BACKGROUND:The severity of myasthenia gravis (MG) varies significantly among individuals, yet the factors underlying this heterogeneity are not fully understood. This study aimed to investigate the association between demographic, clinical, and environmental factors and the severity of MG. METHODS:From February 2017 to December 2021, adult patients registered in the national MG database were enrolled in this cross-sectional study. Baseline data on demographic, clinical, and environmental factors (including the natural environment, socioeconomic status, and lifestyle) were collected. Disease severity was classified as mild or moderate to severe using the MG Activities of Daily Living (MG-ADL) scale and the Quantitative MG (QMG) scale. Logistic regression was used to estimate odds ratios (ORs) and 95% confidence intervals (CIs) for these associations. RESULTS:A total of 2468 patients were included. The median MG-ADL score was 3.0 (interquartile range [IQR] 1.0-6.0), and the median QMG score was 6.0 (IQR 3.0-9.0). Higher MG-ADL scores were associated with age at onset ≥ 65 years (OR 1.85, 95% CI 1.32-2.58), underweight (2.03, 1.35-3.06), generalized onset (2.80, 2.26-3.48), and thymoma (1.70, 1.26-2.30). In contrast, overweight or obesity (OR 0.65, 95% CI 0.52-0.82), medium altitudes (0.64, 0.46-0.90), high educational attainment (0.68, 0.49-0.94), and high monthly household income (0.71, 0.53-0.96) were inversely associated with MG-ADL scores. Factors associated with higher QMG scores included female sex (OR 1.75, 95% CI 1.28-2.37), age at onset ≥ 65 years (1.91, 1.35-2.71), generalized onset (2.50, 2.00-3.13), thymoma (1.97, 1.43-2.71), medium latitudes (1.53, 1.08-2.16), and high altitudes (1.65, 1.10-2.47). Lower QMG scores were correlated with overweight or obesity (OR 0.78, 95% CI 0.61-0.99), thymectomy (0.57, 0.40-0.81), medium altitudes (0.70, 0.49-0.99), and medium sunlight duration (0.67, 0.49-0.93), as well as higher concentrations of particulate matter with an aerodynamic diameter of ≤ 2.5 μm and higher household income. CONCLUSION:In addition to demographic and clinical factors, environmental factors may also influence MG severity. Further research is warranted to clarify these associations and their underlying mechanisms.

BackgroundIn myasthenia crisis (MC), plasma exchange (PE) and intravenous immunoglobulin (IVIG) are confirmed effective treatment options, but PE may not be available in time, and the response rate to IVIG is not always satisfactory. This study aimed to investigate whether addition of efgartigimod confers benefits in patients with MC.MethodsThis real-world retrospective pilot study examined MC patients admitted to the First Affiliated Hospital of Sun Yat-sen University between August 2023 and May 2024, who were categorized into two groups: the traditional immunotherapy group (n = 14) and the add-on efgartigimod group (n = 9). In the efgartigimod group, patients received 20 mg/kg efgartigimod (n = 9), administered on the first and fifth days.ResultsTotally 23 patients were recruited in the study. Total hospital stays, MV duration, and non-invasive ventilation time were shorter in the efgartigimod group compared with the traditional immunotherapy group; however, these differences were not statistically significant (p > 0.05). We subsequently applied a generalized estimating equation (GEE) to the patients over an 8-week period and observed a reduction in Myasthenia Gravis Activities of Daily Living (MG-ADL) scores in both groups; however, no significant differences were found within the groups (p > 0.05). Survival analysis was also conducted, revealing that the addition of efgrtigimod allowed patients to reach the MSE state more quickly, which is statistically significant (p = 0.0499). Additionally, three patients with MC who only received high-dose efgartigimod treatment all achieved favorable treatment outcomes.ConclusionThis suggests that efgartigimod may serve as an alternative option for MC patients or as a rescue treatment option for refractory individuals.

