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1.
BMC Neurol ; 23(1): 427, 2023 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-38041003

RESUMO

PURPOSE: Interictal epileptiform discharges (IEDs) captured in electroencephalography (EEG) have a high diagnostic value for epileptic patients. Extending the recording time may increase the possibility of obtaining IEDs. The purpose of our research was to determine how long it took for various epileptic individuals to receive their first IEDs. METHODS: We retrospectively analyzed patients who were diagnosed with epilepsy and had no anti-seizure medications (ASMs) between September 2018 and March 2019 in the neurology department of the First Affiliated Hospital of Xi'an Jiaotong University. Each individual underwent a 24-h long-term video electroencephalographic monitoring (VEM) procedure. Clinical information including age, gender, age of seizure onset, frequency of seizures, the interval between last seizure and VEM, and results of neuroimaging were gathered. We also calculated the times from the start of the VEM to the first definite IEDs. RESULTS: A total of 241 patients were examined, including 191 with focal-onset epilepsy and 50 with generalized epilepsy. In individuals with focal-onset epilepsy, the median latency to the first IED was 63.0 min (IQR 19.0-299.0 min), as compared to 30.0 min (IQR 12.5-62.0 min) in patients with generalized epilepsy (p < 0.001). The latency to the first IED is significantly related to the age of seizure onset (HR = 0.988, p = 0.049), the interval between last seizure and VEM (HR = 0.998, p = 0.013). But it is not correlated with seizure frequency, gender and age. CONCLUSIONS: IEDs were discovered during 24-h EEG monitoring in 222/241(92.1%) of the epilepsy patients that were included. Compared to focal-onset epilepsy, generalized epilepsy demonstrated a much shorter latency to IED. Patients with late-onset epilepsy or those without recent episodes may require longer EEG monitoring periods.


Assuntos
Epilepsias Parciais , Epilepsia Generalizada , Epilepsia , Humanos , Estudos Retrospectivos , Epilepsia/diagnóstico , Epilepsia/tratamento farmacológico , Eletroencefalografia/métodos , Convulsões
2.
Clin Gerontol ; 46(5): 808-818, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-35603686

RESUMO

OBJECTIVES: This study examines the extent to which depressive symptoms mediate the link between physical activity and cognitive function among older adults in China. METHODS: This study utilizes the 2013-18 China Health and Retirement Longitudinal Study (CHARLS) dataset, of which 3,658 subjects over the age of 50 satisfied inclusion criteria. Degree of physical activity, prevalence of depressive symptoms, and performance in cognitive function are measured by the International Physical Activity Questionnaire (IPAQ), Center for Epidemiological Studies Depression Scale (CESD), the Mini-Mental State Examination (MMSE) instruments. A structural mediation model was built to assess the degree to which depressive symptoms act as mediator between physical activity and cognitive function. RESULTS: Physical activity is positively and significantly associated with cognitive function (std ß = 0.034, p-value = .007), while physical activity is negatively and significantly associated with prevalence of depressive symptoms (std ß = -0.088, p-value < .001). Results indicate that depressive symptoms partially and significantly mediate the relationship between physical activity and cognitive function (std ß = 0.003, p-value = .035). Total influence of physical activity on cognitive performance is evaluated to be 0.037 standard deviations (p-value = .035). CONCLUSIONS: Findings uncover an underexamined mental well-being channel through which physical activity can positively influence late adulthood cognition. CLINICAL IMPLICATIONS: In recommending behavioral modifications to reduce risks of late adulthood cognitive decline, encouraging physical activity for older individuals is key, since it is both directly associated with better cognitive performance, as well as indirectly through lowering prevalence of depressive symptoms.

3.
Front Aging Neurosci ; 14: 970711, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36466599

RESUMO

A plethora of environmental risk factors has been persistently implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS), including metal/metalloids. This study aimed to examine potential associations between cerebral spinal fluid (CSF) metal/metalloids and ALS risks. CSF concentrations of copper (Cu), nickel (Ni), mercury (Hg), arsenic (As), manganese (Mn), and iron (Fe) in ALS (spinal- and bulbar-onset) patients and controls were measured using inductively coupled plasma mass spectrometry (ICP-MS). Results from this study revealed marked differences between control, spinal-onset, and bulbar-onset groups. We report that Cu levels were lower in the ALS and spinal-onset groups compared to the control group. Ni level were higher in the spinal-onset group compared to the control and bulbar-onset groups. In addition, associations between CSF metal/metalloid levels with disease severity, sex, and serum triglycerides were also examined to broach the potential relevance of neurotoxic metal/metalloids in ALS disease heterogeneity.

