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《Neurologic Clinics》2019,37(2):335-344
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We report the case of a 59‐year‐old woman who presented with several episodes of transient ischemic attack (TIA) caused by pathologically confirmed giant cell arteritis. She continued suffering from TIAs during admission despite immunosuppressant and antithrombotic therapy. Sudden neurological deterioration with paraparesis and cognitive impairment developed. A brain magnetic resonance (MR) imaging showed bilateral watershed ischemic lesions. MR angiography demonstrated severe stenosis of both intracranial internal carotid arteries (ICAs). Angioplasty and stenting on the left ICA were performed, with evident clinical improvement occurring within 24 hours. Endovascular therapy may be an alternative option to treat severe GCA with symptomatic intracranial large vessel disease not responsive to intensive conventional medical treatment.  相似文献   

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Amyotrophic lateral sclerosis is a progressive neurodegenerative disease of the motor neurons without a known cure. Based on the possibility of cellular neuroprotection and early preclinical results, stem cells have gained widespread enthusiasm as a potential treatment strategy. Preclinical models demonstrate a protective role of engrafted stem cells and provided the basis for human trials carried out using various types of stem cells, as well as a range of cell delivery methods. To date, no trial has demonstrated a clear therapeutic benefit; however, results remain encouraging and are the basis for ongoing studies. In addition, stem cell technology continues to improve, and induced pluripotent stem cells may offer additional therapeutic options in the future. Improved disease models and clinical trials will be essential in order to validate stem cells as a beneficial therapy.

Electronic supplementary material

The online version of this article (doi:10.1007/s13311-015-0339-9) contains supplementary material, which is available to authorized users.Key words: Amyotrophic lateral sclerosis, Stem cell therapy, Cell transplantation, Neural progenitor cell, Mesenchymal stem cell, Granulocyte-colony stimulating factor, Clinical trials  相似文献   

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Stem cell transplantation holds a promising future for central nervous system repair. Current challenges, however, include spatially and temporally defined cell differentiation and maturation, plus the integration of transplanted neural cells into host circuits. Here we discuss the potential advantages of neuromodulation-based stem cell therapy, which can improve the viability and proliferation of stem cells, guide migration to the repair site, orchestrate the differentiation process, and promote the integration of neural circuitry for functional rehabilitation. All these advantages of neuromodulation make it one potentially valuable tool for further improving the efficiency of stem cell transplantation.  相似文献   

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Purpose of Review

Tauopathies represent a spectrum of incurable and progressive age-associated neurodegenerative diseases that currently are diagnosed definitively only at autopsy. Few clinical diagnoses, such as classic Richardson’s syndrome of progressive supranuclear palsy, are specific for underlying tauopathy and no clinical syndrome is fully sensitive to reliably identify all forms of clinically manifest tauopathy. Thus, a major unmet need for the development and implementation of tau-targeted therapies is precise antemortem diagnosis. This article reviews new and emerging diagnostic therapies for tauopathies including novel imaging techniques and biomarkers and also reviews recent tau therapeutics.

Recent Findings

Building evidence from animal and cell models suggests that prion-like misfolding and propagation of pathogenic tau proteins between brain cells are central to the neurodegenerative process. These rapidly growing developments build rationale and motivation for the development of therapeutics targeting this mechanism through altering phosphorylation and other post-translational modifications of the tau protein, blocking aggregation and spread using small molecular compounds or immunotherapy and reducing or silencing expression of the MAPT tau gene.

Summary

New clinical criteria, CSF, MRI, and PET biomarkers will aid in identifying tauopathies earlier and more accurately which will aid in selection for new clinical trials which focus on a variety of agents including immunotherapy and gene silencing.
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Purpose of Review

This article strives to review and summarize selected recent literature and topics contributing to a greater understanding of the diagnosis and treatments of neonatal seizures that have emerged in the past several years.

Recent Findings

Continuous EEG is recommended as the gold standard for neonatal seizure monitoring as it can provide additional information that may stratify patients by etiology, as well as identify at-risk groups of newborns for neuromonitoring. Investigations are moving beyond traditional antiepileptic agents in search of treatments with better efficacy and with less concern for developmental effects. Targeted therapies for seizures resulting from particular genetic conditions are increasing, highlighting the importance of early genetic diagnosis. Better understanding of the risk of post-neonatal epilepsy based on etiology is emerging with new epidemiological studies.

Summary

Evidence is growing for deleterious effects of seizures on outcomes, elevating the importance of seizure detection and effective treatment. Advances in utilization of continuous EEG monitoring have improved the accuracy of seizure detection and have identified at-risk groups of newborns for neuromonitoring. Ultimately, the goal in management of neonatal seizures is not only clinical stabilization in the acute period but also to influence neurodevelopmental outcome and modify the risk of future epilepsy.
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Over the last decade, RNA interference technology has shown therapeutic promise in rodent models of dominantly inherited brain diseases, including those caused by polyglutamine repeat expansions in the coding region of the affected gene. For some of these diseases, proof-of concept studies in model organisms have transitioned to safety testing in larger animal models, such as the nonhuman primate. Here, we review recent progress on RNA interference-based therapies in various model systems. We also highlight outstanding questions or concerns that have emerged as a result of an improved (and ever advancing) understanding of the technologies employed.  相似文献   

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Human neuroimaging has had a major impact on the biological understanding of epilepsy and the relationship between pathophysiology, seizure management, and outcomes. This review highlights notable recent advancements in hardware, sequences, methods, analyses, and applications of human neuroimaging techniques utilized to assess epilepsy. These structural, functional, and metabolic assessments include magnetic resonance imaging (MRI), positron emission tomography (PET), and magnetoencephalography (MEG). Advancements that highlight non-invasive neuroimaging techniques used to study the whole brain are emphasized due to the advantages these provide in clinical and research applications. Thus, topics range across presurgical evaluations, understanding of epilepsy as a network disorder, and the interactions between epilepsy and comorbidities. New techniques and approaches are discussed which are expected to emerge into the mainstream within the next decade and impact our understanding of epilepsies. Further, an increasing breadth of investigations includes the interplay between epilepsy, mental health comorbidities, and aberrant brain networks. In the final section of this review, we focus on neuroimaging studies that assess bidirectional relationships between mental health comorbidities and epilepsy as a model for better understanding of the commonalities between both conditions.Supplementary InformationThe online version contains supplementary material available at 10.1007/s13311-021-01049-y.Key Words: Neuroimaging, Epilepsy, Neurosurgery, Network disorder, Mental health  相似文献   

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