Neuromyelitis optica spectrum disorder (NMOSD) is a immune-mediated demyelinating disease of the central nervous system, characterized by high recurrence and disability rates. Preventing relapses is crucial in the treatment of this condition. Monoclonal antibodies have emerged as a novel and rapidly evolving clinical therapeutic strategy targeting NMOSD in recent years. An increasing number of studies and clinical trials have also confirmed the effectiveness and safety of monoclonal antibodies. Rituximab, a monoclonal antibody targeting the B-cell surface antigen CD20, has been widely used in the treatment of NMOSD. Currently, in China, the only approved monoclonal antibody for treating NMOSD is Inebilizumab, which targets the B-cell surface antigen CD19. Additionally, various monoclonal antibodies, such as interleukin-6 receptor inhibitors and complement C5 inhibitors, have been used in the treatment of NMOSD. With the deepening of the research on the pathogenesis of NMOSD, the molecular mechanism of disease-related immune network is further clarified, and multi-center clinical trials are widely carried out. More accurate monoclonal antibody treatment strategies for NMOSD will be applied to clinical practice, benefiting more patients.

Objective:To observe the clinical characteristics and optical coherence tomography (OCT) features of pseudopapilledema (PPE) combined with peripapillary hyper-reflective ovoid mass-like structures (PHOMS) in children.Methods:A retrospective observational study. From October 2019 to May 2021, total 22 eyes from 12 children diagnosed as PPE combined with PHOMS in the Neuro-ophthalmology Department of The First Hospital of Xi’an (Affiliated of The First Hospital of Northwest University) were recruited. Among the children, 6 were male and 6 were female. The average age was (10.6±2.7) years. The average course from disease onset to diagnosis of PPE combined with PHOMS was (8.0±7.5) months. All patients underwent best corrected visual acuity (BCVA), relative afferent papillary defect (RAPD), Ishihara's test, fundus photography, OCT, fundus autofluorescence (FAF), ocular B-mode ultrasound, visual field and patternvisual evoked potential (P-VEP). The clinical and OCT characteristics of the patients were observed.Results:The anterior segments of the patients were normal. The intraocular pressures and Ishihara's test were all normal. All RAPD were negative. Total 22 eyes, BCVA was 1.0 in 21 eyes and one eye was 0.12. The fundus photography revealed blurred optic discs margin, showing mild to moderate edema-like elevation with more prominent in the nasal parts, presenting as a "C" shape halo. No obvious abnormal fluorescence was observed in FAF. The OCT scan of involvement eyes showed an elevated appearance in vary degrees, and the sharply marginated ovoid hyper-reflective mass-like structures which laterally herniated into the peripapillary region under retinal nerve fiber layer and above the Bruch membrane were detected with consecutive nasal enlargement scanning, corresponding to the nasal parts in the fundus photography. The higher degree of elevation, the larger the volume. Macular retina pigment epithelium layer and ganglion cell thickness were normal. Ocular B-mode ultrasound showed that the head of the optic nerve in the posterior wall of the eyeball (in front of the optic disc) was elevated in all affected eyes, and there was no strong signal echo in it. Visual field examination showed physical blind spot enlargement in 3 eyes and visual field defect in 2 eyes. P-VEP examination showed that the peak was slightly delayed in 3 eyes and the amplitude was slightly reduced in 3 eyes.Conclusions:Enlarged nasal optic disc OCT scan can improve the detection rate of PHOMS. PHOMS were detected bilaterally in the cases with binocular PPE while only in the effected eye in the cases of monocular PPE; the higher degree of PPE, the lager volume of PHOMS. PHOMS were could contribute to the diagnosis of PPE in children.

Objective:To preliminary investigate the impact of the diagnosis-related groups (DRG) payment method reform on the diagnosis and treatment of inpatient medical insurance patients with neuromyelitis optica spectrum disorders (NMOSD), and to propose potential improvement strategies.Methods:A single-center, retrospective study. From October 1, 2020, to September 30, 2022, 44 hospitalized medical insurance patients with acute-phase NMOSD diagnosed and treated at the First Affiliated Hospital of Northwest University (Xi'an First Hospital) were included in the study. Among them, there were 11 males and 33 females, with an average age of (40.8±20.2) years. According to the implementation time of DRG payment, patients were divided into two groups: group A, which consists of cases one year before the implementation of DRG payment from October 1, 2020 to September 30, 2021, and group B, which consists of cases one year after the implementation of DRG payment from October 1, 2021 to September 30, 2022, with 20 and 24 cases, respectively. Detailed information such as hospitalization duration, treatment methods, and hospitalization costs of the two groups of patients was collected. Comparative analysis was conducted on hospitalization costs and treatment methods between the two groups. For intergroup comparison, t-test was used for normally distributed data, and Mann-Whitney U test was used for skewed distributed data. Results:Among the 44 patients, 5 cases (5/24, 20.8%) received plasma exchange (PE) treatment, all of whom were in group B. The numbers of patients who received and did not receive intravenous immunoglobulin (IVIG) treatment were 9 and 11 in group A, respectively, and 7 and 12 in group B (except for 5 cases who received PE treatment), respectively. Compared with group A, there was no significant decrease in hospitalization duration ( t=0.004) and total hospitalization costs ( Z=0.036), as well as costs for western medicine ( Z=0.036), examinations ( Z=0.011), laboratory tests ( Z=0.040), treatments ( Z=0.017), and nursing ( Z=3.131) in group B, and the differences were not statistically significant ( P>0.05). For patients receiving PE treatment, except for the cost of western medicine ( Z=0.062, P=0.804), the other costs ( Z=8.288, 5.013, 11.400, 10.925, 9.126) were significantly higher than those of patients not receiving PE treatment, and the hospitalization duration ( t=20.474) was significantly prolonged, with statistically significant differences ( P<0.05). The total hospitalization costs of patients receiving IVIG treatment were significantly higher than those not receiving IVIG treatment in both group A and group B, with statistically significant differences ( Z=7.690, 10.314; P<0.05). There was no statistically significant difference in the comparison of total hospitalization costs between patients receiving IVIG treatment in group A and group B ( Z=0.137, P>0.05). Conclusions:There is no significant decrease in various hospitalization costs of NMOSD medical insurance patients in Xi'an after the implementation of DRG payment, especially for patients receiving PE treatment. It is suggested to optimize the rate stratification of NMOSD patients when implementing DRG payment methods.

