Current Neurology and Neuroscience Reports最新文献

Purpose of review: Optimal initial management can have a significant impact in long-term outcome in primary CNS lymphoma. This article reviews recent advances and the state of the field.

Recent findings: Genomic analysis of CSF cell-free DNA has emerged as a new diagnostic tool for PCNSL. Treatment options have likewise evolved, with mature data from first-line chemotherapy-based prospective trials disclosing excellent results in younger (< 60-65) patients, with a cure achieved in a majority. However, results in older patients remain dismal, with several new salvage options under investigation including BTK pathway-targeted therapies, and CAR-T cell treatments. Meanwhile, low-dose radiation has emerged as an additional alternative for consolidation therapy. For younger PCNSL patients, the goal of treatment is now a cure, with the next frontier being the development of therapies affording optimized neurocognitive outcome and lower toxicity. Treatment for older patients remains however an unmet need, with several promising clinical trials ongoing.

Purpose of review: This review seeks to examine the prevalence, pathophysiology, diagnostic challenges, and treatment strategies for movement disorders in patients with systemic lupus erythematosus (SLE).

Recent findings: In recent years, the spectrum and number of autoimmune movement disorders has rapidly expanded with the identification of neuronal and paraneoplastic antibodies which should be considered in the differential for patients with acute to subacute development of a movement disorder. The identification of SLE in a patient with a new onset movement disorder may lead to earlier treatment with immune therapies especially if other systemic manifestations are present. Current treatment for SLE-associated movement disorders involves co-management with rheumatology and is based on expert clinical opinion on symptomatic management. Further understanding of the contributing pathophysiology may lead to advancements in therapeutic approaches.

Purpose of review: Stroke incidence and outcomes are disproportionately unfavorable among Indigenous populations in Western colonized countries. These inequities are often attributed to poor health literacy. This paper summarizes recent evidence on the topic of Indigenous health literacy, describes current gaps, and proposes priorities for future work/research.

Recent findings: Traditionally, much focus has been placed on improving health literacy for Indigenous Peoples as a key intervention to address Indigenous stroke and other health disparities. Recent literature, however, challenges this approach as it risks stigmatization and marginalization and portrays the deficiencies as sitting with Indigenous people. Increasingly, an emphasis is placed on the need for health literacy approaches to be culturally responsive for the populations of interest, for institutions to provide high quality culturally relevant stroke care, and for providers to upskill in cultural safety to better meet the needs of Indigenous patient populations. Very little evidence exists to indicate that stroke care providers are meeting these needs. To close the health gap and improve stroke care for Indigenous Peoples, the focus needs to shift from promoting health literacy among Indigenous Peoples to providing high quality culturally relevant health care. More research into this topic and monitoring of progress over time is needed.

Purpose of review: This review examines the role of different viral infections in epileptogenesis, with a focus on Herpesviruses such as Human Herpesvirus 6 (HHV-6) and Epstein Barr Virus (EBV), Flaviviruses, Picornaviruses, Human Immunodeficiency Virus (HIV), Influenzavirus and Severe Acute Respiratory Syndrome CoronaVirus 2 (SARS-CoV-2).

Recent findings: A growing literature on animal models, such as the paradigmatic Theiler's murine encephalomyelitis virus (TMEV) model, and clinical investigations in patients with epilepsy have started to elucidate cellular mechanisms implicated in seizure initiation and development of epilepsy following viral infections. A central role of neuroinflammation has emerged, with evidence of activation of the innate and adaptive immunity, dysregulation of microglial and astrocytic activity and production of multiple cytokines and other inflammatory mediators. Several chronic downstream effects result in increased blood-brain barrier permeability, direct neuronal damage, and modifications of ion channels ultimately leading to altered neuronal excitability and seizure generation. Key findings underscore the complex interplay between initial viral infection, neuroinflammation, and later development of epilepsy. Further research is needed to elucidate these mechanisms and develop targeted interventions.

Purpose of review: Apraxia typically involves impairments in gesture production and tool use, affecting daily life activities. This article reviews current conceptualizations and developments in diagnostic and therapy.

Recent findings: Apraxia has been studied in various neurological conditions, particularly stroke and dementia, but recent studies show gesturing deficits in psychiatric populations as well. Promising results have emerged from integrative treatment approaches involving intensive practice of gestures or daily activities. However, several reviews have noted the only marginal progress in apraxia therapy research despite new technologies, like virtual reality and brain stimulation, offering fresh opportunities for assessment and therapy. Advances in lesion-symptom mapping and connectivity analyses led to more detailed neuroanatomical models emphasizing parallel and gradual processing. These models facilitate the understanding of underlying mechanisms of motor cognitive performance and its decline. Finally, the digital era prompts the need to study digital tool use in apraxia, with initial efforts underway.