Background:Neuromyelitis optica spectrum disorder (NMOSD) and multiple sclerosis (MS) differ substantially in pathophysiological mechanisms, clinical manifestations and therapeutic management. Objectives:To investigate the disease burden of NMOSD and MS in South China and analyse the differences in disease burden between the two. Methods:We extracted the demographic characteristics, diagnoses, duration of hospitalization and mortality from the direct reporting system of health information. Results:From 2016 to 2022, the study identified 12,799 hospital admissions for 8204 patients with NMOSD and 6783 hospital admissions for 5125 patients with MS. The female-to-male ratio among patients with MS was 1.82. The peak age of NMOSD diagnosis was 45-55 years. The peak age of MS diagnosis was 25-35 years. The average duration of hospitalization for patients with NMOSD was 12 (7-17) days, whereas that for patients with MS was 10 (6-14) days. The percentage of NMOSD admissions to the intensive care unit was higher than that of MS admissions. The mortality rate for both NMOSD and MS was 0.2%. Conclusions:In South China, the disease burden of NMOSD is heavier than that of MS. The NMOSD exhibited a higher number of cases, longer duration of hospitalization and more severe clinical conditions than MS.

OBJECTIVE:To assess the treatment effecitiveness and safety of Chinese patients with chronic inflammatory demyelinating polyneuropathy (CIDP) treated with intravenous efgartigimod in this real-world study. METHODS:This study analyzed data retrospectively from 13 patients with CIDP treated with efgartigimod at the Department of Neurology, First Affiliated Hospital of Sun Yat-Sen University from December 2023 to September 2024. Clinical assessment scales included the Inflammatory Neuropathy Cause and Treatment (INCAT) score and the Medical Research Council (MRC) sum score were utilized to evalutate effecitiveness before and after efgartigimod treatments. Adverse events werer monitored to assess the safety of efgartigimod treatments. RESULTS:The mean onset age of 13 patients was 38.3 years, and the mean duration from symptom onset to diagnosis was 13.1 months. About 46 % patients in our study were typical CIDP subtype. About 77 % patients experienced relapses before efgartigimod treatments. Clinical improvements were observed in 12 patients (92.3 %) with at least 1-point decrease on INCAT scores at 12 weeks follow-up, indicating efgartigimod treatment effectiveness. Eleven patients (84.6 %) achieved clinical improvements at 24 weeks follow-up. At last follow-up, five patients (38.5 %) had discontinued immunotherapy. Adverse events monitoring revealed that only one patient had pruritus which alleviated spontaneously. CONCLUSION:This case series real-world study support the efficacy and safety of efgartigimod treatment in CIDP patients, benefit on partially reducing the usage of glucocorticoids and immunosuppressants. It was well tolerated across age groups. Larger prospective studies are needed to refine dosing and establish guidelines for CIDP management.

Objective: This study was aimed at evaluating the efficacy of the "zipper method," a novel treatment strategy combining alternating plasma exchange (PLEX) and intravenous immunoglobulin (IVIG) pulse therapy, in adult patients with severe Guillain-Barr & eacute; syndrome (GBS) requiring mechanical ventilation.Methods: A retrospective analysis was conducted on seven adult patients diagnosed with severe GBS and treated with mechanical ventilation from June 2022 to August 2023. Three received the "zipper method" (alternating PLEX and IVIG pulse therapy), and the other four were treated with the classic method (PLEX followed by IVIG pulse therapy). Clinical outcomes, including duration of continuous mechanical ventilation (CMV), length of stay (LOS) in the ICU, total hospital stay, muscle strength recovery as measured by the Muscle Research Council (MRC) score, and Guillain-Barr & eacute; Syndrome Disability Scale (GBS-DS) and days to unaided walking, were compared between the two groups.Results: The "zipper method" group exhibited significant improvements in clinical outcomes compared to the classic method group. Specifically, the duration of CMV was reduced to 17.67 days, the LOS in the ICU was 22.33 days, the mean days to hospital discharge were 40.67 days, and the MRC score at 1 month was 43.67 and at 2 months was 56.67. Furthermore, the GBS-DS score at 2 months posttreatment was 1.00 and the mean days to unaided walking were 80.6 days, indicating a marked reduction in disability.Conclusion: The "zipper method" offers a promising new approach for the treatment of severe GBS in adults, leading to faster recovery of muscle strength and shorter ICU stays and length of hospital stay. This treatment strategy has the potential to improve patient outcomes and reduce the burden of severe GBS on healthcare systems. Further research, including prospective studies and randomized controlled trials, is warranted to validate these findings and explore the broader applicability of the "zipper method" in adult GBS treatment.

