Nature Reviews Neurology最新文献

Preparing for disease-modifying dementia therapies in the UK 英国为改变疾病的痴呆症疗法做准备

IF 38.1 1区医学

Nature Reviews Neurology Pub Date : 2024-09-17 DOI: 10.1038/s41582-024-01022-1

Claudia Cooper, Charles R. Marshall, Jonathan M. Schott, Sube Banerjee

引用次数: 0

NMOSD and MOGAD: an evolving disease spectrum NMOSD 和 MOGAD：不断演变的疾病谱

IF 38.1 1区医学

Nature Reviews Neurology Pub Date : 2024-09-13 DOI: 10.1038/s41582-024-01014-1

Akiyuki Uzawa, Frederike Cosima Oertel, Masahiro Mori, Friedemann Paul, Satoshi Kuwabara

{"title":"NMOSD and MOGAD: an evolving disease spectrum","authors":"Akiyuki Uzawa, Frederike Cosima Oertel, Masahiro Mori, Friedemann Paul, Satoshi Kuwabara","doi":"10.1038/s41582-024-01014-1","DOIUrl":"https://doi.org/10.1038/s41582-024-01014-1","url":null,"abstract":"Neuromyelitis optica (NMO) spectrum disorder (NMOSD) is a relapsing inflammatory disease of the CNS, characterized by the presence of serum aquaporin 4 (AQP4) autoantibodies (AQP4-IgGs) and core clinical manifestations such as optic neuritis, myelitis, and brain or brainstem syndromes. Some people exhibit clinical characteristics of NMOSD but test negative for AQP4-IgG, and a subset of these individuals are now recognized to have serum autoantibodies against myelin oligodendrocyte glycoprotein (MOG) — a condition termed MOG antibody-associated disease (MOGAD). Therefore, the concept of NMOSD is changing, with a disease spectrum emerging that includes AQP4-IgG-seropositive NMOSD, MOGAD and double-seronegative NMOSD. MOGAD shares features with NMOSD, including optic neuritis and myelitis, but has distinct pathophysiology, clinical profiles, neuroimaging findings (including acute disseminated encephalomyelitis and/or cortical encephalitis) and biomarkers. AQP4-IgG-seronegative NMOSD seems to be a heterogeneous condition and requires further study. MOGAD can manifest as either a monophasic or a relapsing disease, whereas NMOSD is usually relapsing. This Review summarizes the history and current concepts of NMOSD and MOGAD, comparing epidemiology, clinical features, neuroimaging, pathology and immunology. In addition, we discuss new monoclonal antibody therapies for AQP4-IgG-seropositive NMOSD that target complement, B cells or IL-6 receptors, which might be applied to MOGAD in the near future.","PeriodicalId":19085,"journal":{"name":"Nature Reviews Neurology","volume":null,"pages":null},"PeriodicalIF":38.1,"publicationDate":"2024-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142175002","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Adaptive deep brain stimulation shows promise 自适应深部脑刺激技术大有可为

IF 38.1 1区医学

Nature Reviews Neurology Pub Date : 2024-09-12 DOI: 10.1038/s41582-024-01019-w

Ian Fyfe

引用次数: 0

Hijacked macrophages sustain glioblastoma cells 被劫持的巨噬细胞可维持胶质母细胞瘤细胞的生长

IF 38.1 1区医学

Nature Reviews Neurology Pub Date : 2024-09-12 DOI: 10.1038/s41582-024-01018-x

Ian Fyfe

引用次数: 0

Free DNA activates secondary stroke mechanism 游离 DNA 激活二级中风机制

IF 38.1 1区医学

Nature Reviews Neurology Pub Date : 2024-09-12 DOI: 10.1038/s41582-024-01020-3

Ian Fyfe

引用次数: 0

Blood profile indicates central inflammation in frontotemporal lobar degeneration 血液特征显示额颞叶变性的中枢炎症

IF 38.1 1区医学

Nature Reviews Neurology Pub Date : 2024-09-12 DOI: 10.1038/s41582-024-01021-2

Ian Fyfe

引用次数: 0

Tau phosphorylation correlates with multiple sclerosis disease course Tau 磷酸化与多发性硬化症的病程有关

IF 38.1 1区医学

Nature Reviews Neurology Pub Date : 2024-09-12 DOI: 10.1038/s41582-024-01017-y

Heather Wood

引用次数: 0

Pituitary adenylate cyclase-activating polypeptide signalling as a therapeutic target in migraine 将垂体腺苷酸环化酶激活多肽信号作为偏头痛的治疗靶点

IF 38.1 1区医学

Nature Reviews Neurology Pub Date : 2024-09-10 DOI: 10.1038/s41582-024-01011-4

Håkan Ashina, Rune H. Christensen, Debbie L. Hay, Amynah A. Pradhan, Jan Hoffmann, Dora Reglodi, Andrew F. Russo, Messoud Ashina