4.
Neurol Res Int ; 2022: 3265029, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36340639

RESUMO

Multiple sclerosis (MS) is a chronic debilitating immune-mediated disease of the central nervous system, which causes demyelination and neuroaxonal damage. Low-grade systemic inflammation has been considered to lead to pathogenesis owing to the amplification of pathogenic immune response activation. However, there is a shortage of reliable systemic inflammatory biomarkers to predict the disease activity and progression of MS. In MS patients, a series of cytokines and chemokines promote the proliferation of neutrophils and lymphocytes and their transfer to the central nervous system. The neutrophil-to-lymphocyte ratio (NLR), which combines the information of the inherent and adaptive parts of the immune system, represents a reliable measure of the inflammatory burden. In this review, we aimed to discuss the inflammatory response in MS, mainly the function of lymphocytes and neutrophils, which can be implemented in the utility of NLR as a diagnostic tool in MS patients. The underlying pathophysiology is highlighted to identify new potential targets for neuroprotection and to develop novel therapeutic strategies.

5.
Front Aging Neurosci ; 14: 977321, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36158538

RESUMO

Background: Engagement in physically active lifestyles brings multidimensional health benefits including better cognitive function. While prior studies examined the link between physical activity and cognitive function, a remaining unanswered question is what modifiable factors channel such effects. Objective: This study investigates the extent to which subject's body mass index (BMI) and depression mediate the link between physical activity and cognitive function among older adults in China. Methods: This study builds a parallel structural equation model utilizing the 2013-2018 China Health and Retirement Longitudinal Study (CHARLS) dataset. We screened a total of 14,724 subjects, among which 3,611 subjects met the inclusion criteria. Physical activity, depression, and cognitive function are measured using the International Physical Activity Questionnaire (IPAQ), Center for Epidemiological Research Depression Scale (CES-D), and Mini-Mental State Examination (MMSE) instruments. Results: Parallel mediation analyses indicate that depression significantly mediates the link between physical activity and cognitive function (std. ß = 0.023, p-value = 0.010), while no significant mediation was observed via BMI (std. ß = 0.005, p-value = 0.155). Findings also show that physical activity is positively associated with cognitive function (std. ß = 0.104, p-value = 0.004), whereas physical activity is inversely associated with BMI (std. ß = -0.072, p-value = 0.045). Both BMI (std. ß = -0.071, p-value = 0.042) and depression (std. ß = -0.199, p-value = 0.001) are negatively associated with cognitive function. Conclusion: This study quantifies the positive association between physical activity and cognitive function in older Chinese adults, and uncovers a significant mediation channel occurring through depression. From a clinical perspective, physical behavioral modifications can lead to linked improvements in both mental and cognitive wellbeing for older adults.

6.
J Peripher Nerv Syst ; 27(3): 189-196, 2022 09.
Artigo em Inglês | MEDLINE | ID: mdl-35611446

RESUMO

The early clinical features of nitrous oxide (N2 O)-induced neuropathy were mimicking that of Guillain-Barré syndrome (GBS). We aimed to explore clinical and laboratory characteristics of N2 O-induced neuropathy in comparison with GBS. We retrospectively reviewed data of 15 patients with N2 O-induced neuropathy and compared them with 15 GBS patients. The age of the N2 O-induced neuropathy group was significantly younger than that in the GBS group (22 ± 5 vs 45 ± 17). Paresthesia was more common in N2 O-induced neuropathy group (100% vs 53.3%). The proportion of distal upper limbs weakness was lower than that in GBS group (20.0% vs 93.3%). There was no significant difference in the distal weakness of the lower limbs (100% vs 80.0%). The incidence of motor conduction block and compound muscle action potential amplitude reduction in upper limbs was lower than that in GBS group (6.7% vs 60.0%; 26.7% vs 80.0%). The sensory nerve action potential amplitude drop in the lower limbs was more severe than that in GBS group (53.3% vs 0). The increase of Mean corpuscular volume (MCV) was more pronounced compared to GBS group (96.97 ± 6.00 vs 88.55 ± 5.41). High homocysteine levels were more common in N2 O-related group [29.80(11.60, 70.50) vs 14.35(9.22, 19.30)]. Typical clinical features of the acute N2 O neuropathy appears to be a myeloneuropathy, affecting the lower limbs more than the upper limbs, mixed axonal-demyelinating electrophysiological performance, higher homocysteine level, and larger MCV and common posterior spinal cord involvement in cervical segment.


