ObjectiveTo study on the different therapeutic effects and potential mechanisms of Rhei Radix et Rhizoma（RH） before and after steaming with wine on constipation model mice. MethodsFifty-four male ICR mice were randomly divided into control group， model group， lactulose group（1.5 mg·kg^-1）， high， medium and low dose groups of RH and RH steaming with wine（PRH）（8， 4， 1 g·kg^-1）. Except for the control group， the constipation model was replicated by gavage of loperamide hydrochloride（6 mg·kg^-1） in the other groups. After 2 weeks of modeling， each administration group was gavaged with the corresponding dose of drug solution， and the control and model groups were given an equal volume of normal saline， 1 time/d for 2 consecutive weeks. After administration， the feces were collected for 16S rRNA sequencing， the levels of gastrin（GAS）， motilin（MTL）， interleukin-6（IL-6）， γ-interferon（IFN-γ） in the colonic tissue were detected by enzyme-linked immunosorbent assay（ELISA）， the histopathological changes of colon were observed by hematoxylin-eosin（HE） staining， flow cytometry was used to detect the proportion changes of CD4⁺， CD8⁺ and regulatory T cell（Treg） in peripheral blood. ResultsCompared with the control group， the model group showed significantly decrease in fecal number in 24 h， fecal quality and fecal water rate（P<0.01）， the colon was seen to have necrotic shedding of mucosal epithelium， localized intestinal glands in the lamina propria were degenerated， necrotic and atrophied， a few lymphocytes were seen to infiltrate in the necrotic area in a scattered manner， the contents of GAS and MTL， the proportions of CD4⁺， CD8⁺ and Treg were significantly reduced（P<0.01）， the contents of IL-6 and IFN-γ were significantly elevated（P<0.05， P<0.01）. Compared with the model group， the fecal number in 24 h， fecal quality and fecal water rate of high-dose groups of RH and PRH were significantly increased（P<0.05， P<0.01）， the pathological damage of the colon was alleviated to varying degrees， the contents of GAS， MTL， IL-6 and IFN-γ were significantly regressed（P<0.05， P<0.01）， and the proportions of CD4⁺ and CD8⁺ were significantly increased（P<0.01）， although the proportion of Treg showed an upward trend， there was no significant difference. In addition， the results of intestinal flora showed that the number of amplicon sequence variant（ASV） and Alpha diversity were decreased in the model group compared with the control group， and there was a significant difference in Beta diversity， with a decrease in the relative abundance of Lactobacillus and an increase in the relative abundances of Bacillus and Helicobacter. Compared with the model group， the ASV number and Alpha diversity were increased in the high-dose groups of RH and PRH， and there was a trend of regression of Beta diversity to the control group， the relative abundance of Lactobacillus increased， and the relative abundances of Bacillus and Helicobacter decreased. ConclusionRH and PRH can improve dysbacteriosis， promote immune system activation， inhibit the release of inflammatory factors for enhancing the gastrointestinal function， which may be one of the potential mechanisms of their therapeutic effect on constipation.

Myasthenia gravis (MG) is an autoimmune disorder mediated by autoantibodies that target the neuromuscular junction. The detection of pathogenic autoantibodies is crucial for the diagnosis and assessment of the disease. Commercial testing institutions and hospitals commonly use techniques such as radioimmunoprecipitation, enzyme-linked immunosorbent assay, or cell-based assay to test the serum of MG patients. However, these methods have limitations in terms of sensitivity and specificity, and there is a lack of direct comparative studies using high-level evidence-based medicine. The recent release of the SCREAM study using data from Chinese individuals confirms the advantages of cell-based assay, providing level Ⅰ evidence for the preferred recommendation of MG laboratory diagnosis and filling the gap in both domestic and international fields. However, the results obtained from the aforementioned testing methods are difficult to use for longitudinal comparison of patients and may not serve as the best biomarkers for disease monitoring. This article attempts to discuss this question by combining recent advances in understanding the pathogenic autoantibodies of MG and provides an outlook on future developments.

