-BAP system are still under debate. In this study, we used the MnII-BAP system to oxidize As(III) in that As(III), Mn2+, and HCO3- are common components in As(III)-contaminated groundwater. Kinetic results show that MnII(HCO3-)n [including MnII(HCO3)+ and MnII(HCO3)2] is a key factor in the MnII-BAP system to oxidize As(III). Quenching experiments rule out contributions of OH⢠and 1O2 to As(III) oxidation and reveal that O2â¢- and the oxidizing species generated from O2â¢- play predominant roles in the oxidation of As(III). We further reveal that the MnO2+(HCO3-)n intermediate generated in the reaction between MnII(HCO3-)n and O2â¢-, instead of O2â¢-, is the predominant oxidizing species. Although CO3â¢- also contributes to As(III) oxidation, the high reaction rate constant between CO3â¢- and O2â¢- indicates that CO3â¢- is not the predominant oxidizing species in the As(III)-MnII-BAP system. In addition, the presence of Mn(III) further indicates the important Mn(II)-Mn(III) cycling in the MnII-BAP system. We therefore suggest two important roles of MnII(HCO3-)n in the MnII-BAP system: (i) MnII(HCO3-)n reacts with H2O2 to form the MnIII(HCO3)3 intermediate, followed by a subsequent reaction between MnIII(HCO3)3 and H2O2 to produce O2â¢-; (ii) MnII(HCO3-)n can also stabilize O2â¢- with the formation of MnO2+(HCO3-)n. MnO2+(HCO3-)n is an electrophilic reagent and plays the predominant role in the oxidation of As(III) to As(V).
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Bi2+xWO6 is a cost-effective and environmentally friendly photocatalyst that shows high reactivity in the oxidation of various contaminants under visible light. However, under alkaline conditions, the reactive oxidative species in the Bi2+xWO6 system are still not clear yet. In this study, it is observed that the oxidation rates of As(iii) increase with increasing pH values in the Bi2.15WO6 system. Photoluminescence and the Mott-Schottky analyses confirm that OH- promotes the separation and transfer of photogenerated electron-hole pairs over Bi2.15WO6, thus facilitating the oxidation of As(iii). Electron spin resonance spectra analysis and quenching experiments rule out contributions of â¢OH, O2Ë-, 1O2 and superoxo species to As(iii) oxidation and indicate that surface -OOH and/or H2O2 are indeed the predominant species under alkaline conditions. The improved production of H2O2 by H-donors such as glucose and phenol, as well as the UV-vis diffuse reflectance and Raman analyses, further confirms the formation of surface -OOH on Bi2.15WO6 under alkaline conditions. In the dark, the significant higher oxidation rate of As(iii) by H2O2-Bi2.15WO6 than that by H2O2 alone reveals that surface -OOH, instead of H2O2, plays an important role in As(iii) oxidation. This study enriches our understanding of the diversity of reactive oxygen species (ROS) in the Bi2.15WO6 system and gives new insight into the mechanism involved in the oxidation of As(iii) under alkaline conditions.
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Inspired by fish scales, this study prepares a thermo-responsive underwater oleophobic PNIPAM/PAN/TiO2 nanofibrous membranes by traditional electrospinning technique using poly-N-isopropylacrylamide (PNIPAM) and polyacrylonitrile (PAN). Thermal properties, mechanical properties, surface chemical composition, wettability, photocatalysis, and oil/water separation of PNIPAM/PAN/TiO2 membrane are explored compared to pure PNIPAM membrane. Result reveals that PAN/TiO2 compounds make PNIPAM membrane with a smaller fiber diameter of 141 nm and high tensile stress of 7.4 MPa, and also decompose 98% of rhodamine B after UV light radiation. This bioinspired design structure endows the membrane with superhydrophilicity with a low water contact angle, and underwater superoleophobicity with a high oil contact angle of 157° (petroleum ether) and 151° (dichloromethane). This membrane can efficiency separate oil/water mixture with a high separation efficiency. Moreover, the resultant PNIPAM/PAN/TiO2 membrane has the bionic fish scale structure, and has wettability respond at lower critical solution temperature making the water flux decreased from 10013 ± 367 L m-2·h-1 to 7713 ± 324 L m-2·h-1, and thus has a potential to be used in purification of reclaimed water and separation of oil from water.
