Explore the vast range of titles available.

Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory disease preferentially affects the optic nerve and the spinal cord. The first attack usually occurs in the third or fourth decade, though patients with disease onset in the fifties or later are not uncommon. This study aimed to investigate the clinical characteristics and prognosis in patients with different age of onset and to explore the correlations between age of onset and clinical characteristics and prognostic outcomes. We retrospectively reviewed the medical records of 298 NMOSD patients diagnosed according to the 2015 updated version of diagnostic criteria. Patients were divided into early-onset NMOSD (EO-NMOSD) (<50 years at disease onset) and late-onset NMOSD (LO-NMOSD) (≥50 years at disease onset) based on the age of disease onset. LO-NMOSD patients were divided into two subgroups: relative-late-onset NMOSD (RLO-NMOSD) (50~70 years at disease onset) and very-late-onset NMOSD (≥70 years at disease onset). Clinical characteristics, laboratory findings, neuroimaging features, and prognostic outcomes were investigated. Compared to EO-NMOSD patients, patients with LO-NMOSD showed more frequent transverse myelitis (TM) (58.20% vs. 36.00%, = 0.007) while less frequent optic neuritis (ON) (23.10% vs. 34.80%, = 0.031) and brainstem/cerebral attacks (7.50% vs. 18.30%, = 0.006) as the first attack. Patients with LO-NMOSD showed less frequent relapses, higher Expanded Disability Status Scale (EDSS) score at the last follow-up, fewer NMOSD-typical brain lesions, and longer segments of spinal cord lesions. Patients with older onset age showed a higher proportion of increased protein levels in cerebrospinal fluid during the acute phase of attacks. Age at disease onset positively correlated with length of spinal cord lesions at first attack and at last follow-up, negatively correlated with ARR-1 (ARR excluding the first attack, calculated from disease onset to final follow-up), irrespective of AQP4-IgG serostatus. Patients with older age at disease onset progressed to severe motor disability sooner, and age of onset positively correlated with EDSS score at the last follow-up, irrespective of AQP4-IgG serostatus. Age of disease onset affects clinical characteristics and prognosis outcomes of patients with NMOSD.

To decipher the discrepancies between muscle-specific kinase antibody-positive myasthenia gravis (MuSK-MG) and double-seropositive myasthenia gravis (DSP-MG), and to determine prognostic factors for minimal manifestation status (MMS) achievement in MG patients with MuSK autoantibodies (MuSK-Ab). A total of 34 MG patients seropositive for MuSK-Ab were enrolled in this study. The demographic and clinical features were compared between MuSK-MG (n = 28) and DSP-MG (n = 6) patients, and factors affecting MMS induction in all patients with MuSK-Ab were identified using Cox regression analysis. Compared to MuSK-MG patients, those with DSP-MG had similar clinical characteristics, except that they had a lower frequency of bulbar muscle involvement at nadir (50% vs 92.9%; P = 0.029) and higher proportions of comorbidities with diabetes mellitus (33.3% vs 0%; P = 0.027) and thymic abnormalities (33.3% vs 0%; P = 0.027). Higher MG Activities of Daily Living (MG-ADL) scores (HR = 0.16, 95% CI: 0.037-0.7, P = 0.015) and axial muscle involvement at nadir (HR = 0.39, 95% CI: 0.16-0.94, P = 0.035) were negative prognostic factors for MMS achievement in patients with MuSK-Ab regardless of acetylcholine receptor antibody (AChR-Ab) positivity. Multivariable Cox regression analysis further established higher MG-ADL scores at the nadir (HR = 0.19, 95% CI: 0.04-0.94; P = 0.042) as an independent risk factor for MMS achievement. DSP-MG was comparable to MuSK-MG and could be considered a single entity in our cohort. In all MG patients with MuSK-Ab, a higher MG-ADL score at nadir may herald a lower chance of MMS achievement, with no observed potential effect of AChR-Ab presence.

BackgroundAbnormal expanded GGC repeats within the NOTCH2HLC gene has been confirmed as the genetic mechanism for most Asian patients with neuronal intranuclear inclusion disease (NIID). This cross-sectional observational study aimed to characterise the clinical features of NOTCH2NLC-related NIID in China.MethodsPatients with NOTCH2NLC-related NIID underwent an evaluation of clinical symptoms, a neuropsychological assessment, electrophysiological examination, MRI and skin biopsy.ResultsIn the 247 patients with NOTCH2NLC-related NIID, 149 cases were sporadic, while 98 had a positive family history. The most common manifestations were paroxysmal symptoms (66.8%), autonomic dysfunction (64.0%), movement disorders (50.2%), cognitive impairment (49.4%) and muscle weakness (30.8%). Based on the initial presentation and main symptomology, NIID was divided into four subgroups: dementia dominant (n=94), movement disorder dominant (n=63), paroxysmal symptom dominant (n=61) and muscle weakness dominant (n=29). Clinical (42.7%) and subclinical (49.1%) peripheral neuropathies were common in all types. Typical diffusion-weighted imaging subcortical lace signs were more frequent in patients with dementia (93.9%) and paroxysmal symptoms types (94.9%) than in those with muscle weakness (50.0%) and movement disorders types (86.4%). GGC repeat sizes were negatively correlated with age of onset (r=−0.196, p<0.05), and in the muscle weakness-dominant type (median 155.00), the number of repeats was much higher than in the other three groups (p<0.05). In NIID pedigrees, significant genetic anticipation was observed (p<0.05) without repeat instability (p=0.454) during transmission.ConclusionsNIID is not rare; however, it is usually misdiagnosed as other diseases. Our results help to extend the known clinical spectrum of NOTCH2NLC-related NIID.