Objective:To investigate the risk factors for poor outcome and recurrence at 1 year after first-ever ischemic stroke in non-diabetic patients.Methods:Using Xi'an Stroke Registry Research Database, the clinical data of patients with non-diabetic first-ever ischemic stroke diagnosed in 4 tertiary A hospitals in Xi'an from January to December 2015 were collected. The National Institute of Health Stroke Scale (NIHSS) was used to evaluate the severity of stroke. Prognosis (functional outcome and recurrence) was followed up at 1 year after diagnosis. Functional outcome was assessed using the modified Rankin scale. 0-2 was defined as good outcome and >2 as poor outcome. Recurrence was defined as new focal neurological dysfunction caused by cerebral infarction or cerebral hemorrhage events during follow-up and confirmed by cranial CT or MRI. Multivariable logistic regression analysis was used to identify the independent influencing factors of clinical outcomes at 1 year. Multivariable Cox proportional hazard model was used to identify the independent influencing factors of recurrence within 1 year. Results:A total of 1 214 non-diabetic patients with first-ever ischemic stroke were included. One year follow-up showed that 210 patients (17.3%) had a poor outcome, 88 (7.2%) of them died, and 47 (3.9%) had recurrence. Multivariate logistic regression analysis showed that age (odds ratio [ OR] 1.065, 95% confidence interval [ CI] 1.042-1.090; P<0.001), atrial fibrillation ( OR 3.170, 95% CI 1.588-6.327; P=0.001), white blood cell count ( OR 1.106, 95% CI 1.006-1.216; P=0 037), baseline NIHSS score ( OR 1.210, 95% CI 1.147-1.277; P<0.001), and stroke associated-pneumonia (SAP; OR 3.677, 95% CI 1.451-9.316; P=0.006) were independently associated with poor outcomes. Multivariate Cox proportional hazards regression analysis showed that baseline NIHSS score (hazard ratio [ HR] 1.055, 95% CI 1.003-1.109; P=0.036) and SAP ( HR 7.067, 95% CI 3.154-15.836; P<0.001) were independently associated with recurrence. Kaplan-Meier survival curve analysis showed that the 1-year recurrence rate of patients with severe stroke was significantly higher than that of patients with mild to moderate stroke (log-rank test, P<0.001), and the 1-year recurrence rate of patients with SAP was significantly higher than that of patients without SAP (log-rank test, P<0.001). Conclusion:Age, atrial fibrillation, white blood cell count, baseline NIHSS score and SAP are the independent predictors of poor outcomes at 1 year after first-ever ischemic stroke in non-diabetic patients. Baseline NIHSS score and SAP are the independent predictors of recurrence within 1 year after first-ever ischemic stroke in non-diabetic patients.