BACKGROUND:Thymectomy is beneficial for treating early-onset acetylcholine receptor antibody-positive myasthenia gravis (MG); however, its effects on late-onset MG (LOMG) remain less well understood. Given the increasing incidence of MG among the population 50 years old and above, addressing the question of whether thymectomy is effective for this age group is critically important. This study aimed to assess the present evidence for the efficacy of thymectomy in LOMG and identify potential characteristics that may predict the treatment response. METHODS:Four electronic databases were searched from their inception to September 10, 2024. Six studies with both thymectomy and medical therapies in LOMG patients, along with another 14 studies with only a surgical group, were enrolled in the meta-analysis. The primary outcome was the response (remission and minimal manifestations status) to thymectomy in LOMG. RESULTS:In LOMG, response in the surgical group was greater than in the medical therapies alone group (OR = 1.42 [0.86-2.35], p = 0.169), but not significantly. However, subgroup analysis showed that when the age of MG onset was ≥ 45 years old or the age at thymectomy was ≥ 50 years old, thymectomy appeared better than medical therapies alone (OR = 1.92 [1.06-3.48], p = 0.031). Across all 20 studies, 34% (24%-44%) of LOMG patients improved with thymectomy. A higher response was observed in patients with a preoperative duration of less than 3 years from diagnosis [39% (16%-65%), p < 0.001, q < 0.001]. CONCLUSION:Thymectomy may be a potentially effective treatment for LOMG, particularly in patients who undergo the procedure soon after diagnosis. A randomized controlled study for LOMG patients is needed.

Background: Balamuthia mandrillaris is a free-living amoebic parasite that primarily causes rare opportunistic infections in immunocompromised hosts. Balamuthia amoebic encephalitis (BAE) is a rare yet severe parasitic infection affecting the central nervous system. It has an extremely low incidence in China but can have a mortality rate as high as 98%. The clinical manifestations of amebic infections are similar to those of bacterial and tuberculous meningitis, lacking specificity, which makes accurate diagnosis challenging in the clinical setting. Case Presentation: A 61-year-old immunocompetent woman experienced worsening headache and a moderate fever over the course of five days, initially treated as a common cold. On 25 February 2025, she exhibited behavioral abnormalities, dysphagia, and a high fever of 40.2 °C, which progressed to a coma. On 26 February, her cranial CT scan revealed multifocal hemorrhagic lesions in the right frontotemporoparietal lobes. The MRI revealed similar lesions with slight enhancement and herniation. She underwent an emergency decompressive craniectomy, yet her condition continued to deteriorate following the surgery. On 27 February, serum targeted next-generation sequencing (tNGS) detected B. mandrillaris. Additionally, metagenomic NGS (mNGS) of the cerebrospinal fluid (CSF) sample confirmed the presence on 28 February. Finally, B. mandrillaris was identified through a brain tissue biopsy on 3 March. However, due to the delayed diagnosis and lack of effective drugs, her condition rapidly deteriorated and became irreversible. Her family ultimately chose to withdraw treatment. Conclusions: This study highlights the application of NGS for early diagnosis of patients with severe CNS infection. Both tNGS and mNGS can be considered for the rapid detection of rare or novel pathogens and for facilitating diagnosis.

