Explanipedia

Structural atrophy and functional disturbance in the thalamic nuclei of patients with amyotrophic lateral sclerosis Open

Yun‐Bin Cao, Sijie Zhuang, Zhaoyang Huang, Jia‐Yan Shi, Hongyu Lin , et al. · 2025

Selective structural and functional damage to thalamic subregions is a feature of ALS and could potentially contribute to future diagnostic approaches and assessments of disease severity.

AB018. Targeted biologics for treating thymoma associated myasthenia gravis: a multicenter retrospective study Open

Lei Jin, Dingxian He, Hongxi Chen, Hongyu Zhou, Quantao Zeng , et al. · 2025

Immune checkpoint changes correlate with the progression and prognosis of amyotrophic lateral sclerosis Open

Sheng Chen, Chun‐Zuan Xu, Changyun Liu, Jiaqi Li, Shanlin Ke , et al. · 2025

Our research demonstrated a considerable increase in membrane-bound and soluble PD-1 in ALS patients, correlating with disease progression and worse prognosis. Furthermore, we explored 13 other immune checkpoint molecules. Collectively, th…

Effectiveness of tetramethylpyrazine nitrone in amyotrophic lateral sclerosis: A randomized clinical trial Open

Xiaolu Liu, Huifang Shang, Qianqian Wei, Xiaoli Yao, Ling Lian , et al. · 2025

Efgartigimod combined with steroids as a fast-acting therapy for anti-SRP immune-mediated necrotizing myopathy Open

Qiqi Peng, Xiaoping Yao, Sheng Chen, Xiaofeng Li, Feifei Lin , et al. · 2025

Background Immune-mediated necrotizing myopathy (IMNM) is a rare autoimmune disease. Efgartigimod is a human IgG antibody Fc fragment, can enhance the degradation of IgG and thus may be a promising therapeutic agent for IMNM. Methods All t…

Association of glymphatic system disturbance with neural dysfunction in amyotrophic lateral sclerosis Open

Nao‐Xin Huang, J. Y. Zeng, Hui‐Wei Huang, S.H. Fang, Sheng Chen , et al. · 2025

The glymphatic system function was impaired in ALS. This may contribute to spontaneous neural activity disturbance and could represent a mechanism for the development of sensorimotor deficits frequently observed in patients with ALS.

Tetramethylpyrazine Nitrone in Amyotrophic Lateral Sclerosis Open

Xiaolu Liu, Huifang Shang, Qianqian Wei, Xiaoli Yao, Ling Lian , et al. · 2025

Importance Tetramethylpyrazine nitrone has exhibited promising results in improving motor dysfunction in neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS). Objective To evaluate the safety and efficacy of orally adm…

Associations of cachexia and frailty with amyotrophic lateral sclerosis Open

Tracy L. Peters, Weihong Qiu, Haomin Yang, Wuqing Huang, Yizhen Hu , et al. · 2025

Patterns and predictors of therapeutic response to efgartigimod in acetylcholine receptor-antibody generalized myasthenia gravis subtypes Open

Lei Jin, Zhang‐Yu Zou, Qinzhou Wang, Wenshuang Zeng, Qilong Jiang , et al. · 2025

Background: Efgartigimod is an approved biologic for generalized myasthenia gravis (gMG), which is an autoimmune disease and can potentially be life-threatening. However, the therapeutic response to efgartigimod among the acetylcholine rec…

Clinical and functional characterization of a novel STUB1 mutation in a Chinese spinocerebellar ataxia 48 pedigree Open

Jiaqi Li, Wenyi Xie, Jian-Min Chen, Chun-Zuan Xu, Yali Huang , et al. · 2024

Background Spinocerebellar ataxias (SCAs) encompass a wide spectrum of inherited neurodegenerative diseases, primarily characterized by pathological changes in the cerebellum, spinal cord, and brainstem degeneration. Autosomal dominant spi…

Clinical and Functional Characterization of a Novel STUB1 Mutation in a Chinese Spinocerebellar Ataxia 48 Pedigree Open

Jiaqi Li, Wenyi Xie, Jian-Min Chen, Chun-Zuan Xu, Yali Huang , et al. · 2024

Background Spinocerebellar ataxias (SCAs) encompass a wide spectrum of inherited neurodegenerative diseases, primarily characterized by pathological changes in the cerebellum, spinal cord, and brainstem degeneration. Autosomal dominant spi…

