Hasil untuk "Neurology. Diseases of the nervous system"

K. Nave

B. Masel, D. Dewitt

A. Chaudhuri, P. Behan

E. Farkas, P. Luiten

The aging of the central nervous system and the development of incapacitating neurological diseases like Alzheimer's disease (AD) are generally associated with a wide range of histological and pathophysiological changes eventually leading to a compromised cognitive status. Although the diverse triggers of the neurodegenerative processes and their interactions are still the topic of extensive debate, the possible contribution of cerebrovascular deficiencies has been vigorously promoted in recent years. Various forms of cerebrovascular insufficiency such as reduced blood supply to the brain or disrupted microvascular integrity in cortical regions may occupy an initiating or intermediate position in the chain of events ending with cognitive failure. When, for example, vasoconstriction takes over a dominating role in the cerebral vessels, the perfusion rate of the brain can considerably decrease causing directly or through structural vascular damage a drop in cerebral glucose utilization. Consequently, cerebral metabolism can suffer a setback leading to neuronal damage and a concomitant suboptimal cognitive capacity. The present review focuses on the microvascular aspects of neurodegenerative processes in aging and AD with special attention to cerebral blood flow, neural metabolic changes and the abnormalities in microvascular ultrastructure. In this context, a few of the specific triggers leading to the prominent cerebrovascular pathology, as well as the potential neurological outcome of the compromised cerebral microvascular system are also going to be touched upon to a certain extent, without aiming at total comprehensiveness. Finally, a set of animal models are going to be presented that are frequently used to uncover the functional relationship between cerebrovascular factors and the damage to neural networks.

L. Luo

S. Prusiner, A. Woerman, Daniel A Mordes et al.

Meijuan Zhang, Chengrui An, Yanqin Gao et al.

P. Norat, Sauson Soldozy, J. Sokolowski et al.

Mitochondria are fundamental for metabolic homeostasis in all multicellular eukaryotes. In the nervous system, mitochondria-generated adenosine triphosphate (ATP) is required to establish appropriate electrochemical gradients and reliable synaptic transmission. Notably, several mitochondrial defects have been identified in central nervous system disorders. Membrane leakage and electrolyte imbalances, pro-apoptotic pathway activation, and mitophagy are among the mechanisms implicated in the pathogenesis of neurodegenerative diseases, such as Alzheimer’s, Parkinson’s, and Huntington’s disease, as well as ischemic stroke. In this review, we summarize mitochondrial pathways that contribute to disease progression. Further, we discuss pathological states that damaged mitochondria impose on normal nervous system processes and explore new therapeutic approaches to mitochondrial diseases.

Yu Yamazaki, T. Kanekiyo

Brain capillary endothelial cells form the blood-brain barrier (BBB), which is covered with basement membranes and is also surrounded by pericytes and astrocyte end-feet in the neurovascular unit. The BBB tightly regulates the molecular exchange between the blood flow and brain parenchyma, thereby regulating the homeostasis of the central nervous system (CNS). Thus, dysfunction of the BBB is likely involved in the pathogenesis of several neurological diseases, including Alzheimer’s disease (AD). While amyloid-β (Aβ) deposition and neurofibrillary tangle formation in the brain are central pathological hallmarks in AD, cerebrovascular lesions and BBB alteration have also been shown to frequently coexist. Although further clinical studies should clarify whether BBB disruption is a specific feature of AD pathogenesis, increasing evidence indicates that each component of the neurovascular unit is significantly affected in the presence of AD-related pathologies in animal models and human patients. Conversely, since some portions of Aβ are eliminated along the neurovascular unit and across the BBB, disturbing the pathways may result in exacerbated Aβ accumulation in the brain. Thus, current evidence suggests that BBB dysfunction may causatively and consequently contribute to AD pathogenesis, forming a vicious cycle between brain Aβ accumulation and neurovascular unit impairments during disease progression.

Micol Falabella, H. Vernon, M. Hanna et al.

Over the past decade, it has become clear that lipid homeostasis is central to cellular metabolism. Lipids are particularly abundant in the central nervous system (CNS) where they modulate membrane fluidity, electric signal transduction, and synaptic stabilization. Abnormal lipid profiles reported in Alzheimer’s disease (AD), Parkinson’s disease (PD), amyotrophic lateral sclerosis (ALS), and traumatic brain injury (TBI), are further support for the importance of lipid metablism in the nervous system. Cardiolipin (CL), a mitochondria-exclusive phospholipid, has recently emerged as a focus of neurodegenerative disease research. Aberrant CL content, structure, and localization are linked to impaired neurogenesis and neuronal dysfunction, contributing to aging and the pathogenesis of several neurodegenerative diseases, such as AD and PD. Furthermore, the highly tissue-specific acyl chain composition of CL confers it significant potential as a biomarker to diagnose and monitor the progression in several neurological diseases. CL also represents a potential target for pharmacological strategies aimed at treating neurodegeneration. Given the equipoise that currently exists between CL metabolism, mitochondrial function, and neurological disease, we review the role of CL in nervous system physiology and monogenic and neurodegenerative disease pathophysiology, in addition to its potential application as a biomarker and pharmacological target.