OBJECTIVE:Limited evidence has led to ongoing debate about the benefits of thymectomy for late-onset myasthenia gravis (LOMG). This study aims to compare the outcomes of patients with LOMG who underwent thymectomy versus those who received medical treatment alone, and evaluate the incidence of surgical adverse events. METHODS:Non-thymomatous acetylcholine-receptor antibody positive patients with LOMG were selected from a multi-center longitudinal clinical database. Rates of and time to response (remission and minimal manifestations status) were compared between 2 groups by propensity score matching (PSM), Kaplan-Meier analysis, and Cox regression models. Additionally, the incidence of adverse events about thymectomy was compared between LOMG and younger patients with myasthenia gravis (MG) aged 40 to 50 years old. RESULTS:A total of 55 and 210 patients with LOMG were enrolled in the thymectomy and medical treatment groups, respectively. The thymectomy group exhibited significantly younger onset age (56.31 ± 6.15 vs. 62.04 ± 7.83, p < 0.001). After PSM adjustment, thymectomy demonstrated a greater cumulative probability (p < 0.001), and the 2.356-fold (95% confidence interval [CI] = 1.537-3.612, p < 0.001) higher chance of better outcomes compared to medical treatment. In subgroup analysis, thymectomy showed significantly higher response rates compared to medical treatment alone at 24 ± 2 months (48.9%, 95% CI = 33.7-64.1% vs 23.8%, 95% CI = 14.2-33.3%, p = 0.004), and 36 ± 2 months (59.5%, 95% CI = 42.9-76.1% vs 28.9%, 95% CI = 18.5-39.4%, p = 0.002). The incidences of adverse events were comparable between patients with LOMG and younger patients with MG (32.0% vs 22.4%, p = 0.286). INTERPRETATION:Thymectomy may be an effective therapeutic option for LOMG. Our findings highlight the need for further development of a randomized trial targeting patients with LOMG. ANN NEUROL 2026;99:629-638.

Myasthenic crisis (MC) refers to rapid deterioration of myasthenia gravis (MG), affecting lung and bulbar muscles and causing breathing difficulties. Currently, efgartigimod has shown good therapeutic effects in patients with generalized myasthenia gravis (GMG). This retrospective real-world study explored the effectiveness of efgartigimod in patients with MC. Reviewing the clinical data of five patients (including four patients with refractory MC) with MC who received efgartigimod at the First Affiliated Hospital of Sun Yat-sen University, all of these patients were admitted from September 2023 to December 2023. Each patient received 20 mg/kg of efgartigimod on the first and fifth day. After discharge, all patients showed a clinically meaningful decrease in Myasthenia Gravis Activities of Daily Living (MG-ADL) scale (a decrease of ≥ 2 points) and an improvement in their lung function. Additionally, all patients had a decrease in IgG levels (58.59 ± 18.48

OBJECTIVES:Efgartigimod has been approved as an effective and safe treatment for myasthenia gravis (MG). However, real-world experience on multi-cycle efgartigimod treatment and its comparison with Standard of Care (SoC) remain limited. This study aimed to evaluate minimal symptom expression (MSE) as the treatment goal and compared the proportion and time to achieving it between two groups. METHODS:Patients receiving multi-cycle efgartigimod and contemporaneous counterparts treated with SoC were included. The rate of MSE achievement were compared using Kaplan-Meier analysis and Cox regression. Subgroup analysis focused on efgartigimod group, observing involved muscles and oral medication for further insights. RESULTS:A total of 76 and 124 MG patients were enrolled in the efgartigimod and SoC groups, respectively. Efgartigimod group demonstrated a higher rate (73.3 % vs. 22.6 %, p < 0.001) and shorter time [0.7 (0.5, 3.1) vs. 3.3 (3, 6.1), months, p < 0.001] to achieving MSE compared to SoC group. Kaplan-Meier analysis revealed efgartigimod group had a higher MSE probability with a median time of 2.27 (95 %CI, 0.70, 4.39) months. MG Patients had a 9.69 fold (95 %CI, 5.54, 16.92) greater chance of achieving MSE compared to SoC group, remaining significant at 9.44 fold (95 %CI, 5.36, 16.60) after adjusting for ADL scores. After treatment, respiratory and bulbar symptoms improved significantly, with average scores from 0.57 ± 0.87 to zero, and 2.62 ± 2.56 to 0.37 ± 0.96. Additionally, the daily dosage of corticosteroid dropped from 20(10, 25) mg to 10(10,20) mg, with only 7 (9.2 %) patients requiring over 20 mg/day. CONCLUSION:Multi-cycle efgartigimod treatment achieves early MSE more effectively than SoC, serving as a fast-acting therapy for MG.

