Featured
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Review Article |
NMOSD and MOGAD: an evolving disease spectrum
This Review summarizes the history and current concepts of neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), including epidemiology, clinical and neuroimaging features and pathophysiology. It also discusses new molecularly targeted therapies for NMOSD that might be also applied to MOGAD in the future.
- Akiyuki Uzawa
- , Frederike Cosima Oertel
- & Satoshi Kuwabara
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Research Highlight |
Tau phosphorylation correlates with multiple sclerosis disease course
New research adds to growing evidence of altered tau phosphorylation in multiple sclerosis.
- Heather Wood
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Review Article |
Pituitary adenylate cyclase-activating polypeptide signalling as a therapeutic target in migraine
Pituitary adenylate cyclase-activating polypeptide signalling has been linked to migraine pathogenesis. In this Review, Ashina and co-workers explore the molecular and cellular mechanisms of pituitary adenylate cyclase-activating polypeptide signalling and discuss emerging therapeutic strategies to target this pathway for migraine treatment.
- Håkan Ashina
- , Rune H. Christensen
- & Messoud Ashina
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Review Article |
Assessing disease progression and treatment response in progressive multiple sclerosis
The approval of therapies for progressive multiple sclerosis has heightened the need for thorough assessment of disease progression and treatment response. This Review provides a comprehensive summary of available and emerging techniques, including advanced imaging, fluid biomarkers and patient-reported outcomes, highlighting their combined use for the accurate assessment of disease.
- Giancarlo Comi
- , Gloria Dalla Costa
- & Letizia Leocani
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Expert Recommendation |
The influence of MOGAD on diagnosis of multiple sclerosis using MRI
Myelin oligodendrocyte glycoprotein antibody-associated disease is an immune-mediated demyelinating disease that is distinct from multiple sclerosis but shares some of its characteristics. This Expert Recommendation, based on a Magnetic Resonance Imaging in MS workshop, proposes a diagnostic algorithm for the differential diagnosis of myelin oligodendrocyte glycoprotein antibody-associated disease and multiple sclerosis, using serological, imaging and clinical features.
- Ruth Geraldes
- , Georgina Arrambide
- & Jacqueline Palace
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Review Article |
Stiff-person syndrome and related disorders — diagnosis, mechanisms and therapies
Stiff-person syndrome is an autoimmune neuronal hyperexcitability disorder that causes limb stiffness, painful spasms and falls, and increased awareness of the disease is creating diagnostic and management challenges. In this Review, Dalakas provides an overview of the current clinical and mechanistic understanding of stiff-person syndrome and related disorders and discusses current and emerging therapeutic interventions.
- Marinos C. Dalakas
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Review Article |
Insights from 25 years of onabotulinumtoxinA in migraine — mechanisms and management
OnabotulinumtoxinA was first linked to beneficial effects in migraine 25 years ago and has since become a widely used treatment for chronic migraine. In this Review, Pozo-Rosich and colleagues consider the impact that onabotulinumtoxinA has had on the management of chronic migraine and on the research field.
- Patricia Pozo-Rosich
- , Alicia Alpuente
- & Rami Burstein
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Research Highlight |
Antisense oligonucleotide shows potential in Charcot–Marie–Tooth disease
Antisense oligonucleotide treatment has therapeutic potential in a severe form of Charcot–Marie–Tooth disease, according to a new study.
- Ian Fyfe
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Review Article |
Peripheral nervous system immune-related adverse events due to checkpoint inhibition
Some patients who are treated with checkpoint inhibitors experience peripheral nervous system (PNS) immune-related adverse events (irAEs). O’Hare and Guidon describe the spectrum of PNS irAE phenotypes, discuss their underlying mechanisms and outline a consensus-based, pathophysiology-driven approach to their clinical management.
- Meabh O’Hare
- & Amanda C. Guidon
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News & Views |
Disentangling clinical and biological trajectories of neurodegenerative diseases
In recent years, we have seen a shift towards defining sporadic neurodegenerative diseases as a biological continuum. Here, we discuss the risks associated with this shift, emphasize the importance of maintaining a strong connection between disease definitions and subsequent clinical outcomes, and suggest clinicobiological frameworks to disentangle multiple discrete nosological entities.