Cortical microstructural abnormalities in amyotrophic lateral sclerosis: a gray matter-based spatial statistics study Open

Xin-Yun Xiao, Jing-Yi Zeng, Yun‐Bin Cao, Nelson L.S. Tang, Zhang‐Yu Zou , et al. · 2024

The decreases in NDI and ODI involved both motor-related and extra-motor regions and indicated the presence of gray-matter microstructural impairment in ALS. NODDI parameters are potential imaging biomarkers for evaluating disease severity…

Efgartigimod for generalized myasthenia gravis: A multicenter real‐world cohort study in China Open

Sushan Luo, Qilong Jiang, Wenshuang Zeng, Qinzhou Wang, Zhang‐Yu Zou , et al. · 2024

Objective Efgartigimod, a neonatal Fc receptor antagonist, facilitates antibody degradation including pathogenic IgGs. The ADAPT study demonstrated the tolerability and efficacy of efgartigimod in the treatment of generalized myasthenia gr…

Serum cytokines profile changes in amyotrophic lateral sclerosis Open

Chun-Zuan Xu, Xiao Huan, Sushan Luo, Huahua Zhong, Chongbo Zhao , et al. · 2024

In this study, we identified systemic cytokine profile changes in the serum of ALS patients, especially the elevated IL-18, as well as the decreased IL-21 in elder patients. These changes in serum cytokine profiles may shed new light on an…

Batoclimab vs Placebo for Generalized Myasthenia Gravis Open

Chong Yan, Yao-Xian Yue, Yuzhou Guan, Bitao Bu, Qing Ke , et al. · 2024

Importance Myasthenia gravis (MG) is caused by autoantibodies that disrupt the neuromuscular junction. The neonatal fragment crystallizable receptor (FcRn) antagonists, efgartigimod and rozanolixizumab, reduce immunoglobulin G (IgG) level …

Neurite orientation dispersion and density imaging quantifies microstructural impairment in the thalamus and its connectivity in amyotrophic lateral sclerosis Open

Yun‐Bin Cao, Ye Wu, Qiu‐Yi Dong, Nao‐Xin Huang, Zhang‐Yu Zou , et al. · 2024

Aims To evaluate microstructural impairment in the thalamus and thalamocortical connectivity using neurite orientation dispersion and density imaging (NODDI) in amyotrophic lateral sclerosis (ALS). Methods This study included 47 healthy co…

Eculizumab in thymoma-associated myasthenia gravis: a real-world cohort study Open

Lei Jin, Dingxian He, Quantao Zeng, Song Tan, Jian‐Quan Shi , et al. · 2024

Background: Thymoma-associated myasthenia gravis (TAMG) is a subtype of myasthenia gravis (MG) that is associated with more severe symptoms and a relatively poor prognosis. Eculizumab, an inhibitor to target human C5 component of the compl…

Engineering a Wirelessly Self-Powered Neural Scaffold Based on Primary Battery Principle to Accelerate Nerve Regeneration Open

Cijun Shuai, Huixing Li, Xiong Shuai, Yanyan Chen, Jian Xiong , et al. · 2024

Eomesodermin expression in CD4+T‐cells associated with disease progression in amyotrophic lateral sclerosis Open

Sheng Chen, Xiao Huan, Chun‐Zuan Xu, Sushan Luo, Chongbo Zhao , et al. · 2023

Aim To clarify the role of Eomesodermin (EOMES) to serve as a disease‐relevant biomarker and the intracellular molecules underlying the immunophenotype shifting of CD4 + T subsets in amyotrophic lateral sclerosis (ALS). Methods The derivat…

A Novel TTBK2 Mutation in a Chinese Pedigree with Spinocerebellar Ataxia 11 Open

Ying‐Qian Lu, Jianmin Chen, Yali Huang, Zhang‐Yu Zou · 2023

Spinocerebellar ataxia type 11 (SCA11) is a rare disease and tau tubulin kinase 2 (TTBK2) gene was the causative gene. To date, only seven SCA11 families have been reported. Here, we reported a Chinese SCA11 pedigree with cerebellar ataxia…

Novel mutations in the ABCD1 gene caused adrenomyeloneuropathy in the Chinese population Open

Raoli He, Jian Zhang, Tianwen Huang, Guoen Cai, Zhang‐Yu Zou , et al. · 2023

Background As a rare genetic disease, adrenomyeloneuropathy (AMN) is the most common adult phenotype of X-linked adrenoleukodystrophy (X-ALD). Mutations in the ABCD1 gene have been identified to cause AMN. Methods We applied clinical evalu…