ObjectiveTo explore the scientific connotation of fried charcoal survivability of Lonicerae Japonicae Flos（LJF） by analyzing the correlation between the color change and the intrinsic components during the processing of LJF Carbonisata（LJFC）， and taking pH， charcoal adsorption and microscopic characteristics as indexes. MethodLJFC samples with different degrees of processing were prepared according to the stir-frying time of 0.0， 1.5， 3.0， 4.5， 6.0， 7.5， 9.0， 10.5 min（numbered S1-S8）， and the contents of gallic acid， chlorogenic acid， cryptochlorogenic acid， rutin， luteoloside， isochlorogenic acid A and isochlorogenic acid C were determined by high performance liquid chromatography（HPLC）， and the L^*（brightness）， a^*（red-greenness） and b^*（yellow-blueness） of LJFC samples with different degrees of processing were determined by spectrophotometer， and the correlation analysis and principal component analysis（PCA） between the contents of seven representative components and the color of the samples were carried out by SPSS 26. 0 and SIMCA-P 14.1. Then pH， adsorption force and characteristic structure of different samples of LJFC were detected and the processing pattern of LJFC was analyzed. ResultThe results of quantitative analysis revealed that the contents of luteoloside， rutin， chlorogenic acid and isochlorogenic acid A gradually decreased， and the contents of cryptochlorogenic acid， isochlorogenic acid C and gallic acid firstly increased and then decreased. The L^* and b^* of the sample powders decreased， and a^* showed a trend of increasing and then decreasing. The L^* and b^* were positively correlated with the contents of chlorogenic acid， rutin， luteoloside， isochlorogenic acid A， b^* was positively correlated with the content of gallic acid， and a^* was positively correlated with the contents of cryptochlorogenic acid and isochlorogenic acid C. PCA revealed that samples could be clearly divided into 3 groups， S1-S2 as one group， S3-S5 as one group， and S6-S8 as one group， with S3 having the highest score. The results of regression analysis showed that only isochlorogenic acid C could be used to predict the contents of components by colorimetric values combined with regression equations. Physicochemical analysis showed that pH of LJFC increased with the increase of degree of charcoal stir-frying， while adsorption force showed a tendency of increasing and then decreasing， with the highest adsorption force in the S5 sample， and the non-glandular hairs， calcium oxalate clusters and pollen grains had a varying degree of decreasing with the deepening of processing degree， and the microstructures of S6-S8 samples were obviously charred with pollen grains almost invisible. ConclusionThe changes in chemical composition and color characteristics of LJFC during the processing have certain correlations， combined with the changes in physicochemical properties， S5 sample is found to be the optimal processed products， which can provide a reference for the processing standardization and quality evaluation of LJFC， and enrich the scientific connotation of fried charcoal survivability of LJF.

ObjectiveTo investigate the therapeutic effects and difference in the effects of Arisaematis Rhizoma （AR） before and after processing （i.e.， Arisaematis Rhizoma Preparatum， ARP） with Zingiberis Rhizoma Recens-Alumen on allergic asthma in rats and to provide a basis for the theory of processing improving the efficacy. MethodA rat model of allergic asthma was established in 70 SD rats by intraperitoneal injection of ovalbumin （OVA）-aluminum hydroxide. The rats were administrated with the aqueous extracts of AR （1.2， 0.3 g∙kg^-1） and ARP （1.2， 0.3 g∙kg^-1） aqueous extracts by gavage， and montelukast sodium （0.001 g∙kg^-1） was used as the positive drug. The T helper cell type 1/type 2 （Th1/Th2） ratio in the serum and bronchoalveolar lavage fluid （BALF） and percentages of inflammatory cells in BALF were determined. Polymerase chain reaction （PCR） was employed to determine the mRNA level of mucin 5AC （MUC5AC） in the lung tissue. The pathological changes in the lung tissue were observed by hematoxylin-eosin （HE） staining and PAS staining. Immunohistochemical assay was employed to measure the expression of c-Jun amino-terminal kinase （JNK）， extracellular signal regulated protein kinase （ERK）， and p38 mitogen-activated protein kinase （p38 MAPK） in rat lung tissue. Western blot was employed to determine the protein levels of ERK， p-ERK， JNK， p-JNK， p38， p-p38 in the lung tissue. The effects of AR and ARP were compared based on overall desirability. ResultCompared with the blank group， the levels of interleukin-12 （IL-12） and γ interferon （IFN-γ） in serum and BALF of rats in the model group were significantly lower （P<0.05， P<0.01）， and the levels of interleukin-4 （IL-4）， interleukin-5 （IL-5） and interleukin-13 （IL-13） were significantly higher （P<0.05， P<0.01）. Compared with the model group， the serum and BALF contents of IL-12 and IFN-γ in rats in the montelukast sodium group， high-dose AR group and high-dose ARP group were significantly higher （P<0.05， P<0.01）， and the contents of IL-4， IL-5 and IL-13 were significantly lower （P<0.05， P<0.01）， and the serum contents of IFN-γ in rats in the low-dose AR group and low-dose ARP group were in BALF was significantly higher （P<0.05） and IL-4 and IL-13 were significantly lower （P<0.05， P<0.01）， the percentages of macrophages， lymphocytes， neutrophils， and eosinophils were reduced in BALF， and the expression of JNK/ERK/p38 MAPK signaling pathway and MUC5AC protein was inhibited in lung tissues. Overall assessment of the normalized analysis revealed that the ARP group was slightly more potent than the AR group after administration of the same dose. ConclusionAR and ARP can effectively treat allergic asthma by inhibiting JNK/ERK/p38 MAPK signaling pathway， and the effect is better after concoction， which can provide data support for its "concoction efficiency".