- Nicolas Villain
- & Vincent Planche
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Review Article |
Identifying and reducing risks of neurological complications associated with vaccination
Vaccination has transformed the global fight against infectious diseases and has a highly favourable benefit–risk profile in most people, although adverse events, including neurological complications, can occasionally occur. This article reviews the links between vaccination and neurological disease and considers the role of neurologists in identifying safety signals and managing risk.
- Lahiru Handunnetthi
- , Maheshi N. Ramasamy
- & David P. J. Hunt
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Review Article |
Gaucher disease provides a unique window into Parkinson disease pathogenesis
The association between the rare, monogenic lysosomal storage disorder Gaucher disease and Parkinson disease has provided insights into the pathogenesis of this far more common neurodegenerative disease. Here, Sidransky and colleagues review the knowledge gained from decades of Gaucher disease research and explore the relationship between GBA1 and parkinsonism.
- Ellen Hertz
- , Yu Chen
- & Ellen Sidransky
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Comment |
Food is brain medicine — relevance and translation to neurology
The importance of diet for brain health is increasingly recognized by neurologists, but many neurological disorders impair the ability of individuals to eat healthily. A new initiative known as ‘Food Is Medicine’ has the potential to facilitate healthier eating among people with neurological disorders to improve and maintain brain health.
- Mitchell S. V. Elkind
- & Kevin G. Volpp
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News & Views |
Environmental–genetic interactions in ageing and dementia across Latin America
A new study on dementia prevalence in Latin America has found a stronger effect of social determinants of health than ancestry effects specific to this region, highlighting the need for interventions involving lifestyle changes, healthcare access and education.
- Agustin Ibanez
- & Andrea Slachevsky
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Research Highlight |
Plasma extracellular vesicle biomarkers for frontotemporal dementia and related disorders
Concentrations of TDP-43 and tau in extracellular vesicles extracted from blood show potential as biomarkers for frontotemporal dementia, amyotrophic lateral sclerosis and progressive supranuclear palsy.
- Lisa Kiani
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News & Views |
Precision neuroimmunology in multiple sclerosis — the horizon is near
Immunological profiling has revealed biological signatures of multiple sclerosis (MS) that could help with early, accurate diagnosis of the disease and with identifying disease subtypes that could inform treatment decisions. The findings are important steps along the path towards precision medicine for people with MS.
- Jiwon Oh
- & Amit Bar-Or
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Review Article |
Brain–body mechanisms contribute to sexual dimorphism in amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis (ALS) differs considerably in prevalence and manifestation between sexes. This Review summarizes sexual dimorphism in the epidemiology, clinical presentation and disease mechanisms of ALS and explores the role of brain–body interactions in driving sex-dependent pathogenesis.
- Sarah M. Jacob
- , Sukyoung Lee
- & Minh Dang Nguyen
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Comment |
Spinal cord stimulation for the treatment of chronic pain
Spinal cord stimulation is seen as a last-resort therapy for the treatment of chronic pain. Controversies surrounding the treatment might be addressed through collaborative efforts to conduct innovative clinical trials and reach consensus on treatment guidelines.
- Cecile C. de Vos
- & Kaare Meier
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Comment |
Focused ultrasound brain therapy is a new tool in the box
Similar to any innovation that disrupts the status quo, the advent of magnetic resonance-guided focused ultrasound in neurology was accompanied by controversy and debate. However, evidence suggests that this therapeutic tool, which is already widely used to treat tremor and Parkinson disease, is gaining acceptance and will become a viable therapeutic option for various other neurological conditions in the near future.
- Raúl Martínez-Fernández
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Comment |
Neuromodulation for severe brain injury: time for a paradigm shift?
Neuromodulation represents a promising approach for promoting neural plasticity following a brain injury, especially for non-communicative patients with prolonged disorders of consciousness. However, so far, the outcomes have been limited and inconsistent, driving researchers to explore alternative strategies to improve the efficacy of brain stimulation techniques.