Editorial: Current Concept and Translational Study in ALS–FTD Spectrum: From Genetics, Neuroinflammation to Neurodegeneration Open

Wenting Guo, Zhang‐Yu Zou, Yue Leng, Dingding Shen, Sheng Chen · 2022

EDITORIAL article Front. Mol. Neurosci., 10 June 2022Sec. Brain Disease Mechanisms https://doi.org/10.3389/fnmol.2022.935115

Neuroimmune Crosstalk Between the Peripheral and the Central Immune System in Amyotrophic Lateral Sclerosis Open

Weiyi Yu, Ji He, Xiying Cai, Zhou Yu, Zhang‐Yu Zou , et al. · 2022

Amyotrophic lateral sclerosis (ALS) is a fatal disease characterized by the degeneration and death of motor neurons. Systemic neuroinflammation contributes to the pathogenesis of ALS. The proinflammatory milieu depends on the continuous cr…

Therapeutic Effects of Batoclimab in Chinese Patients with Generalized Myasthenia Gravis: A Double-Blinded, Randomized, Placebo-Controlled Phase II Study Open

Chong Yan, Rui‐Sheng Duan, Huan Yang, Haifeng Li, Zhang‐Yu Zou , et al. · 2022

This study was registered at ClinicalTrials.gov (NCT04346888) on 15 April 2020, with the first patient enrolled on 23 July 2020.

Dynamic Alterations in Functional Connectivity Density in Amyotrophic Lateral Sclerosis: A Resting-State Functional Magnetic Resonance Imaging Study Open

Jia‐Yan Shi, Limin Cai, Jiahui Lin, Zhang‐Yu Zou, Xiaohong Zhang , et al. · 2022

Background and Aims Current knowledge on the temporal dynamics of the brain functional organization in amyotrophic lateral sclerosis (ALS) is limited. This is the first study on alterations in the patterns of dynamic functional connection …

Novel Intronic Mutations of TBK1 Promote Aberrant Splicing Modes in Amyotrophic Lateral Sclerosis Open

Ying‐Qian Lu, Jian‐Min Chen, Lin Han, Shuyan Feng, Chun‐Hui Che , et al. · 2022

TANK-binding kinase 1 ( TBK1 ) has been identified as a causative gene of amyotrophic lateral sclerosis (ALS) in the Caucasian population in 2015. Here, we sequenced for TBK1 variants in a cohort of 15 familial ALS (fALS) and 275 sporadic …

Phenotype of VCP Mutations in Chinese Amyotrophic Lateral Sclerosis Patients Open

Shuyan Feng, Lin Han, Chun‐Hui Che, Huapin Huang, Changyun Liu , et al. · 2022

Mutations in the valosin-containing protein (VCP) gene have been linked to amyotrophic lateral sclerosis (ALS) in the Caucasian populations. However, the phenotype of VCP mutations in Chinese patients with (ALS) remains unclear. Targeted n…

Novel Variants in the FIG4 Gene Associated With Chinese Sporadic Amyotrophic Lateral Sclerosis With Slow Progression Open

Chang-Yun Liu, Jilan Lin, Shuyan Feng, Chun‐Hui Che, Huapin Huang , et al. · 2022

Our findings suggest that ALS patients carrying FIG4 mutations are not common in the Chinese population and are more likely to exhibit slow progression.

Abnormal Stability of Dynamic Functional Architecture in Amyotrophic Lateral Sclerosis: A Preliminary Resting-State fMRI Study Open

Wei Jin, Jiahui Lin, Limin Cai, Jia‐Yan Shi, Xiaohong Zhang , et al. · 2021

Purpose: Static and dynamic analyses for identifying functional connectivity (FC) have demonstrated brain dysfunctions in amyotrophic lateral sclerosis (ALS). However, few studies on the stability of dynamic FC have been conducted among AL…

In‐depth peripheral CD4+ T profile correlates with myasthenic crisis Open

Xiao Huan, Sushan Luo, Huahua Zhong, Xueying Zheng, Jie Song , et al. · 2021

Objective Myasthenia gravis (MG) is an autoimmune disease caused by autoantibodies against neuromuscular junctions. Myasthenic crisis (MC) represents the most severe state of MG with high in‐hospital mortality. We aimed to identify immune …

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