BACKGROUND:Glial fibrillary acidic protein-immunoglobulin G (GFAP-IgG) positivity is associated with autoimmune GFAP astrocytopathy (GFAP-A), but also with other autoimmune encephalitides and viral infections. We attempted to elucidate the characteristics of GFAP-A in relation to other GFAP-IgG-positive encephalitides and constructed a differential diagnosis model. METHODS:141 GFAP-IgG-positive cases were identified, including 52 astrocytopathy (GFAP-A group), 48 autoimmune encephalitis (AE-G), and 41 viral encephalitis (VE-G). Multivariate logistic regression was employed to create a diagnostic model, with validation using an external cohort. RESULT:Compared to the AE-G group, the GFAP-A patients showed more onset age ≥ 50 years, headache, fever, consciousness disturbance, MRI radial vascular enhancement, cerebrospinal fluid (CSF) antibody titer grade ≥ 4, and CSF proteins ≥ 700 mg/L, but less female sex, limb numbness, visual disturbances, and CSF chloride ≤ 120 mmol/L. Among these, CSF antibody titer grade ≥ 4, CSF protein ≥ 700 mg/L, and absence of visual disturbances were independent risk factors for GFAP-A diagnosis. Compared to the VE-G group, the GFAP-A patients showed more course ≥ 14 days, onset age ≥ 50 years, limb weakness, serum potassium ≤ 3.9 mmol/L, CSF antibody titer grade ≥ 4, CSF leukocytes ≤ 46*10, MRI radial vascular enhancement, MRI involvement of brainstem, and MRI involvement of spinal cord, but less headache, fever, nausea, and vomiting. Among these, serum potassium ≤ 3.9 mmol/L, MRI spinal cord involvement, and absence of nausea and vomiting were independent risk factors for GFAP-A diagnosis. CONCLUSIONS:Based on critical clinical indicators identified, we constructed a differential diagnosis model for GFAP-A.

The literature lacks consistent information on the correlation between baseline body mass index (BMI), clinical presentation, and prognosis in patients with myasthenia gravis (MG). This observational multicenter prospective cohort study included patients with MG from February 2017 to June 2023, categorizing them by baseline BMI. The primary outcome was the time to generalization of ocular MG. Secondary outcomes included the time to Activities of Daily Living (ADL) response and Minimal Symptom Expression (MSE). Kaplan-Meier curves and multivariable Cox proportional hazards regression models were used to assess the impact of BMI on these outcomes. Out of 940 MG patients (510 women) included, 524 had a low BMI and 416 had a high BMI, with a median age of 50.00 years. Patients in the high BMI group were significantly older (p < 0.001), had a lower percentage of females (p < 0.001), and had a shorter disease duration (p = 0.014) compared to those with a low BMI. They also had higher rates of ocular onset (p < 0.001), ocular MG classification (p = 0.001), and acetylcholine receptor antibody seropositivity (p = 0.007), but a lower incidence of thymectomy (p = 0.027). During a median follow-up of 33.00 months, the adjusted Cox models revealed that a higher baseline BMI was associated with an increased risk of ocular MG generalization (HR 1.06; 95

Ofatumumab is a fully human anti-CD20 monoclonal antibody that selectively and highly depletes B cells. However, limited data on ofatumumab treatment are available in patients with neuromyelitis optica spectrum disorders (NMOSD). In this study, we aimed to evaluate the efficacy and safety of subcutaneous ofatumumab in patients with NMOSD. We conducted a retrospective multicenter cohort study of patients with NMOSD who received ofatumumab treatment at 15 tertiary hospitals in China. The primary outcome was the annualized relapse rate (ARR). The secondary outcomes included disability measures (Expanded Disability Status Scale score, EDSS; the Aminoff–Logue Disability Scale, ALS), changes in aquaporin-4 IgG (AQP4-IgG) titers, and safety profiles during ofatumumab treatment. A total of 112 patients (88