Guo-hong Li, Zhiziong Ning, Yi-Ming Liu et al.

Dengue counts among the most commonly encountered arboviral diseases, representing the fastest spreading tropical illness in the world. It is prevalent in 128 countries, and each year >2.5 billion people are at risk of dengue virus infection worldwide. Neurological signs of dengue infection are increasingly reported. In this review, the main neurological complications of dengue virus infection, such as central nervous system (CNS), peripheral nervous system, and ophthalmic complications were discussed according to clinical features, treatment and possible pathogenesis. In addition, neurological complications in children were assessed due to their atypical clinical features. Finally, dengue infection and Japanese encephalitis were compared for pathogenesis and main clinical manifestations.

F. Al Saiegh, Ritam Ghosh, A. Leibold et al.

Background Emergence of the novel corona virus (severe acute respiratory syndrome (SARS)-CoV-2) in December 2019 has led to the COVID-19 pandemic. The extent of COVID-19 involvement in the central nervous system is not well established, and the presence or the absence of SARS-CoV-2 particles in the cerebrospinal fluid (CSF) is a topic of debate. Case description We present two patients with COVID-19 and concurrent neurological symptoms. Our first patient is a 31-year-old man who had flu-like symptoms due to COVID-19 and later developed an acute-onset severe headache and loss of consciousness and was diagnosed with a Hunt and Hess grade 3 subarachnoid haemorrhage from a ruptured aneurysm. Our second patient is a 62-year-old woman who had an ischaemic stroke with massive haemorrhagic conversion requiring a decompressive hemicraniectomy. Both patients’ CSF was repeatedly negative on real-time PCR analysis despite concurrent neurological disease. Conclusion Our report shows that patients’ CSF may be devoid of viral particles even when they test positive for COVID-19 on a nasal swab. Whether SARS-CoV-2 is present in CSF may depend on the systemic disease severity and the degree of the virus’ nervous tissue tropism and should be examined in future studies.

S. Natoli, V. Oliveira, P. Calabresi et al.

The current coronavirus disease (COVID‐19) outbreak, caused by the novel severe acute respiratory syndrome coronavirus 2 (SARS‐CoV‐2), has raised the possibility of potential neurotropic properties of this virus. Indeed, neurological sequelae of SARS‐CoV‐2 infection have already been reported and highlight the relevance of considering the neurological impact of coronavirus (CoV) from a translational perspective. Animal models of SARS and Middle East respiratory syndrome, caused by structurally similar CoVs during the 2002 and 2012 epidemics, have provided valuable data on nervous system involvement by CoVs and the potential for central nervous system spread of SARS‐CoV‐2. One key finding that may unify these pathogens is that all require angiotensin‐converting enzyme 2 as a cell entry receptor. The CoV spike glycoprotein, by which SARS‐CoV‐2 binds to cell membranes, binds angiotensin‐converting enzyme 2 with a higher affinity compared with SARS‐CoV. The expression of this receptor in neurons and endothelial cells hints that SARS‐CoV‐2 may have higher neuroinvasive potential compared with previous CoVs. However, it remains to be determined how such invasiveness might contribute to respiratory failure or cause direct neurological damage. Both direct and indirect mechanisms may be of relevance. Clinical heterogeneity potentially driven by differential host immune‐mediated responses will require extensive investigation. Development of disease models to anticipate emerging neurological complications and to explore mechanisms of direct or immune‐mediated pathogenicity in the short and medium term is therefore of great importance. In this brief review, we describe the current knowledge from models of previous CoV infections and discuss their potential relevance to COVID‐19.

Zhihong Chen, B. Trapp

J. Sleigh, A. Rossor, Alexander D Fellows et al.

R. Kölliker-Frers, L. Udovin, M. Otero-Losada et al.

The concept of central nervous system (CNS) inflammation has evolved over the last decades. Neuroinflammation is the response of reactive CNS components to altered homeostasis, regardless of the cause to be endogenous or exogenous. Neurological diseases, whether traumatic, neoplastic, ischemic, metabolic, toxic, infectious, autoimmune, developmental, or degenerative, involve direct and indirect immune‐related neuroinflammation. Brain infiltrates of the innate and adaptive immune system cells appear in response to an infective or otherwise noxious agent and produce inflammatory mediators. Mediators of inflammation include local and recruited cells and signals. Processes derived from extrinsic and intrinsic CNS diseases also elicit the CNS inflammatory response. A deeper understanding of immune‐related inflammation in health and disease is necessary to find potential therapeutic targets for preventing or reducing CNS damage. This review is aimed at discussing the innate and adaptive immune system functions and their roles in regulating brain cell responses in disease and homeostasis maintenance.

