Nature Reviews Neurology最新文献_第4页

Low-field MRI for equitable epilepsy diagnosis in resource-limited settings 低场磁共振成像在资源有限的环境中公平的癫痫诊断。

IF 31.2 1区医学

Nature Reviews Neurology Pub Date : 2026-07-29 DOI: 10.1038/s41582-026-01252-5

Melody T. Asukile, Josemir W. Sander, John S. Duncan

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Newborn screening is the missing step in spinal muscular atrophy care 新生儿筛查是脊髓性肌萎缩症护理中缺失的一步。

IF 31.2 1区医学

Nature Reviews Neurology Pub Date : 2026-07-28 DOI: 10.1038/s41582-026-01250-7

Jesy Nelson, Giles Lomax

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Brain-first and body-first subtypes of Lewy body disease 路易体病的脑优先亚型和体优先亚型

IF 31.2 1区医学

Nature Reviews Neurology Pub Date : 2026-07-16 DOI: 10.1038/s41582-026-01241-8

Per Borghammer, Nathalie Van Den Berge, Daniela Berg, Ryosuke Takahashi, Ronald B. Postuma, Jacob Horsager

{"title":"Brain-first and body-first subtypes of Lewy body disease","authors":"Per Borghammer, Nathalie Van Den Berge, Daniela Berg, Ryosuke Takahashi, Ronald B. Postuma, Jacob Horsager","doi":"10.1038/s41582-026-01241-8","DOIUrl":"10.1038/s41582-026-01241-8","url":null,"abstract":"People with Lewy body disease exhibit heterogeneous clinical symptoms, diverse patterns of neurodegeneration on imaging and variable Lewy body pathology at post-mortem. The brain-first versus body-first (BvB) model provides a unifying framework that may account for much of this variability. According to the BvB model, Lewy pathology originates either in autonomic nerves of the gut or other peripheral neurons (body first) or in the olfactory bulb with subsequent spread to the limbic system (brain first). Both subtypes are thought to begin largely outside the CNS, possibly triggered by exogenous factors such as infectious agents or toxins. The model is supported by several observations. First, some individuals develop autonomic dysfunction and sleep disorders years before diagnosis, whereas other individuals experience these symptoms only after diagnosis. Second, in people with prodromal sleep disorders, cardiac sympathetic denervation precedes nigrostriatal degeneration by approximately a decade, whereas in individuals without sleep disorders, these systems degenerate in the opposite sequence. Third, post-mortem studies often reveal bottom-up or top-down gradients of Lewy pathology, consistent with body-first versus olfactory-first origins. This Review provides a critical appraisal of the BvB model, highlighting supporting evidence and current limitations. The article also considers implications for diagnostics, therapy and prevention, and outlines key avenues for future research. The brain-first versus body-first model proposes that Lewy body disease can originate from different pathogenic routes, accounting for heterogeneity in symptoms, neurodegeneration patterns and post-mortem pathology. Here, Borghammer et al. evaluate the evidence for this model, outline its implications for diagnosis, therapy and prevention, and identify priorities for future research.","PeriodicalId":19085,"journal":{"name":"Nature Reviews Neurology","volume":"22 9","pages":"564-580"},"PeriodicalIF":31.2,"publicationDate":"2026-07-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148459631","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}

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Neurological symptoms in survivors of Ebola virus disease 埃博拉病毒病幸存者的神经系统症状。

IF 31.2 1区医学

Nature Reviews Neurology Pub Date : 2026-07-14 DOI: 10.1038/s41582-026-01247-2

Lisa Kiani

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Astrocytic lipid dysregulation as an early driver of neurodegeneration 星形细胞脂质失调是神经退行性变的早期驱动因素