An MR scan of brain at another hospital showed asymmetric confluent white matter hyperintensities, mild temporal lobe atrophy and normal intracranial arteries (figure 1). Cognitive assessment identified impaired temporal and spatial orientation and problems with long-term and short-term memory. [...]the initial investigation should include investigations for these causes, including repeat MR scan of brain with contrast and cerebrospinal fluid (CSF) examination. The following investigations were normal or negative: full blood count, urine and stool analysis, liver and renal function, cardiac enzymes, electrolytes, blood glucose, lipids, coagulation studies, thyroid function, homocysteine, vitamins, serum tumour markers, paraneoplastic antibodies (anti-Hu, Yo, Ri, Ma2/Ta, CV2/CRMP5 and amphiphysin), lupus panel, perinuclear antineutrophil cytoplasmic antibody (pANCA), cytoplasmic antineutrophil cytoplasmic antibody (cANCA), erythrocyte sedimentation rate (ESR), C reactive protein, procalcitonin (PCT), ACE, T cell based interferon-gamma release assay (T-SPOT.TB), tuberculosis (TB) antibodies, HIV, syphilis, JC virus, chest X-ray and ECG.

The present study aimed to summarize the clinical features, alterations in cerebrospinal fluid (CSF), imaging characteristics, diagnostic methods, treatment regimens and outcomes of adult Chinese patients with tuberculous meningitis (TBM). Clinical data of 189 cases with 4 cases confirmed with definite TBM, 65 cases of probable (diagnostic score, ≥12 with imaging or ≥10 without imaging) and 120 cases of possible (diagnostic score, 6-11 with imaging or 6-9 without imaging) TBM admitted to Xiangya Hospital of Central South University between January 2009 and January 2015 were investigated retrospectively. Data on the clinical, laboratory and demographic characteristics of patients, as well as the results of radiological investigations and the clinical outcome, were collected for all patients. A total of 89.9% patients illustrated symptoms of acute or sub-acute TBM. The most frequent symptoms and signs were fever (78.3%), headache (89.2%), decreased level of consciousness (48.1%), meningeal irritation (73%), impairment of cranial nerve function and increased intracranial pressure (60%). The CSF protein concentration was significantly elevated and CSF glucose was greatly decreased in these cases. Imaging data were available for 144 cases, with 66 cases presenting abnormal chest X-ray or computed tomography findings, and 127 cases presenting abnormal brain magnetic resonance imaging findings among the 144 patients examined. All patients received anti-tuberculosis (TB) therapy, while 7 patients underwent neurosurgical drainage due to hydrocephalus and 3 patients succumbed to the disease. Among the survivors, 87% presented significant improvement. In conclusion, the diagnosis of TBM should combine clinical manifestations, CSF examination and the effect of anti-TB therapy. Differential diagnosis and trial anti-TB therapy may be of help for diagnosis. Positive CSF smear, CSF culture and biopsy of the brain, or biopsy of meninges are golden standards for the diagnosis of TBM. Early diagnosis and treatment are very important for improving the outcome.

Background A novel class of transcripts, long non-coding RNAs (lncRNAs), has recently emerged as a key player in several biological processes, and important roles for these molecules have been reported in a number of complex human diseases, such as autoimmune diseases, neurological disorders, and various cancers. However, the aberrant lncRNAs implicated in myasthenia gravis (MG) remain unknown. The aim of the present study was to explore the abnormal expression of lncRNAs in peripheral blood mononuclear cells (PBMCs) and examine mRNA regulatory relationship networks among MG patients with or without thymoma. Methods Microarray assays were performed, and the outstanding differences between lncRNAs or mRNA expression were verified through RT-PCR. The lncRNAs functions were annotated for the target genes using Gene Ontology (GO) and the Kyoto Encyclopedia of Genes and Genomes (KEGG) biological pathway. The potential regulatory relationships between the lncRNAs and target genes were analyzed using the ‘cis’ and ‘trans’ model. Outstanding lncRNAs were organized to generate a TF-lncRNA-gene network using Cytoscape software. Results The lncRNA and mRNA expression profile analysis revealed subsets of differentially expressed genes in MG patients with or without thymoma. A total of 12 outstanding dysregulated expression lncRNAs, such as lncRNA oebiotech_11933, were verified through real-time PCR. Several GO terms including the cellular response to interferon-γ, platelet degranulation, chemokine receptor binding and cytokine interactions were very important in MG pathogenesis. The chromosome locations of some lncRNAs and associated co-expression genes were demonstrated using ‘cis’ analysis. The results of the ‘trans’ analysis revealed that some TFs (i.e., CTCF, TAF1and MYC) regulate lncRNA and gene expression. The outstanding lncRNAs in each group were implicated in the regulation of the TF-lncRNA-target gene network. Conclusion The results of the present study provide a perspective on lncRNA expression in MG. We identify a subset of aberrant lncRNAs and mRNAs as potential biomarkers for the diagnosis of MG. The GO and KEGG pathway analysis provides an annotation to determine the functions of these lncRNAs. The results of the ‘cis’ and ‘trans’ analyses provide information concerning the modular regulation of lncRNAs.

