333 results match your criteria: "National Key Clinical Department and Key Discipline of Neurology[Affiliation]"

Clinical efficacy of efgartigimod combined with intravenous methylprednisolone in the acute phase of neuromyelitis optica spectrum disorders.

Orphanet J Rare Dis

December 2024

Department of Neurology, The First Affiliated Hospital, Guangdong Provincial Key Laboratory of Diagnosis and Treatment of Major Neurological Diseases, National Key Clinical Department and Key Discipline of Neurology, Sun Yat-Sen University, No. 58 Zhongshan Road 2, Guangzhou, 510080, China.

Background: Neuromyelitis Optica Spectrum Disorders (NMOSD) comprise a group of autoimmune-mediated, inflammatory, demyelinating central nervous system diseases caused by aquaporin-4 (AQP4) IgG autoantibodies. Efgartigimod is a human IgG Fc fragment that reduces antibody titers by targeting the neonatal Fc receptor (FcRn). This study documents the efficacy of efgartigimod combined with intravenous methylprednisolone (IVMP) in the acute phase of NMOSD.

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Mitochondrial dysfunction of Astrocyte induces cell activation under high salt condition.

Heliyon

December 2024

Department of Neurology, The First Affiliated Hospital, Sun Yat-sen University, Guangdong Provincial Key Laboratory of Diagnosis and Treatment of Major Neurological Diseases, National Key Clinical Department and Key Discipline of Neurology, No.58 Zhongshan Road 2, Guangzhou, 510080, China.

Excess dietary sodium can accumulate in brain and adversely affect human health. We have confirmed in previous studies that high salt can induce activation of astrocyte manifested by the secretion of various inflammatory factors. In order to further explore the effect of high salt on the internal cell metabolism of astrocytes, RNA sequencing was performed on astrocytes under high salt environment, which indicated the oxidative phosphorylation and glycolysis pathways of astrocytes were downregulated.

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The use of F-wave study may help to gain insight into electrophysiological significance of spinocerebellar Ataxias (SCAs). Particularly, the difference of F-wave features between Chinese SCA1, SCA2 and SCA3 patients were scarcely reported. 20 SCA1, 20 SCA2, 46 SCA3 patients and 30 healthy controls underwent nerve (median, ulnar, tibial) conduction and F-wave studies, and electrophysiology parameters were compared between them.

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Global and Regional Brain Grey and White Matter Morphometry Alterations in Type 1, 2, and 3 Spinocerebellar Ataxias (SCAs) Patients.

Cerebellum

December 2024

Department of Radiology, The First Affiliated Hospital, Sun Yat-Sen University, 58th, The Second Zhongshan Road, Guangzhou, 518000, Guangdong, People's Republic of China.

Spinocerebellar ataxias (SCAs) types 1, 2, and 3 are the most common subtypes of SCAs. However, the atrophy patterns of these three subtypes still need to be fully clarified. In this study, a total of 130 genetically confirmed SCA patients (SCA1: n = 16; SCA2: n = 13; symptomatic SCA3: n = 76; pre-symptomatic SCA3: n = 25) along with 65 age- and sex-matched healthy controls (HCs) were enrolled.

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Association between gastroesophageal reflux disease and the risk of four ear diseases: A bidirectional Mendelian randomization study.

Asian J Surg

November 2024

Department of Neurology, The First Affiliated Hospital, Sun Yat-sen University, Guangdong Provincial Key Laboratory of Diagnosis and Treatment of Major Neurological Diseases, National Key Clinical Department and Key Discipline of Neurology, No.58 Zhongshan Road 2, Guangzhou, 510080, China. Electronic address:

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Myasthenia gravis (MG) is an autoimmune neuromuscular disorder that most frequently affects the extraocular muscles (EOMs), which causes symptoms such as ptosis and restricted eye movement. The EOMs in MG patients are representative of autoimmune inflammatory changes in muscle tissue. Currently, there is no reliable, and sensitive imaging technique for monitoring EOM changes to assist in the evaluation of underlying pathological changes.

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Circulating CD45RAFoxp3 Treg cells serve as a biomarker for predicting minimal clinical manifestations status of myasthenia gravis.

Life Sci

December 2024

Department of Neurology, Zhengzhou University People's Hospital, Henan Provincial People's Hospital, Zhengzhou 450003, Henan, China. Electronic address:

Aims: Regulatory T cells (Tregs) are key mediators of the induction of immune tolerance; however, the mechanisms by which they regulate myasthenia gravis (MG) are not fully understood. This study aimed to explore the characteristics of Tregs and their subpopulations in the peripheral blood of patients with minimal clinical manifestations (MM) of MG and identify biomarkers that predict MM-MG for treatment guidance.