Myotonic dystrophy type 1(DM1)is a multisystem trinucleotide repeat expansion disorder usually referred to the department of neurology with complaints of progressive muscle weakness and myotonia.This article reported a 33-year-old female patient with DM1 presenting with acute respiratory failure.Muscle bi-opsy in vastus lateralis showed significantly increased internal nuclei.Genetic test show CTG repeat expansions with the size of(847±76)in dystrophia myotonica protein kinase(DMPK)gene on chromosome 19.This case report broadens the clinician's understanding of the atypical clinical manifestations of DM1,so as to avoid missed diagnosis and misdiagnosis.

The clinical symptoms of myasthenia gravis (MG) in women are closely related to pregnancy, and the outcome of pregnancy and neonates is related to MG disease control, comorbidities and antibody types. The exacerbation of muscle weakness usually appeared in the first trimester and postpartum 0-3 months. The adverse pregnancy outcomes of MG mothers are mainly fetal arrest and abortion. Neonatal muscle weakness and congenital joint flexion are common neuromuscular lesions in childbirth. During pregnancy, the immune system of MG pregnant women is regulated by estrogen/progesterone/prolactin/glucocorticoid, and the main result is to inhibit pro-inflammatory response and promote immune tolerance. However, the specific interaction between MG and pregnancy remains to be elucidated in prospective cohort studies.

Objective:To investigate the detecting method and clinical characteristics of anti-nodal/paranodal antibodies in chronic inflammatory demyelinating polyradiculopathy.Methods:Serum samples were collected from 212 patients with chronic inflammatory demyelinating polyradiculopathy who were admitted to Huashan Hospital of Fudan University or from other clinical centers from January 2018 to July 2021. Autoantibodies (anti-NF155, anti-NF186, anti-CNTN1) and IgG subtypes were detected with cell-based assay. According to the test results, patients were divided into anti-NF155 positive group, anti-NF186 positive group and anti-CNTN1 positive group, clinical characteristics of patients in each group, including limb weakness, superficial sensation and proprioception, tremor, cerebrospinal fluid protein level, brachial plexus magnetic resonance (MRI) were retrospectively analyzed and compared.Results:A total of 23 patients (10.8%,23/212) were positive for anti-NF155 antibody, 12 (5.7%,12/212) for anti-NF186 antibody, and 4 (1.9%,4/212) for anti-CNTN1 antibody. IgG 4 was the predominant subtype in anti-NF155 and anti-CNTN1 groups. In the anti-NF186 group, all cases were IgG positive and antibody subtypes could be detected in 4 cases (4/12). In anti-NF155 group, 23 patients (100%,23/23) had limb weakness and deep sensory disturbance, 19 patients (82.6%,19/23) had superficial sensory disturbance, 22 patients (95.7%,22/23) were symmetrically involved, 18 patients (78.3%,18/23) showed tremor, 19 patients (19/19) showed abnormal in brachial plexus MRI. In anti-NF186 group, 12 patients had limb weakness (12/12), 9 patients (9/12) and 6 patients (6/12) had superficial sensory disturbance and deep sensory disturbance respectively, 8 patients (8/12) were asymmetrically involved, and only 1 patient (1/12) showed tremor, 1 (1/7) showed abnormal brachial plexus MRI. In anti-CNTN1 group, 4 cases showed symmetrical limb weakness and sensory disturbance, 3 patients had tremor, and four patients showed brachial plexus MRI abnormality. There were statistically significant differences in onset age, proprioception, tremor and MRI abnormalities of brachial plexus among the 3 groups ( P<0.01). Conclusions:The clinical characteristics of CIDP patients with anti-NF155, anti-NF186 and anti-CNTN1 antibodies are different. Screening anti-nodal/paranodal antibodies is of great significance for accurate diagnosis and treatment of patients with peripheral neuropathy.