Shi Liu, Shi Liu, Zixin Huang et al.

BackgroundIn recent years, although numerous meta-analyses/systematic reviews (MAs/SRs) have explored the therapeutic effect of noninvasive brain stimulation (NIBS) on poststroke depression, the reliability and quality of its clinical evidence remain uncertain. Therefore, this study aims to conduct an overview of systematic reviews to evaluate the effectiveness and safety of NIBS for PSD, thereby providing evidence-based support for clinical decision-making.MethodsA comprehensive search of multiple databases, including PubMed, EMBASE, the Cochrane Library, Web of Science, CNKI, VIP, Wan Fang, and CBM, was conducted to identify systematic reviews and meta-analyses of NIBS for PSD. Following the literature screen and data extraction, the quality of the included studies was assessed using the PRISMA statement for reporting quality, the AMSTAR-2 tool for methodological quality, and the GRADE system for evidence quality. We extracted the main outcome indicators of depressive symptoms and the secondary outcome indicators of neurological function, cognitive function, daily living ability, anxiety symptoms, clinical efficiency and adverse reactions for analysis.ResultsA total of 20 MAs/SRs were included. According to the PRISMA statement, 6 (30%) reports were relatively complete, 13 (65%) had problems, and 1 (5%) had serious information deficiencies. The results of the AMSTAR-2 evaluation revealed that 3 articles (15%) were of moderate quality, 5 (25%) were of low quality, and 12 (60%) were of critically low quality. Thus, the overall quality was not high. The GRADE evaluation revealed that a total of 66 evidence bodies were included: 9 (13.6%) had moderate evidence, 15 (22.8%) had low-level evidence, and 42 (63.6%) had very low-level evidence, and no high-quality outcome indicator was identified.ConclusionThe included studies revealed that NIBS is an effective and safe treatment for PSD. However, because the methodology and results of the MAs/SRs were generally not of high quality, the reliability of the conclusions is limited to a certain extent. Future research should focus on conducting more high-quality, large-sample, multicenter follow-up studies to further verify the value of applying NIBS in PSD treatment.Systematic review registrationhttps://www.crd.york.ac.uk/PROSPERO/view/CRD42025633044, identifier CRD42025633044.

M. Camilleri

The extrinsic and autonomic nervous system intricately controls the major functions of the gastrointestinal tract through the enteric nervous system; these include motor, secretory, sensory, storage, and excretory functions. Disorders of the nervous system affecting gastrointestinal tract function manifest primarily as abnormalities in motor (rather than secretory) functions. Common gastrointestinal symptoms in neurologic disorders include sialorrhea, dysphagia, gastroparesis, intestinal pseudo-obstruction, constipation, diarrhea, and fecal incontinence. Diseases of the entire neural axis ranging from the cerebral hemispheres to the peripheral autonomic nerves can result in gastrointestinal motility disorders. The most common neurologic diseases affecting gastrointestinal function are stroke, parkinsonism, multiple sclerosis, and diabetic neuropathy. Diagnosis involves identification of the neurologic disease and its distribution, and documentation of segmental gut dysfunction, typically using noninvasive imaging, transit measurements, or intraluminal measurements of pressure activity and coordination of motility. Apart from treatment of the underlying neurologic disease, management focuses on restoration of normal hydration and nutrition and pharmacologic treatment of the gut neuromuscular disorder.

Leenor Alfahel, Aleksandar Rajkovic, Adrian Israelson

Nele Hellweg, Heide Glaesmer, Barbara Stelzl-Marx et al.

Background: During the post-World War II occupation of Austria, approximately 20,000–30,000 ‘children born of war’ (CBOW), also called occupation children were born through intimate contacts between Austrian women and occupation soldiers. Research on other CBOW populations indicates that CBOW mostly grow up under difficult conditions, sometimes with strong long-term mental health consequences.Objective: To examine whether comparable psychosocial consequences can be found in Austrian occupation children (AOC), a first quantitative study was carried out.Method: Child maltreatment, post-traumatic stress disorder, depression and somatization, and general life satisfaction were assessed in a sample of 98 AOC using self-report instruments. Results were compared to a sample of German occupation children (GOC; N = 146).Results: High prevalence of above threshold full (10.2%) and partial (14.3%) PTSD, somatic (16.3%) and depressive (11.1%) symptomatology were found in AOC. They were at high risk of child maltreatment (e.g. emotional abuse: 53.6%), which was associated with current symptomatology. Notably, AOC tended to report high levels of general life satisfaction. No differences were found between GOC and AOC.Conclusions: Findings highlight the complex and long-term effects of developmental conditions and childhood maltreatment on mental health of CBOW, even decades later. Findings of high life satisfaction provide evidence of resilience and maturation processes across the lifespan.