Background: By extracting and sequencing miRNAs from serum exosomes of patients with early-onset ocular myasthenia gravis (OMG), generalized myasthenia gravis (GMG) and healthy controls, we screened differentially expressed miRNAs and explored the possibility as potential biomarkers for early-onset OMG. Methods: Peripheral blood samples were collected from patients with early-onset OMG, early-onset GMG, and age-matched healthy subjects, with 6 samples in each group. All these patients were diagnosed as MG for the first time and did not undergo any treatment. Exosomes miRNAs were extracted from the serum and performed deep sequencing; the differentially expressed miRNAs were compared and analyzed between OMG, GMG, and healthy control groups using edgeR. The differential expression standard was set to | log2FC |>1, p < 0.05. Target prediction of mRNAs were performed using miRTarBase, TargetScan, and miRDB databases, and a protein- -protein interaction (PPI) network was constructed subsequently. The miRNAs with a significant difference were validated using RT-qPCR (10 early-onset OMG patients, 10 early-onset GMG patients and 10 age-sex-matched healthy subjects), and the value of the area under the ROC curve (AUC) was used to assess the diagnostic ac-curacy and evaluate clinical prognostic value. Results: In total, one upregulated (miR-130a-3p) miRNA was obtained through the upregulated intersection between control vs OMG and OMG vs GMG; four downregulated (miR-4712-3p; miR-6752-5p; miR-320d; miR-3614-3p) miRNAs were obtained through the downregulated intersection between control vs OMG and OMG vs GMG. A total of 408 target genes were predicted for the five differentially expressed miRNAs. The mTOR signaling pathway and Rap1 signaling pathway were significantly enriched based on the enrichment results. RT-qPCR findings revealed that for the OMG, the expression of miR-320d, miR-4712-3p and miR-3614-3p was markedly up-/down-regulated as compared to GMG and healthy control group. The AUC for the three miRNAs between OMG and healthy control groups were 0.78, 0.79 and 0.79 respectively; the AUC between OMG and GMG was 0.84. Conclusions: The present study identified three novel miRNAs as candidate biomarkers for early-onset OMG patients and it was expected to provide a possibility and a new orientation for serum exosomal miRNAs as OMG diagnostic biomarkers.

Background:Since there is no clear priority or selection principle in the guidelines for myasthenia crisis, therapeutic plasma exchange (TPE) and intravenous immunoglobulin are often administered randomly. However, it should be more prudent in taking TPE due to its higher cost and risk. Studying its early response factors is crucial for managing myasthenia crisis and can improve medical and economic benefits.Methods:A prospective observational study was conducted, and patients classified as having "impending myasthenia crisis" or experiencing a myasthenia crisis and treated by TPE were included. The primary endpoint was the response after TPE. Univariate logistic regression analysis and repeated measurement were performed to analyze factors related to TPE efficacy.Results:A total of 30 patients who treated with TPE as their fast-acting treatments were enrolled. After TPE, those whose QMGs and/or MGCs decreased by ≥5 points or ≥30% of the baseline were judged as "response group", accounting for 66.67% (20/30). Respiratory symptoms had a response rate of 72.00% (18/25), showing the most remarkable improvement. Meanwhile, extraocular symptoms were the least sensitive, with only 8.00% (2/25) showing efficacy. Thymoma (100.00% vs 50.00%, P=0.002) and a high concentration of AChR-Ab (37.37 nmol/L vs 25.4 nmol/L, P=0.039) were common in the early response group. Repeated measures showed significant changes in AChR-Ab and CD19+ B cells before and after TPE (all with P < 0.05). After treatment, the CD19+ B cells tended to decrease in the response group.Discussion:These results indicated that, for AChR-Ab positive generalized MG, TPE can quickly improve respiratory symptoms. Thymoma and a high concentration of AChR-Ab before TPE predict an early better response. Additionally, TPE may work by decreasing AChR-Ab levels and inducing immune regulation. Future prospective and randomized controlled studies are needed.