IF 31.2 1区医学

Nature Reviews Neurology Pub Date : 2026-07-13 DOI: 10.1038/s41582-026-01238-3

Woojin Scott Kim, Glenda M. Halliday

{"title":"Astrocytic lipid dysregulation as an early driver of neurodegeneration","authors":"Woojin Scott Kim, Glenda M. Halliday","doi":"10.1038/s41582-026-01238-3","DOIUrl":"10.1038/s41582-026-01238-3","url":null,"abstract":"Astrocytes have traditionally been cast as supportive glia, but they are increasingly recognized as metabolic hubs that regulate cholesterol synthesis, fatty acid detoxification, lipid droplet dynamics and redox homeostasis in the CNS. Neurons have a limited intrinsic capacity for lipid storage and detoxification and rely heavily on astrocytes to maintain a safe lipid environment. Emerging evidence indicates that dysregulation of astrocytic lipid homeostasis precedes overt neuronal degeneration in a range of neurodegenerative diseases, including Alzheimer disease, Parkinson disease, amyotrophic lateral sclerosis, frontotemporal dementia and Huntington disease. Perturbations in astrocytic lipid handling can drive maladaptive reactive states, promote oxidative stress, impair lysosomal and mitochondrial function and disrupt neuron–glia lipid exchange, collectively creating an environment that leads to neurodegeneration. Therefore, lipid dysregulation within astrocytes could trigger or amplify neuronal vulnerability. In this Review, we assess evidence that astrocytic lipid metabolism is not solely protective or pathological but has instructive physiological roles and that astrocytic lipid dysregulation is an early driver of neurodegeneration. We critically evaluate disease-specific evidence, distinguishing correlative observations from causal mechanisms. We propose that targeting of astrocytic lipid homeostasis represents a promising strategy for preventing or minimizing neurodegeneration and opens new avenues for early detection and biomarker development. This Review highlights the central role of astrocytes in brain lipid homeostasis and reviews evidence for astrocytic lipid dysregulation in various neurodegenerative diseases, including Alzheimer disease, Parkinson disease, amyotrophic lateral sclerosis, frontotemporal dementia and Huntington disease. The authors propose that targeting of astrocytic lipid homeostasis represents a promising strategy for preventing or minimizing neurodegeneration.","PeriodicalId":19085,"journal":{"name":"Nature Reviews Neurology","volume":"22 8","pages":"485-497"},"PeriodicalIF":31.2,"publicationDate":"2026-07-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148432044","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}

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Chromosome 21 gene DSCAM drives Down syndrome phenotypes 21号染色体基因DSCAM驱动唐氏综合症表型

IF 31.2 1区医学

Nature Reviews Neurology Pub Date : 2026-07-13 DOI: 10.1038/s41582-026-01246-3

Lisa Kiani

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Lewy bodies do not correlate with neuronal degeneration in DLB 路易小体与DLB的神经元变性无关

IF 31.2 1区医学

Nature Reviews Neurology Pub Date : 2026-07-13 DOI: 10.1038/s41582-026-01245-4

Lisa Kiani

引用次数: 0

Autoantibodies could trigger neurological symptoms of long COVID 自身抗体可能引发长冠状病毒的神经系统症状。

IF 31.2 1区医学

Nature Reviews Neurology Pub Date : 2026-07-09 DOI: 10.1038/s41582-026-01243-6

Heather Wood

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Ataxia Global Initiative recommend MRI end-points for clinical trials in ataxias 共济失调全球倡议推荐MRI终点在共济失调的临床试验。

IF 31.2 1区医学

Nature Reviews Neurology Pub Date : 2026-06-30 DOI: 10.1038/s41582-026-01242-7

Hélio A. G. Teive

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Moving artificial intelligence from research to real-world clinical use in neurology 将人工智能从研究转移到神经病学的实际临床应用。

IF 31.2 1区医学

Nature Reviews Neurology Pub Date : 2026-06-29 DOI: 10.1038/s41582-026-01225-8

James T. Teo, Arman Eshaghi, Mark P. Richardson, Lara Jehi, Sandor Beniczky

{"title":"Moving artificial intelligence from research to real-world clinical use in neurology","authors":"James T. Teo, Arman Eshaghi, Mark P. Richardson, Lara Jehi, Sandor Beniczky","doi":"10.1038/s41582-026-01225-8","DOIUrl":"10.1038/s41582-026-01225-8","url":null,"abstract":"Artificial intelligence (AI) applications in neurology have reached an inflection point. Despite US Food and Drug Administration approval of numerous algorithms in neuroimaging, neurophysiology, genetics and chatbots, their real-world impact remains limited. This disconnect between research promise and clinical reality represents a gap in understanding how to translate AI algorithms into clinical benefit for patients globally. In this Perspective, we examine the challenges that prevent clinical AI use in neurology moving beyond pilot studies towards meaningful clinical impact. We consider the steps required in the process of translation, including research, validation of AI models, regulatory approval pathways and clinical implementation. We discuss implementation of AI models as stand-alone products versus embedded platforms, and the requirements for sustainable deployment. Beyond traditional clinical decision support tools, we examine paraclinical applications of AI, including chatbots and ambient voice documentation. We recommend expanding capacity for prospective validation and scaling by implementing and validating technologies across multiple sites and countries, which requires infrastructure from long-term partnerships. Neurology must shift from asking whether AI can work to understanding how to use it safely at scale. Numerous artificial intelligence (AI) applications are being developed in neurology, but their real-world impact remains limited. In this Perspective, Teo et al. examine the challenges that prevent clinical AI use in neurology moving beyond pilot studies towards meaningful clinical impact.","PeriodicalId":19085,"journal":{"name":"Nature Reviews Neurology","volume":"22 8","pages":"498-506"},"PeriodicalIF":31.2,"publicationDate":"2026-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148348912","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