Objective:To investigate the clinical characteristics of vascular neuro-ophthalmology in patients with central retinal artery occlusion (CRAO).Methods:A single-center, prospective clinical study. From January 2018 to December 2020, 49 eyes of 49 CRAO patients of The Neuro-ophthalmology Department of Xi'an First Hospital were included in the study. Data on patient demographic characteristics, vascular risk factors, disease characteristics, digital subtraction angiography (DSA) imaging characteristics of internal carotid arteries, treatment, treatment-related adverse events, and 1-month follow-up vascular events were collected. All patiens were examined by visual acuity, head CT and or magnetic resonance imaging. At the same time, 35 cases of internal carotid artery vascular DSA were examined; 14 cases of head and neck CT angiography were examined. The anatomical variation of the extracranial segment of the internal carotid artery was divided into tortuous, tortuous, and coiled; the aortic arch was divided into type Ⅰ , type Ⅱ , type Ⅲ, and bovine type. Intravenous thrombolysis, arterial thrombolysis, conservative treatment were performed. The follow-up time was1 month after treatment. Functional vision was defined as vision ≥20/100. Vascular events were strokes, cardiovascular events, deaths and neovascular glaucoma during follow-up.Results:Among 49 eyes of 49 cases, 40 eyes were male (81.6%, 40/49), and 9 eyes were female (18.4%, 9/49); the average age was 60.7±12.9 years. There were 33, 17, and 16 cases with hypertension, type 2 diabetes, and cerebrovascular disease, respectively; 27 and 34 cases had a history of smoking and tooth loss, respectively. Taking antihypertensive, hypoglycemic, antiplatelet aggregation/anticoagulation, and hypolipidemic drugs were 15, 5, 8, and 5 patients, respectively. There were 11 cases of transient amaurosis before the onset, and 17 cases of CRAO after waking up. There were 33 cases (67.3%, 33/49) with infarction of the affected side of the brain tissue. DSA was performed in 35 cases, and the stenosis rate of the internal carotid artery on the affected side was 70%-99% and 100% were 3 (8.6%, 3/35) and 4 (11.4%, 4/35) cases, respectively. The ophthalmic artery on the affected side originated from the external carotid artery in 5 cases (14.3%, 5/35). There were 17 (54.8%, 17/31) and 2 (6.5%, 2/31) cases of tortuousity and kinking in the extracranial segment of the internal carotid artery. There were 15 (42.9%, 15/35), 6(17.1%, 6/35), and 2 (5.7%, 2/35) cases of aortic arch type Ⅱ, type Ⅲ, and bovine type, respectively. Intravenous thrombolysis and arterial thrombolysis were performed in 13 and 29 cases, respectively. Complications occurred in 2 cases during treatment; 3 cases of symptoms fluctuated after treatment, and 10 cases of asymptomatic new infarcts occurred in imaging studies. Forty-eight cases were treated with antiplatelet aggregation/anticoagulation and hypolipidemic treatment. At discharge and 1 month after treatment, the recovery of functional vision was 7 and 17 cases, respectively. One month after treatment, 1 case died because myocardial infarction; 2 cases of neovascular glaucoma occurred.Conclusion:The proportion of CRAO patients with vascular risk factors and internal carotid artery abnormalities on the affected side is relatively high; the prognosis is relatively good after intravenous thrombolysis and/or arterial thrombolysis and secondary stroke prevention.

Autism spectrum disorder (ASD) is a neurodevelopmental disorder characterized by deficits in social interaction and communication, along with repetitive and restrictive patterns of behaviors or interests. Normal brain development is crucial to behavior and cognition in adulthood. Abnormal brain development, such as synaptic and myelin dysfunction, is involved in the pathogenesis of ASD. Microtubules and microtubule-associated proteins (MAPs) are important in regulating the processes of brain development, including neuron production and synaptic formation, as well as myelination. Increasing evidence suggests that the level of MAPs are changed in autistic patients and mouse models of ASD. Here, we discuss the roles of MAPs.
Animals ; Autism Spectrum Disorder ; metabolism ; physiopathology ; Autistic Disorder ; metabolism ; physiopathology ; Disease Models, Animal ; Humans ; Microtubule-Associated Proteins ; metabolism ; Neurons ; metabolism ; Social Behavior

Background/Aims: Four-week treatment of linvencorvir (RO7049389) was generally safe and well tolerated, and showed anti-viral activity in chronic hepatitis B (CHB) patients. This study evaluated the efficacy, safety, and pharmacokinetics of 48-week treatment with linvencorvir plus standard of care (SoC) in CHB patients. Methods: This was a multicentre, non-randomized, non-controlled, open-label phase 2 study enrolling three cohorts: nucleos(t)ide analogue (NUC)-suppressed patients received linvencorvir plus NUC (Cohort A, n=32); treatment-naïve patients received linvencorvir plus NUC without (Cohort B, n=10) or with (Cohort C, n=30) pegylated interferon-α (Peg-IFN-α). Treatment duration was 48 weeks, followed by NUC alone for 24 weeks. Results: 68 patients completed the study. No patient achieved functional cure (sustained HBsAg loss and unquantifiable HBV DNA). By Week 48, 89% of treatment-naïve patients (10/10 Cohort B; 24/28 Cohort C) reached unquantifiable HBV DNA. Unquantifiable HBV RNA was achieved in 92% of patients with quantifiable baseline HBV RNA (14/15 Cohort A, 8/8 Cohort B, 22/25 Cohort C) at Week 48 along with partially sustained HBV RNA responses in treatment-naïve patients during follow-up period. Pronounced reductions in HBeAg and HBcrAg were observed in treatment-naïve patients, while HBsAg decline was only observed in Cohort C. Most adverse events were grade 1–2, and no linvencorvir-related serious adverse events were reported. Conclusions 48-week linvencorvir plus SoC was generally safe and well tolerated, and resulted in potent HBV DNA and RNA suppression. However, 48-week linvencorvir plus NUC with or without Peg-IFN did not result in the achievement of functional cure in any patient.