Background: Combined first-line therapies have been frequently adopted for patients with anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis. Plasma exchange (PE) or immunoadsorption (IA) was used as an add-on option following initial immunotherapies, including high-dose steroids and intravenous immunoglobulin (IVIG). However, whether a shorter delay of PE or IA can improve the early recovery prognosis of patients with anti-NMDAR encephalitis remains largely unknown. Objective: To compare short-term clinical improvement between patients with early and late initiation of PE or IA in anti-NMDAR encephalitis. Design: A retrospective study was conducted for patients admitted with anti-NMDAR encephalitis between January 2015 and December 2023 ( n = 29), including 21 patients who received PE or IA as synergistic therapies. Methods: The clinical prognosis was compared between the early PE/IA group and the late PE/IA group in the research. Primary outcome included changes in the Clinical Assessment Scale for Autoimmune Encephalitis (∆CASE) at 90 and 120 days after encephalitis onset. Secondary outcomes included changes in the modified Rankin scale (∆mRS) after 90 and 120 days from encephalitis onset, and the length of intensive care unit (ICU) stay for patients with severe anti-NMDAR encephalitis. Results: The ∆CASE scores after 90 and 120 days from encephalitis onset revealed a significant difference between patients with early and late initiation of PE or IA ( p ⩽ 0.05). A significant difference in the ∆mRS was also found between patients with early and late initiation of PE or IA in severe encephalitis ( p ⩽ 0.05). No significant difference was found in the length of ICU admission ( p = 0.101). Conclusion: Our findings emphasize the importance of considering PE or IA as early as feasible for patients with anti-NMDAR encephalitis, even when steroids and IVIG are in use.

Summary Background The environmental effects on the prognosis of ocular myasthenia gravis (OMG) remain largely unexplored. Aim To investigate the association between specific environmental factors and the generalization of OMG. Design The cohort study was conducted in China based on a nationwide multicenter database. Methods Adult patients with OMG at onset, who were followed up for at least 2 years until May 2022, were included. We collected data on demographic and clinical factors, as well as environmental factors, including latitude, socioeconomic status (per capita disposable income [PDI] at provincial level and education) and smoking. The study outcome was the time to the development of generalized myasthenia gravis (GMG). Cox models were employed to examine the association between environmental exposures and generalization. Restricted cubic spline was used to model the association of latitude with generalization risk. Results A total of 1396 participants were included. During a median follow-up of 5.15 (interquartile range [IQR] 3.37–9.03) years, 735 patients developed GMG within a median of 5.69 (IQR 1.10–15.66) years. Latitude of 20–50°N showed a U-shaped relation with generalization risk, with the lowest risk at around 30°N; both higher and lower latitudes were associated with the increased risk (P for non-linearity <0.001). Living in areas with lower PDI had 1.28–2.11 times higher risk of generalization. No significant association was observed with education or smoking. Conclusions Latitude and provincial-level PDI were associated with the generalization of OMG in China. Further studies are warranted to validate our findings and investigate their potential applications in clinical practice and health policy.

ObjectivesTo determine risk factors for the occurrence of adverse outcomes in patients with new-onset refractory status epilepsy (NORSE) and to construct a concomitant nomogram.MethodsSeventy-six adult patients with NORSE who were admitted to the Department of Neurology, First Affiliated Hospital of Sun Yat-sen University between January 2016 and December 2022 were enrolled for the study. Participants were divided into two—those with good and poor functional outcomes—and their pertinent data was obtained from the hospital medical recording system. Univariate analysis was used to identify potential causes of poor outcomes in both groups and a multivariate logistic regression model was used to identify risk factors for the occurrence of poor outcomes. Using the R programming language RMS package, a nomogram was created to predict the occurrence of poor outcomes.ResultsThe NORSE risk of adverse outcome nomogram model included four predictors, namely duration of mechanical ventilation (OR = 4.370, 95% CI 1.221–15.640, p = 0.023), antiviral therapy (OR = 0.045, 95% CI 0.005–0.399, p = 0.005), number of anesthetics (OR = 13.428, 95% CI 2.16–83.48, p = 0.005) and neutrophil count/lymphocyte count ratio (NLR) (OR = 5.248, 95% CI 1.509–18.252, p = 0.009). The nomogram had good consistency and discrimination in predicting risk and can thus assist clinical care providers to assess outcomes for NORSE patients. Through ordinary bootstrap analyses, the results of the original set prediction were confirmed as consistent with those of the test set.ConclusionThe nomogram model of risk of adverse outcomes in NORSE adult patients developed in this study can facilitate clinicians to predict the risk of adverse outcomes in NORSE patients and make timely and reasonable interventions for patients at high risk of adverse outcomes.