Localized peripheral neuropathy amyloidosis is a rare disease that mainly occurred in elder people who present with focal neurological symptoms. AL is the main type of amyloid protein. Biopsy is the golden standard for diagnosis. Mass spectrometry and immunohistochemical analysis help to confirm the type of amyloid protein. This paper retrospectively analyzes the clinical and imaging data, auxiliary examinations, histological, and immunohistochemical markers. The patient, a 34-year-old woman, presented with a right neck mass and weakness of the right arm. Brachial plexus magnetic resonance imaging (MRI) showed a tumor-like lesion in the nerve root at C5 and C6 and in upper trunk. Electrophysiological studies revealed damage in the upper trunk of the brachial plexus. Positive staining with Congo red was found in brachial plexus biopsy. Mass spectrometry showed that the type of amyloid protein was AHL(G-λ). The patient underwent nerve graft for treatment. Meanwhile, literature review revealed that the average onset age of localized spinal nerve amyloidosis was 62.4 years old.The radial nerve was the most susceptible, followed by the lumbosacral plexus. Fifty percent of the type of amyloid protein is AL.Until now, no consolidated treatment is available. Here, we summarize the clinical characteristics of localized peripheral neuropathy amyloidosis in order to raise the awareness of the disease.

Objective:To investigate the clinical features, imaging features and gene mutation of a paitent with alanyl-transfer ribonucleic acid synthetase 2 (AARS2) gene mutation- related leukodystrophy and further improve the understanding of this rare disease.Methods:Clinical data of a patient with leukodystrophy associated with AARS2 gene mutation diagnosed in October 2020 at Xiamen Hospital of Beijing University of Chinese Medicine and Huashan Hospital of Fudan University were collected.Results:The male patient, 25 years old, was admitted with the clinical manifestations, including chronic onset dyskinesia, ataxia, nystagmus and psoriasis. Head magnetic resonance imaging (MRI) showed bilateral white matter lesions and cerebellar atrophy. Spine MRI showed vertebral body incomplete fusion. Gene detection showed heterozygous compound AARS2 gene mutation [c.985C>T chr6:44275041(p.R329C) and c.452T>C chr6:44279256(p.M151T)].Conclusions:AARS2 gene mutation-related leukodystrophy is a rare mitochondrial disease in clinical practice. The patient presented with progressive motor deficits in the lower limbs, ataxia, relatively retained cognitive function. MRI revealed abnormal symmetry of corpus callosum and bilateral paraventricular white matter. Heterozygous compound AARS2 gene mutations [c.985C>T chr6:44275041 (p.R329C) and c.452T>C chr6:44279256 (p.M151T)] are one of the pathogenic factors leading to hereditary leukodystrophy.

Leukoencephalopathy with ataxia (LKPAT) is a rare autosomal recessive disorder caused by mutations of CLCN2 gene. LKPAT is clinically characterized by cerebellar ataxia, headache and cognition impairment. Brain magnetic resonance imaging showed characteristic hyperintensities along the pyramidal fiber tracts. Few cases have been reported so far. This article reported the clinical data of a 48 years old female patient with LKPAT for clinical reference.