{"title":"Pituitary adenylate cyclase-activating polypeptide signalling as a therapeutic target in migraine","authors":"Håkan Ashina, Rune H. Christensen, Debbie L. Hay, Amynah A. Pradhan, Jan Hoffmann, Dora Reglodi, Andrew F. Russo, Messoud Ashina","doi":"10.1038/s41582-024-01011-4","DOIUrl":"https://doi.org/10.1038/s41582-024-01011-4","url":null,"abstract":"Migraine is a disabling neurological disorder that affects more than one billion people worldwide. The clinical presentation is characterized by recurrent headache attacks, which are often accompanied by photophobia, phonophobia, nausea and vomiting. Although the pathogenesis of migraine remains incompletely understood, mounting evidence suggests that specific signalling molecules are involved in the initiation and modulation of migraine attacks. These signalling molecules include pituitary adenylate cyclase-activating polypeptide (PACAP), a vasoactive peptide that is known to induce migraine attacks when administered by intravenous infusion to people with migraine. Discoveries linking PACAP to migraine pathogenesis have led to the development of drugs that target PACAP signalling, and a phase II trial has provided evidence that a monoclonal antibody against PACAP is effective for migraine prevention. In this Review, we explore the molecular and cellular mechanisms of PACAP signalling, shedding light on its role in the trigeminovascular system and migraine pathogenesis. We then discuss emerging therapeutic strategies that target PACAP signalling for the treatment of migraine and consider the research needed to translate the current knowledge into a treatment for migraine in the clinic.","PeriodicalId":19085,"journal":{"name":"Nature Reviews Neurology","volume":null,"pages":null},"PeriodicalIF":38.1,"publicationDate":"2024-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142166237","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Assessing disease progression and treatment response in progressive multiple sclerosis 评估进展期多发性硬化症的疾病进展和治疗反应

IF 38.1 1区医学

Nature Reviews Neurology Pub Date : 2024-09-09 DOI: 10.1038/s41582-024-01006-1

Giancarlo Comi, Gloria Dalla Costa, Bruno Stankoff, Hans-Peter Hartung, Per Soelberg Sørensen, Patrick Vermersch, Letizia Leocani

{"title":"Assessing disease progression and treatment response in progressive multiple sclerosis","authors":"Giancarlo Comi, Gloria Dalla Costa, Bruno Stankoff, Hans-Peter Hartung, Per Soelberg Sørensen, Patrick Vermersch, Letizia Leocani","doi":"10.1038/s41582-024-01006-1","DOIUrl":"https://doi.org/10.1038/s41582-024-01006-1","url":null,"abstract":"Progressive multiple sclerosis poses a considerable challenge in the evaluation of disease progression and treatment response owing to its multifaceted pathophysiology. Traditional clinical measures such as the Expanded Disability Status Scale are limited in capturing the full scope of disease and treatment effects. Advanced imaging techniques, including MRI and PET scans, have emerged as valuable tools for the assessment of neurodegenerative processes, including the respective role of adaptive and innate immunity, detailed insights into brain and spinal cord atrophy, lesion dynamics and grey matter damage. The potential of cerebrospinal fluid and blood biomarkers is increasingly recognized, with neurofilament light chain levels being a notable indicator of neuro-axonal damage. Moreover, patient-reported outcomes are crucial for reflecting the subjective experience of disease progression and treatment efficacy, covering aspects such as fatigue, cognitive function and overall quality of life. The future incorporation of digital technologies and wearable devices in research and clinical practice promises to enhance our understanding of functional impairments and disease progression. This Review offers a comprehensive examination of these diverse evaluation tools, highlighting their combined use in accurately assessing disease progression and treatment efficacy in progressive multiple sclerosis, thereby guiding more effective therapeutic strategies.","PeriodicalId":19085,"journal":{"name":"Nature Reviews Neurology","volume":null,"pages":null},"PeriodicalIF":38.1,"publicationDate":"2024-09-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142160611","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Stiff-person syndrome and related disorders — diagnosis, mechanisms and therapies 僵人综合征及相关疾病--诊断、机制和疗法

IF 38.1 1区医学

Nature Reviews Neurology Pub Date : 2024-09-03 DOI: 10.1038/s41582-024-01012-3

Marinos C. Dalakas

{"title":"Stiff-person syndrome and related disorders — diagnosis, mechanisms and therapies","authors":"Marinos C. Dalakas","doi":"10.1038/s41582-024-01012-3","DOIUrl":"https://doi.org/10.1038/s41582-024-01012-3","url":null,"abstract":"Stiff-person syndrome (SPS) is the prototypical and most common autoimmune neuronal hyperexcitability disorder. It presents with stiffness in the limbs and axial muscles, stiff gait with uncontrolled falls, and episodic painful muscle spasms triggered by anxiety, task-specific phobias and startle responses, collectively leading to disability. Increased awareness of SPS among patients and physicians has created concerns about diagnosis, misdiagnosis and treatment. This Review addresses the evolving diagnostic challenges in SPS and overlapping glutamic acid decarboxylase (GAD) antibody spectrum disorders, highlighting the growing number of overdiagnoses and focusing on the progress made in our understanding of SPS pathophysiology, antibodies against GAD and other inhibitory synaptic antigens, and the fundamentals of neuronal hyperexcitability. It considers the role of impaired GABAergic or glycinergic inhibition in the cortex and at multiple levels in the neuraxis; the underlying autoimmunity and involvement of GAD antibodies; immunopathogenic mechanisms beyond antibodies, including environmental triggers; familial and immunogenetic susceptibility; and potential T cell cytotoxicity. Finally, the mechanistic rationale for target-specific therapeutic interventions is presented along with the available therapeutic approaches, including enhancers of GABA signalling drugs and immunotherapies.","PeriodicalId":19085,"journal":{"name":"Nature Reviews Neurology","volume":null,"pages":null},"PeriodicalIF":38.1,"publicationDate":"2024-09-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142123702","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0