- Aurore Thibaut
- & Géraldine Martens
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Comment |
Non-invasive deep brain stimulation: interventional targeting of deep brain areas in neurological disorders
A non-invasive technique using transcranial electrical stimulation offers an improvement in focality over other non-invasive techniques, presenting an opportunity to target deep brain structures for the treatment of neurological disorders.
- Friedhelm C. Hummel
- & Maximilian J. Wessel
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Comment |
Non-invasive stimulation for treating cognitive impairment in Alzheimer disease
The use of non-invasive brain stimulation techniques to treat mild cognitive impairment and dementia in Alzheimer disease is expanding. Trials have produced varying results depending on the differing stimulation techniques, targeted brain regions and degrees of cognitive impairment among the treated cohorts.
- Irena Rektorová
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Review Article |
Multifaceted roles of APOE in Alzheimer disease
Apolipoprotein E (APOE) is the greatest genetic modulator of sporadic Alzheimer disease risk. This Review provides a comprehensive update on our current knowledge of the genetics of APOE and its role in Alzheimer and other neurodegenerative diseases, and summarizes emerging APOE-targeted therapies designed to prevent or slow down Alzheimer disease.
- Rosemary J. Jackson
- , Bradley T. Hyman
- & Alberto Serrano-Pozo
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Review Article |
From spreading depolarization to blood–brain barrier dysfunction: navigating traumatic brain injury for novel diagnosis and therapy
Overall survival rates for traumatic brain injury have improved, but affected individuals often experience persistent and debilitating long-term complications. In this Review, the authors discuss recent evidence for the role of spreading depolarization in the initiation of long-term pathology in traumatic brain injury, including effects on blood–brain barrier dysfunction and neuroinflammation.
- Gerben van Hameren
- , Refat Aboghazleh
- & Alon Friedman
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Consensus Statement |
Acceptable performance of blood biomarker tests of amyloid pathology — recommendations from the Global CEO Initiative on Alzheimer’s Disease
Anti-amyloid treatments for early symptomatic Alzheimer disease have greatly increased the need for biomarker confirmation of amyloid pathology and blood biomarker tests offer an accessible and scalable biomarker test. This Consensus Statement provides recommendations for the minimum acceptable performance of blood biomarker tests for clinical use.
- Suzanne E. Schindler
- , Douglas Galasko
- & Oskar Hansson
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Review Article |
Alzheimer disease blood biomarkers: considerations for population-level use
Blood-based biomarkers have the potential to transform the Alzheimer disease diagnostic pathway, but many questions remain regarding their implementation and utilization. This Review considers factors that might affect the interpretation of blood-based biomarker tests, including comorbidities, sex and race or ethnicity, and discusses broader issues surrounding their use at the population level.
- Michelle M. Mielke
- & Nicole R. Fowler
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Research Highlight |
Microglial senescence is a potential therapeutic target for Alzheimer disease
A new study has identified prematurely senescent microglia in the vicinity of amyloid-β plaques in the brains of individuals with Alzheimer disease.
- Heather Wood
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Review Article |
Clinical implications of brain asymmetries
Functional and structural brain asymmetries are a fundamental principle of brain organization, and research suggests substantial individual variability in these asymmetries that needs to be considered in clinical practice. This Review provides an overview of brain asymmetries, variations in such asymmetries and their relevance in the context of clinical neurology.
- Sebastian Ocklenburg
- , Annakarina Mundorf
- & Guy Vingerhoets
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Review Article |
IDH inhibition in gliomas: from preclinical models to clinical trials
Gliomas are the most common malignant primary brain tumours in adults, and they frequently contain mutations in the isocitrate dehydrogenase 1 (IDH1) or IDH2 gene. Small-molecule inhibitors of mutant IDH are emerging as a new therapeutic strategy for IDH-mutant cancers, and this Review charts their pathway of development for IDH-mutant gliomas.
- Roberta Rudà
- , Craig Horbinski
- & Riccardo Soffietti
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