Assuntos
Síndrome de Guillain-Barré , Doenças do Sistema Nervoso Periférico , Síndrome de Guillain-Barré/induzido quimicamente , Síndrome de Guillain-Barré/diagnóstico , Homocisteína , Humanos , Condução Nervosa/fisiologia , Óxido Nitroso/efeitos adversos , Estudos Retrospectivos
7.
CNS Neurosci Ther ; 28(7): 1072-1080, 2022 07.
Artigo em Inglês | MEDLINE | ID: mdl-35429132

RESUMO

AIMS: This multicenter, open-label, randomized study (Registration No. ChiCTR-OCH-14004528) aimed to compare the efficacy and effects of oxcarbazepine (OXC) with levetiracetam (LEV) as monotherapies on patient quality of life and mental health for patients with newly diagnosed focal epilepsy from China. METHODS: Patients with newly diagnosed focal epilepsy who had experienced 2 or more unprovoked seizures at greater than a 24-h interval during the previous year were recruited. Participants were randomly assigned to the OXC group or LEV group. Efficacy, safety, quality of life, and mental health were evaluated over 12-week and 24-week periods. RESULTS: In total, we recruited 271 newly diagnosed patients from 23 centers. Forty-four patients were excluded before treatment for reasons. The rate of seizure freedom of OXC was significantly superior to that of LEV at 12 weeks and 24 weeks (p < 0.05). The quality of life (except for the seizure worry subsection) and anxiety scale scores also showed significant differences from before to after treatment in the OXC and LEV groups. CONCLUSIONS: OXC monotherapy may be more effective than LEV monotherapy in patients with newly diagnosed focal epilepsy. Both OXC and LEV could improve the quality of life and anxiety state in adult patients with focal epilepsy.


Assuntos
Epilepsias Parciais , Qualidade de Vida , Adulto , Anticonvulsivantes/uso terapêutico , Epilepsias Parciais/tratamento farmacológico , Humanos , Levetiracetam/uso terapêutico , Oxcarbazepina/uso terapêutico , Convulsões/tratamento farmacológico , Resultado do Tratamento
8.
Neurotoxicology ; 89: 12-19, 2022 03.
Artigo em Inglês | MEDLINE | ID: mdl-35007622

RESUMO

Amyotrophic lateral sclerosis is an unremitting neurodegenerative (ND) disease characterized by progressive and fatal loss of motor neuron function. While underlying mechanisms for ALS susceptibility are complex, current understanding suggests that interactions between age, genetic, and environmental factors may be the key. Environmental exposure to metal/metalloids has been implicated in various ND diseases including ALS, Alzheimer's Disease (AD), and Parkinson's Disease (PD). However, most of currently available population-based ALS studies in relation to metal exposure are based on individuals from European ancestry, while East Asian populations, especially cohorts from China, are less well-characterized. This study aims to examine the association between metal/metalloid levels and ALS onset by evaluating blood cadmium (Cd), lead (Pb), Cu, Zn, calcium (Ca), magnesium (Mg), and iron (Fe) levels in controls and sporadic ALS patients from North Western China. We report that Cu and Fe levels are found at higher levels in ALS patients compared to the controls. Spinal and bulbar onset patients show significant difference in Ca levels. Moreover, Cd, Pb, Cu, and Ca levels are positively correlated with high disease severity. Results from this study may provide new insights for understanding not only the role of metal/metalloids in ALS susceptibility, but also progression and forms of onset.