Materials And Methods: The clinical data of patients with general MG who visited our hospital were retrospectively analyzed.

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Alzheimer's disease (AD) is hallmarked by amyloid-β (Aβ) plaques and hyperphosphorylated tau (p-tau) neurofibrillary tangles. While Aβ-centric therapies have shown promise, the complex pathology of AD requires a multifaceted therapeutic approach. The weak association between Aβ levels and cognitive decline highlights the need for alternative theranostic strategies.

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Background: The projection indicates that compound drought and hot events (CDHEs) will intensify, posing risks to cardiovascular health by potentially increasing stroke incidents. However, epidemiological evidence on this topic remains scarce. This study investigates the association between exposure to CDHEs and the risk of daily stroke admissions, specifically examining the effects on various stroke categories such as Subarachnoid Hemorrhage (SAH), Intracerebral Hemorrhage (ICH), Ischemic Stroke (IS), Transient Ischemic Attack (TIA), and other types of stroke.

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SARS-CoV-2 spike S1 protein induces microglial NLRP3-dependent neuroinflammation and cognitive impairment in mice.

Exp Neurol

January 2025

Department of Neurology, The First Affiliated Hospital, Sun Yat-sen University, Guangdong Provincial Key Laboratory of Diagnosis and Treatment of Major Neurological Diseases, National Key Clinical Department and Key Discipline of Neurology, No.58 Zhongshan Road 2, Guangzhou 510080, China. Electronic address:

Cognitive impairment is often found at the acute stages and sequelae of coronavirus disease 2019 (COVID-19), and the underlying mechanisms remain unclear. The S1 protein from severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) might be a cause of cognitive impairment associated with COVID-19. The nucleotide-binding domain, leucine-rich-containing family, pyrin domain-containing-3 (NLRP3) inflammasome and neuroinflammation play important roles in Alzheimer's disease (AD) with cognitive impairment.

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Combination of low-dose, long-term immunoglobulin and mirtazapine is effective in progressive multifocal leukoencephalopathy caused by JC virus infection.

BMC Neurol

October 2024

Department of Neurology, The First Affiliated Hospital, Sun Yat-sen University, Guangdong Provincial Key Laboratory of Diagnosis and Treatment of Major Neurological Diseases, National Key Clinical Department and Key Discipline of Neurology, Guangzhou, China.

Article Synopsis
  • Progressive multifocal leukoencephalopathy (PML) is a severe brain disease caused by the John Cunningham Virus (JCV), with no widely accepted antiviral treatment available.
  • A case study reports a patient with previous immunosuppressive therapy for dermatomyositis, who was diagnosed with PML and treated with a combination of low-dose immunoglobulin and mirtazapine, leading to significant clinical improvement and a decrease in JCV levels to zero after four months.
  • This findings suggest that this combined treatment approach may effectively regulate the immune response and control the progression of PML, presenting a potential new therapy option.
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Pathogenic Th17 cells are a potential therapeutic target for tacrolimus in AChR-myasthenia gravis patients.

J Neuroimmunol

November 2024

Department of Neurology, National Key Clinical Department and Key Discipline of Neurology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou 510080, China. Electronic address:

Article Synopsis
  • The study examined how tacrolimus (TAC) affects CD4+ T cell subsets in 41 patients with AChR-MG over 12 weeks.
  • Results showed that 27 patients responded positively to TAC treatment by exhibiting improved myasthenia gravis scores, while 14 did not respond.
  • The findings suggest that TAC's clinical effectiveness is linked to its ability to lower Th17 and pathogenic Th17 cells, which may improve management of myasthenia gravis by modulating the immune response.
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Sleep dysfunction and gut dysbiosis related amino acids metabolism disorders in cynomolgus monkeys after middle cerebral artery occlusion.

Exp Neurol

December 2024

Department of Neurology, The First Affiliated Hospital, Sun Yat-sen University, Guangdong Provincial Key Laboratory of Diagnosis and Treatment of Major Neurological Diseases, National Key Clinical Department and Key Discipline of Neurology, No. 58 Zhongshan Road 2, Guangzhou 510080, China. Electronic address:

Introduction: This study aimed to explore the characteristics of post-stroke sleep dysfunction and verify their association with gut dysbiosis and the related amino acid metabolism disorders. This was achieved by using fecal microbiota transplantation (FMT) in a non-human primate stroke model.