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Nanofibras , Aceites , Resinas Acrílicas , Animales , TitanioRESUMEN
Objective To explore the clinical characteristics of autoimmune disease with dual seropositive antibodies of leucine-rich glioma inactivated 1(LGI1)and contactin-associated protein 2(Caspr2).Methods The clinical data of seven patients with dual seropositive LGI1 and Caspr2 antibodies who were admitted to the Neurology Department of Peking Union Medical College Hospital from July 2014 to December 2017 were retrospectively analyzed.Results Central,peripheral and autonomic nervous systems were all involved in the seven cases;100%(7/7)presented with insomnia,myokymia,neuropahic pain and hyperhydrosis;71%(5/7)showed memory decline or psychiatric and behavioral symptoms;57%(4/7)had urinary hesitation or constipation;and 43%(3/7)had seizure.Electromyography showed 100%(6/6) of the patients had prolonged afterdischarges following normal M waves and/or abnormal spontaneous firing.Electroencephalography revealed slow waves or basic rhythm slowing in 71%(5/7)of patients.Electrocardiography showed sinus tachycardia,axis deviation,and prolonged QT intervals in 71%(5/7)of patients.One patient died from arrhythmia before immunotherapy.One died from pulmonary infection after immunotherapy.Improvement with immunotherapy was documented in the other five cases.No relapse was noted during the 1-2-year follow-up.Conclusions Autoimmune disease with dual seropositive antibodies of LGI1 and Caspr2 can diffusely affect the central,peripheral,and autonomic nervous systems.The possibility of this disease should be considered in patients with acute and subacute onset of neuropsychiatric symptoms,especially in patients with accompanying insomnia,myokymia,and hyperhydrosis.
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Autoanticuerpos/sangre , Enfermedades Autoinmunes/inmunología , Proteínas de la Membrana/inmunología , Proteínas del Tejido Nervioso/inmunología , Proteínas/inmunología , Humanos , Péptidos y Proteínas de Señalización Intracelular , Estudios RetrospectivosRESUMEN
OBJECTIVE: To investigate the clinical features of children with anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis with normal or abnormal cranial magnetic resonance imaging (MRI) findings via a comparative analysis. METHODS: A retrospective analysis was performed for the clinical data of 33 children with anti-NMDAR encephalitis. The clinical features and prognosis were compared between the children with normal and abnormal cranial MRI findings. RESULTS: In the 33 children with anti-NMDAR encephalitis, the most common initial symptoms were seizures (61%) and involuntary movement (61%), followed by language disorder (54%), mental and behavioral abnormalities (52%), and disturbance of consciousness (30%). All children had positive anti-NMDAR antibody in the cerebrospinal fluid, and 29 children (88%) had positive serum antibody. Of all the children, 15 (46%) had increased leukocytes in the cerebrospinal fluid, 3 (9%) had an increase in protein, and 29 (88%) had positive oligoclonal band; 26 children (79%) had electroencephalographic abnormalities (epileptic wave, slow wave, or a combination of these two types of waves). One child experienced respiratory failure. One child was found to have germinoma in the sellar region during follow-up. Of all the 33 children, 13 (39%) had abnormal cranial MRI findings, with hypointensity or isointensity on T1W1 and hyperintensity on T2WI and T2-FLAIR; 2 children had dural enhancement. As for the location of lesion, 5 children (38%) had lesions in the temporal lobe, 3 (23%) in the frontal lobe, 3 (23%) in the basal ganglia, 2 (15%) in the parietal lobe, 2 (15%) in the occipital lobe, 2 (15%) in the brainstem, 1 (8%) in the thalamus, and 1 (8%) in the cerebellum. Among the 13 children with abnormal cranial MRI findings, 5 (38%) had lesions mainly in the grey matter and 8 (62%) had lesions mainly in the white matter. Compared with the children with normal cranial MRI findings, the children with abnormal cranial MRI findings had significantly higher proportion of children with prodromal infection, incidence rate of disturbance of consciousness, probability of recurrence, Glasgow score, incidence rate of increased leukocytes in the cerebrospinal fluid, and application rate of second-line treatment (P<0.05). CONCLUSIONS: Children with anti-NMDAR encephalitis and abnormal cranial MRI findings have certain clinical features, which may provide guidance for the evaluation of disease conditions and the selection of diagnostic and treatment measures.
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Encefalitis Antirreceptor N-Metil-D-Aspartato/diagnóstico por imagen , Encéfalo/diagnóstico por imagen , Imagen por Resonancia Magnética/métodos , Adolescente , Niño , Preescolar , Femenino , Humanos , MasculinoRESUMEN
OBJECTIVE: The objective of this study was to advance the characterization of seizure semiology in leucine-rich glioma-inactivated protein 1 (LGI1) antibody-associated limbic encephalitis (LE). METHODS: Eighteen patients diagnosed with LGI1 LE were identified. Seizure semiology, demographic features, MRI and fluorodeoxyglucose positron emission tomography (FDG-PET), electroencephalograms, and outcomes following immunotherapy were evaluated. RESULTS: Patients were divided into the following groups based on seizure semiology: faciobrachial dystonic seizure only (FBDS-only, n=4), epileptic seizure without FBDS (Non-FBDS, n=6), and FBDS plus epileptic seizure (FBDS+, n=8). In the group with Non-FBDS, the majority of patients (5/6) manifested mesial temporal lobe epilepsy (MTLE) like semiology (i.e., fear, epigastric rising, staring, and automatisms) with a frequency of 7±5 times per day and a duration of 15.3±14.3s. In the group with FBDS+, the distinctive symptom was FBDS followed by epileptic events, especially automatisms (7/8), with a frequency of 16±12 times per day and a duration of 13.0±8.0s. In these cases, 67% and 50% of the patients showed abnormalities on MRI and FDG-PET, respectively, and the mesial temporal lobe structures were most often involved. Ictal discharges were observed in 0/4, 6/6, and 8/8 of the patients in the groups with FBDS only, Non-FBDS, and FBDS+, respectively. The temporal lobe was mainly affected. Immunotherapy had favorable therapeutic effects. SIGNIFICANCE: The LGI1 LE should be considered as one disease syndrome with a series of clinical manifestation. Identifying types of unique semiology features will facilitate the early diagnosis and the timely initiation of immunotherapy.