Assuntos
Esclerose Lateral Amiotrófica , Metaloides , Doença de Parkinson , Esclerose Lateral Amiotrófica/diagnóstico , Esclerose Lateral Amiotrófica/genética , Humanos , Metais , Índice de Gravidade de Doença
9.
BMC Ophthalmol ; 21(1): 370, 2021 Oct 19.
Artigo em Inglês | MEDLINE | ID: mdl-34663261

RESUMO

BACKGROUND: Despite effectiveness in delaying the spread of the pandemic, frequent and extended disruption to children's livelihoods have fomented new norms in which learning routines encounter immense change. In particular, increased sedentary e-learning engagement with electronic screens and exposure to stressful circumstances are likely to pose adverse risks for children's vision development. METHODS: This present study examines the link between near-sighted refractive error, and sedentary exposure to electronic screens, psychosocial stress level, and outdoor activities. A Rapid Survey Methodology (RSM) design was utilized to collect information on subject's vision condition, sedentary electronic screen use, and level of psychosocial stress, in addition to detailed socio-demographic background characteristics. RESULTS: This study involves 2234 subjects enrolled in 1st to 6th grade in primary schools. Every 1 diopter hour increase in electronic screen use per day is associated with 1.036 OR (95% CI =1.024-1.047, p-value< 0.050), while every 1 h • W m- 2 sr- 1 of illuminance-weighted electronic screen use per day is associated with 2.285 OR (95% CI =1.829-2.855, p-value< 0.050) increased likelihood of near-sighted refractive error. Higher level of psychosocial stress is associated with 2.441 OR (95% CI =1.870-3.187, p-value< 0.050) and 2.403 OR (95% CI =1.839-3.141, p-value< 0.050) increased likelihood of near-sighted refractive error. Frequency of outdoor activity is not significantly associated with increased likelihood of near-sighted refractive error (p-value> 0.050). CONCLUSIONS: Findings in this study show that many factors, including grade level and prior vision condition, contribute to increased risks of near-sighted refractive error during the COVID-19 pandemic. More strikingly, pandemic-related behavioral modifications such as lengthy sedentary electronic screen use and elevated levels of psychosocial stress are two critical channels affecting children's eye health.


Assuntos
COVID-19 , Erros de Refração , Criança , China/epidemiologia , Humanos , Pandemias , Prevalência , Erros de Refração/epidemiologia , SARS-CoV-2 , Inquéritos e Questionários
10.
Sci Rep ; 11(1): 20786, 2021 10 21.
Artigo em Inglês | MEDLINE | ID: mdl-34675267

RESUMO

To identify differential metabolites and metabolic pathways and provide guidance for the novel biomarkers for diagnosis and prognosis of amyotrophic lateral sclerosis (ALS). ALS patients and people without nervous diseases were recruited. Metabolomic analysis was performed using gas chromatography-mass spectrometry (GC/MS). The orthogonal projections to latent structures discriminant analysis (OPLS-DA) were used to identify differential metabolites. Kyoto Encyclopedia of Genes and Genomes and MetaboAnalyst were used to identify metabolic pathways. 75 metabolites were detected and aligned. The OPLS-DA showed the metabolomic profile of ALS patients and those in the fast-progression and slow-progression ALS groups differed from that of CTRL (p < 0.05). The levels of maltose, glyceric acid, lactic acid, beta-alanine, phosphoric acid, glutamic acid, ethanolamine and glycine in ALS were significantly higher, while 2,4,6-tri-tert-butylbenzenethiol was lower. Glycine, serine and threonine metabolism, D-glutamine and D-glutamate metabolism, alanine, aspartate, and glutamate metabolism, beta-alanine metabolism, and pyruvate metabolism were significantly altered metabolic pathways in ALS. ROC was used to discriminate ALS from CTRL with an AUC of 0.898 (p < 0.001) using 2,4,6-tri-tert-butylbenzenethiol, beta-alanine, glycine, and ethanolamine. The serum metabolites and metabolic pathways in ALS patients are significantly altered compared with CTRL. These findings may contribute to the early diagnosis of ALS.