Methods: Twenty adult male cynomolgus monkeys were divided into the sham (n = 4), middle cerebral artery occlusion (MCAO, n = 5), MCAO + FMT (n = 3), and donor (n = 8) groups.

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Treatment of neurologic pathology and inflammation in Machado-Joseph disease through in vivo self-assembled siRNA.

Brain

September 2024

Nanjing Drum Tower Hospital Center of Molecular Diagnostic and Therapy, State Key Laboratory of Pharmaceutical Biotechnology, Jiangsu Engineering Research Center for MicroRNA Biology and Biotechnology, NJU Advanced Institute of Life Sciences (NAILS), School of Life Sciences, Nanjing University, Nanjing, Jiangsu 210023, China.

Machado-Joseph disease, also known as Spinocerebellar ataxia type 3 (MJD/SCA3), is a fatal autosomal dominant hereditary ataxia characterized by cerebellar ataxia resulting from the abnormal expansion of CAG repeats in exon 10 of the ATXN3 gene. Presently, there is no effective treatment for SCA3. Small interfering RNAs (siRNAs) are emerging as potential therapeutic strategies to specifically target the disease-causing mutant ATXN3 (mATXN3) protein.

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Ruthenium(ii)-Arene Complex Triggers Immunogenic Ferroptosis for Reversing Drug Resistance.

J Med Chem

November 2024

MOE Key Laboratory of Bioinorganic and Synthetic Chemistry, State Key Laboratory of Anti-Infective Drug Development, IGCME, GBRCE for Functional Molecular Engineering, School of Chemistry, Sun Yat-Sen University, Guangzhou 510000, PR China.

Chemoresistance remains an arduous challenge in oncology, but ferroptosis shows potential for overcoming it by stimulating the immune system. Herein, a novel high-performance ruthenium(II)-based arene complex [Ru(η--cym)(BTBpy)Cl] () is developed for ferroptosis-enhanced antitumor immunity and drug resistance reversal via glutathione (GSH) metabolism imbalance. shows significantly enhanced antiproliferation activity against cisplatin (CDDP)-resistant lung cancer cells (A549R), with 26.

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Ofatumumab treatment for severe refractory anti-NMDAR encephalitis: A case series.

J Neuroimmunol

November 2024

Department of Neurology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, People's Republic of China; Guangdong Provincial Key Laboratory of Diagnosis and Treatment of Major Neurological Diseases, National Key Clinical Department and Key Discipline of Neurology, Guangzhou, People's Republic of China. Electronic address:

Rituximab is recommended as the preferred second-line immunotherapy for autoimmune encephalitis (AE). However, Ofatumumab (OFA), a novel fully human anti-CD20 antibody, has been reported infrequently in patients with AE. Among the various forms of AE, anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis is the most common and severe.

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Circular RNAs (circRNAs) are involved in several neurological disorders; however, the mechanisms underlying their involvement remain to be clarified. We attempted to explore the expression profiles of circRNAs and their potential functions and mechanisms in the pathogenesis of intracerebral hemorrhage (ICH) in Northern Chinese males. The microarray results showed that 50 circRNAs were significantly upregulated, while 194 circRNAs were significantly downregulated in ICH patients compared with healthy controls (p < 0.

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Conventional dendritic cells are more activated in the hyperplastic Thymus of myasthenia gravis patients.

J Neuroimmunol

October 2024

Department of Neurology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou 510080, China; Guangdong Provincial Key Laboratory of Diagnosis and Treatment of Major Neurological Diseases, Guangzhou 510080, China; National Key Clinical Department and Key Discipline of Neurology, Guangzhou 510080, China. Electronic address:

Introduction: Dendritic cells (DCs) are crucial to form ectopic germinal centers (GCs) in the hyperplastic thymus (HT), which are typically found in anti-acetylcholine receptor autoantibody-positive myasthenia gravis (MG) patients. However, the characteristics of such DCs in the HT and their roles in thymic hyperplasia formation remain unclear.

Methods: We collected thymic tissue from MG patients and patients who underwent cardiac surgery.

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Unlocking multi-photon excited luminescence in pyrazolate trinuclear gold clusters for dynamic cell imaging.

Nat Commun

August 2024

GBRCE for Functional Molecular Engineering, MOE Key Laboratory of Bioinorganic and Synthetic Chemistry, Southern Marine Science and Engineering Guangdong Laboratory (Zhuhai), School of Chemical Engineering and Technology, Sun Yat-sen University, Zhuhai, 519082, PR China.