Asunto(s)
Autoanticuerpos , Encefalitis Límbica/complicaciones , Proteínas/inmunología , Convulsiones/etiología , Adulto , Anciano , Electroencefalografía/métodos , Femenino , Humanos , Inmunoterapia , Péptidos y Proteínas de Señalización Intracelular , Encefalitis Límbica/inmunología , Encefalitis Límbica/terapia , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Convulsiones/inmunología , Convulsiones/terapia , Resultado del TratamientoRESUMEN
BACKGROUND: Morvan syndrome is a rare disorder characterized by the combination of peripheral nerve hyperexcitability, encephalopathy and dysautonomia with marked insomnia. It was reported to have association to antibodies to voltage-gated potassium channels including contactin associated protein-like 2 antibodies (CASPR2-Ab) and leucine-rich glioma inactivated protein 1 antibodies (LGI1-Ab). LGI1-Ab was reported to associate with seizures, amnesia, confusion, hyponatraemia and a good prognosis, while CASPR2-Ab with peripheral presentations, probable risk for tumor and a poor prognosis. The vast majority of Morvan syndrome patients were male, with normal magnetic resonance imaging of the brain. CASE PRESENTATION: We report a female case presenting with a combination of bilateral leg pain, widespread myokymia, memory disturbance, seizure, hyperhidrosis and insomnia. She had antibodies targeting CASPR2 and LGI1, tested by the indirect immunofluorescence test, which demonstrated the diagnosis of typical Morvan syndrome as well as classical limbic encephalitis. Cranial MRI revealed bilateral hyper-intensity of the medial temporal lobe, insular lobe and basal ganglia on T2/FLAIR and DWI sequence. As the treatment carried on, her serum LGI1-Ab disappeared and her memory loss, seizure and confusion quickly relieved. But her peripheral presentations did not relieve until serum CASPR2-Ab turned negative. Intravenous immunoglobulin treatment showed limited efficacy while she achieved almost complete remission with corticosteroids therapy. CONCLUSIONS: This case provides a rare female resource of Morvan syndrome, which is the first patient with both CASPR2-Ab and LGI1-Ab positive Morvan syndrome in China and one of the few female patients with Morvan syndrome reported so far. Through the detailed analysis of her clinical course, the diverse and overlapping clinical phenotype of CASPR2-Ab and LGI1-Ab in patients with Morvan syndrome was obvious and interesting.
Asunto(s)
Encefalitis Límbica/inmunología , Proteínas de la Membrana/inmunología , Proteínas del Tejido Nervioso/inmunología , Siringomielia/inmunología , Adulto , Amnesia/etiología , Autoanticuerpos/sangre , China , Femenino , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Imagen por Resonancia Magnética , Dolor/etiología , Canales de Potasio con Entrada de Voltaje , Convulsiones/etiología , Siringomielia/fisiopatologíaRESUMEN
Two new 28-nor-oleanane-type triterpene saponins, oleiferoside U (1), and oleiferoside V (2) were isolated from the 50% EtOH extract of the roots of Camellia oleifera C. Abel. Their structures were elucidated as camellenodiol 3ß-O-ß-d-galactopyranosyl-(1â2)-ß-d-xylopyranosyl-(1â2)-[ß-d-galactopyranosyl-(1â3)]-ß-d-glucuronopyranoside and camellenodiol 3ß-O-ß-d-galactopyranosyl-(1â3)-ß-d-xylopyranosyl-(1â2)-[ß-d-galactopyranosyl-(1â3)]-ß-d-glucuronopyranoside. Their chemical structures were established mainly on the basis of integrated spectroscopic techniques. In vitro, cytotoxic activities of the two new triterpene saponins were evaluated against three human tumor cell lines (A549, SMMC-7721, and MCF-7) using the MTT assay. Both of them showed a certain cytotoxic activities toward the tested cell lines and gave IC50 values in the range of 45.04-63.22 µM.