Purpose of review: Misdiagnosis of multiple sclerosis (MS) is a prevalent worldwide problem. This review discusses how MS misdiagnosis has evolved over time and focuses on contemporary challenges and potential strategies for its prevention.

Recent findings: Recent studies report cohorts with a range of misdiagnosis between 5 and 18%. Common disorders are frequently misdiagnosed as MS. Overreliance on MRI findings and misapplication of MS diagnostic criteria are often associated with misdiagnosis. Emerging imaging biomarkers, including the central vein sign and paramagnetic rim lesions, may aid diagnostic accuracy when evaluating patients for suspected MS. MS misdiagnosis can have harmful consequences for patients and healthcare systems. Further research is needed to better understand its causes. Concerted and novel educational efforts to ensure accurate and widespread implementation of MS diagnostic criteria remain an unmet need. The incorporation of diagnostic biomarkers highly specific for MS in the future may prevent misdiagnosis.

Purpose of review: Sickle cell anemia (SCA) is an autosomal recessive inherited hemoglobinopathy that results in a high risk of stroke. SCA primarily affects an underserved minority population of children who are frequently not receiving effective, multi-disciplinary, preventative care. This article reviews primary and secondary stroke prevention and treatment for children with SCA for the general adult and pediatric neurologist, who may play an important role in providing critical neurologic evaluation and care to these children.

Recent findings: Primary stroke prevention is efficacious at reducing ischemic stroke risk, but it is not consistently implemented into clinical practice in the United States, resulting in these children remaining at high risk. Acute symptomatic stroke management requires neurology involvement and emergent transfusion to limit ischemia. Furthermore, while chronic transfusion therapy is a proven secondary preventative modality for those with prior symptomatic or silent cerebral infarcts, it carries significant burden. Newer therapies (e.g., stem cell therapies and voxelotor) deserve further study as they may hold promise in reducing stroke risk and treatment burden. Effective primary and secondary stroke prevention and treatment remain a challenge. Informing and engaging neurology providers to recognize and provide critical neurologic evaluation and treatment has potential to close care gaps.

Purpose of review: We aim to critically review animal and human studies of the glymphatic system in migraine and propose a model for how the glymphatic system may function in migraine, based on the available evidence.

Recent findings: Early studies in animal models report migraine attacks temporarily disrupt glymphatic flow. Human imaging studies suggest chronic migraine may be associated with alterations in glymphatic system function, albeit with conflicting results. Presently, it remains unknown whether repetitive migraine attacks or frequent nights of insomnia impair glymphatic system function over time in those with migraine, and whether alterations in glymphatic function could contribute to worsening migraine disability or risk for cognitive disease. Longitudinal studies of glymphatic function in patients with migraine and insomnia, with inclusion of cognitive assessments, may be informative.

Purpose of review: Recent stroke treatment advances have necessitated agile, broad-scale healthcare system redesign, to achieve optimal patient outcomes and access equity. Optimised hyperacute stroke care requires integrated pre-hospital, emergency department, stroke specialist, radiology, neurosurgical and endovascular neurointervention services, guided by a population-wide needs analysis. In this review, we survey system integration efforts, providing case studies, and identify common elements of successful initiatives.

Recent findings: Different regions and nations have evolved varied acute stroke systems depending on geography, population density and workforce. However, common facilitators to these solutions have included stroke unit care as a foundation, government-clinician synergy, pre-hospital pathway coordination, service centralisation, and stroke data guiding system improvement. Further technological advantages will minimize the geographical distance disadvantages and facilitate virtual expertise redistribution to remote areas. Continued treatment advances necessitate an integrated, adaptable, population-wide trans-disciplinary approach. A well-designed clinician-led and government-supported system can facilitate hyperacute care and scaffold future system enhancements.

Purpose of review: Pediatric low-grade gliomas (pLGGs) often result in significant long-term morbidities despite high overall survival rates. This review aims to consolidate the current understanding of pLGG biology and molecular features and provide an overview of current and emerging treatment strategies.

Recent findings: Surgical resection remains a primary treatment modality, supplemented by chemotherapy and radiotherapy in specific cases. However, recent advances have elucidated the molecular underpinnings of pLGGs, revealing key genetic abnormalities such as BRAF fusions and mutations and the involvement of the RAS/MAPK and mTOR pathways. Novel targeted therapies, including MEK, BRAF and pan-RAF inhibitors, have shown promise in clinical trials, demonstrating significant efficacy and manageable toxicity. Understanding of pLGGs has significantly improved, leading to more personalized treatment approaches. Targeted therapies have emerged as effective alternatives, potentially reducing long-term toxicities. Future research should focus on optimizing therapy sequences, understanding long-term impacts, and ensuring global accessibility to advanced treatments.