The family of coinage-metal-based cyclic trinuclear complexes exhibits abundant photophysical properties, promising for diverse applications. However, their utility in biochemistry is often hindered by large particle size and strong hydrophobicity. Meanwhile, the investigation into multi-photon excited luminescence within this family remained undocumented, limiting their potential in bio-imaging.

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Iridium(III) Photosensitizers Induce Simultaneous Pyroptosis and Ferroptosis for Multi-Network Synergistic Tumor Immunotherapy.

Angew Chem Int Ed Engl

December 2024

MOE Key Laboratory of Bioinorganic and Synthetic Chemistry, State Key Laboratory of Anti-Infective Drug Development, IGCME, GBRCE for Functional Molecular Engineering, School of Chemistry, Sun Yat-Sen University, Guangzhou, 510006, P. R. China.

The integration of pyroptosis and ferroptosis hybrid cell death induction to augment immune activation represents a promising avenue for anti-tumor treatment, but there is a lack of research. Herein, we developed two iridium (III)-triphenylamine photosensitizers, IrC and IrF, with the capacity to disrupt redox balance and induce photo-driven cascade damage to DNA and Kelch-like ECH-associated protein 1 (KEAP1). The activation of the absent in melanoma 2 (AIM2)-related cytoplasmic nucleic acid-sensing pathway, triggered by damaged DNA, leads to the induction of gasdermin D (GSDMD)-mediated pyroptosis.

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Neural correlates of anxiety in adult-onset isolated dystonia.

Neuroscience

October 2024

Department of Neurology, The First Affiliated Hospital, Sun Yat-sen University, Guangdong Provincial Key Laboratory for Diagnosis and Treatment of Major Neurological Diseases, National Key Clinical Department and Key Discipline of Neurology, Guangzhou 510080, China. Electronic address:

Psychiatric disturbances are commonly associated with adult-onset isolated dystonia (AOID); however, the mechanisms underlying psychiatric abnormalities in AOID remain unknown. We aimed to investigate the structural and functional brain changes in AOID patients with anxiety, and identify imaging biomarkers for diagnosing anxiety. Structural and functional magnetic resonance was performed on 69 AOID patients and 35 healthy controls (HCs).

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Article Synopsis
  • - The study investigates the role of Sestrin2 (Sesn2) in the myogenic differentiation of C2C12 myoblasts and its effects in mdx mice, a model for Duchenne muscular dystrophy (DMD).
  • - Results indicate that reducing Sesn2 levels impairs myogenic differentiation in myoblasts and that miR-182-5p negatively regulates Sesn2, further inhibiting myogenesis.
  • - In an in vivo DMD model, knocking down Sesn2 decreased Myogenin (Myog) levels and increased Pax7 expression, while overexpressing Sesn2 improved Myog levels and boosted slow-switch myofiber proportion, highlighting Sesn2’s importance in
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Familial mesial temporal lobe epilepsy phenotype is associated with novel LGI1 variants: A report of two families.

Seizure

August 2024

Department of Neurology, The First Affiliated Hospital, Sun Yat-sen University, Guangdong Provincial Key Laboratory of Diagnosis and Treatment of Major Neurological Diseases, National Key Clinical Department and Key Discipline of Neurology, Guangzhou 510080, PR China. Electronic address:

Objective: To expand the clinical phenotype and mutation spectrum of familial mesial temporal lobe epilepsy (FMTLE) and provide a new perspective for exploring the pathological mechanisms of epilepsy caused by leucine-rich glioma inactivated 1 (LGI1) variants.

Methods: We reported clinical data from two families with FMTLE and screened patients for variants in the LGI1 gene using Whole-exome sequencing and Sanger sequencing. The clinical features of FMTLE were analysed.

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Application of GWAS summary data and drug-induced gene expression profiles of neural progenitor cells in psychiatric drug prioritization analysis.

Mol Psychiatry

January 2025

Program in Bioinformatics, Zhongshan School of Medicine and The Fifth Affiliated Hospital, Sun Yat-sen University, Guangzhou, 510080, Guangdong, China.

Article Synopsis
  • Common psychiatric disorders present a major global healthcare challenge, but developing effective drugs is tough due to the complexities of targeting multiple disease-related genes.
  • A new drug prioritization strategy was proposed that focuses on how drugs can selectively affect genes linked to psychiatric conditions like schizophrenia, depression, and bipolar disorder, using existing genetic and drug response data.
  • The findings indicate that this approach not only identifies known effective drugs but also highlights the potential of targeting multiple pathways in the drug development process for these common mental health disorders.
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