A water-soluble polysaccharide from Sarcodia ceylonensis was obtained by using the method of water-extraction and ethanol-precipitation. The polysaccharide was further purified by chromatography on AB-8 and ADS-7 columns, yielding a pure polysaccharide termed SCP-60. The molecular weight (Mw) of SCP-60 was calculated to be 50.0 kDa, based on the calibration curve obtained with a series of Dextran T standards. The results of FT-IR indicated that the polysaccharide contains the α-configuration of sugar units. GC-MS analysis revealed that SCP-60 was mainly composed of galactose and glucose. NMR spectroscopy revealed SCP-60 had the backbone consisting of →6)-α-Manp-(1→, α-d-Glcp-(1→, →6)-α-d-Glcp-(1→ and →6)-α-Galp-(1→. In order to evaluate the antitumor activity in vivo of the polysaccharide, a sarcoma 180 model was used. The results showed SCP-60 had strong antitumor ability, meanwhile, SCP-60 at a high dose (100 mg/kg) could significantly increase the thymic and splenic indices of S180 mice, and strongly promote the secretion of IL-2, TNF-α and IFN-γ, increase the SOD activities and reduce the concentrations of MDA in blood. Therefore the polysaccharide SCP-60 should be explored as a novel potential antitumor drug.

Dendrobium devonianum Paxt ( D. devonianum ) is widely used as a healthy food and traditional Chinese medicine. It is rich in bioactive polysaccharides, but the acidic polysaccharide have not been reported. In this research, the enzyme-assisted extraction and structure characterizations of an acid polysaccharide (DDPp2) and its antioxidant activities were studied. The results showed the optimum extraction conditions were at enzyme concentration of 0.94% at 51.06 °C and pH 4.94. The molecular weight of DDPp2 was 306 kDa and composed of four monosaccharides in the molar ratio of mannose:glucose:galactose:glucuronic acid = 6.37:4.99:1.71:2.39 with α-configuration sugar. SEM and TEM analysis showed that the surface of the polysaccharide was smooth and holes in the sheet and multiple molecular chains were intertwined with each other. Free radical scavenging tests in vitro showed DDPp2 had very strong scavenging abilities for hydroxyl radical, ABTS radical and superoxide anion free radicals. Cell experiment showed DDPp2 had no cytotoxicity and could significantly increase the activities of CAT, SOD and GPX, reduce the content of MDA in RAW264.7 cells. The investigation showed that DDPp2 is a potential antioxidant.

In the realm of autonomous driving, robust perception under out-of-distribution conditions is paramount for the safe deployment of vehicles. Challenges such as adverse weather, sensor malfunctions, and environmental unpredictability can severely impact the performance of autonomous systems. The 2024 RoboDrive Challenge was crafted to propel the development of driving perception technologies that can withstand and adapt to these real-world variabilities. Focusing on four pivotal tasks -- BEV detection, map segmentation, semantic occupancy prediction, and multi-view depth estimation -- the competition laid down a gauntlet to innovate and enhance system resilience against typical and atypical disturbances. This year's challenge consisted of five distinct tracks and attracted 140 registered teams from 93 institutes across 11 countries, resulting in nearly one thousand submissions evaluated through our servers. The competition culminated in 15 top-performing solutions, which introduced a range of innovative approaches including advanced data augmentation, multi-sensor fusion, self-supervised learning for error correction, and new algorithmic strategies to enhance sensor robustness. These contributions significantly advanced the state of the art, particularly in handling sensor inconsistencies and environmental variability. Participants, through collaborative efforts, pushed the boundaries of current technologies, showcasing their potential in real-world scenarios. Extensive evaluations and analyses provided insights into the effectiveness of these solutions, highlighting key trends and successful strategies for improving the resilience of driving perception systems. This challenge has set a new benchmark in the field, providing a rich repository of techniques expected to guide future research in this field.