Assuntos
Esclerose Lateral Amiotrófica/sangue , Cromatografia Gasosa-Espectrometria de Massas/métodos , Metabolômica/métodos , Biomarcadores/sangue , Estudos de Casos e Controles , Humanos
11.
J Glob Health ; 11: 05020, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34484707

RESUMO

BACKGROUND: Around the globe, various self-quarantine, social distancing, and school-closure policies were implemented during the coronavirus disease-19 (COVID-19) outbreak to reduce disease transmission. Many economies/territories were compelled to consider digital learning modalities. In particular, increased digital learning engagement with digital devices and mounting psychosocial stress due to social isolation are likely to pose adverse risks for youth visual health globally. This study examines the association between increased digital device use, psychosocial stress, and myopia symptoms among Chinese youth during the COVID-19 pandemic. METHODS: This is a retrospective observational population study consisting of 3918 participants enrolled in primary, secondary, and university in China. Participants are recruited through an online survey, which included self-reported information on daily digital device use, psychosocial stress level, condition of visual acuity, and demographic information. We utilize statistical tools including χ2 test, paired sample t test, and multiple multivariate logistic regression. RESULTS: Each hour increase in digital device use is associated with 1.25 odds ratio OR (95% confidence interval (CI) = 1.21-1.30; P < 0.001) increased risk of developing myopic symptoms, each additional hour of digital device use weighted by near-view and blue-light exposure is associated with OR = 1.04 OR (95% CI = 1.03-1.05; P < 0.001) and OR = 2.25 (95% CI = 1.94-2.60; P < 0.001) increased risk respectively. Subjects reporting under stressful conditions are between OR = 1.98 (95% CI = 1.67-2.36; P < 0.001) and OR = 2.03 (95% CI = 1.71-2.42; P < 0.001) more likely to develop myopic symptoms, relative to those citing less stress. CONCLUSIONS: The COVID-19 pandemic led to favorable conditions for myopigenic behavioral changes characterized by extended sedentary engagement with digital devices, which are significantly associated with higher risks of myopia incidence. Relatedly, psychosocial stress accompanying prolonged social isolation during the pandemic is a less noticeable, albeit significant risk factor for myopia development.


Assuntos
COVID-19 , Educação a Distância , Pandemias , Isolamento Social , Acuidade Visual , Adolescente , China , Humanos , Miopia , Fatores de Risco
12.
Front Cell Dev Biol ; 9: 718803, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34568332

RESUMO

Recent advances in the neurobiology and neurogenerative diseases have attracted growing interest in exosomes and their ability to carry and propagate active biomolecules as a means to reprogram recipient cells. Alterations in exosomal protein content and nucleic acid profiles found in human biological fluids have been correlated with various diseases including amyotrophic lateral sclerosis (ALS). In ALS pathogenesis, these lipid-bound nanoscale vesicles have emerged as valuable candidates for diagnostic biomarkers. Moreover, their capacity to spread misfolded proteins and functional non-coding RNAs to interconnected neuronal cells make them putative mediators for the progressive motor degeneration found remarkably apparent in ALS. This review outlines current knowledge concerning the biogenesis, heterogeneity, and function of exosomes in the brain as well as a comprehensive probe of currently available literature on ALS-related exosomal proteins and microRNAs. Lastly, with the rapid development of employing nanoparticles for drug delivery, we explore the therapeutic potentials of exosomes as well as underlying limitations in current isolation and detection methodologies.

13.
Front Neurosci ; 15: 675444, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34149349

RESUMO

INTRODUCTION: The brainstem is an important component in the pathology of amyotrophic lateral sclerosis (ALS). Although neuroimaging studies have shown multiple structural changes in ALS patients, few studies have investigated structural alterations in the brainstem. Herein, we compared the brainstem structure between patients with ALS and healthy controls. METHODS: A total of 33 patients with ALS and 33 healthy controls were recruited in this study. T1-weighted and diffusion tensor imaging (DTI) were acquired on a 3 Tesla magnetic resonance imaging (3T MRI) scanner. Volumetric and vertex-wised approaches were implemented to assess the differences in the brainstem's morphological features between the two groups. An atlas-based region of interest (ROI) analysis was performed to compare the white matter integrity of the brainstem between the two groups. Additionally, a correlation analysis was used to evaluate the relationship between ALS clinical characteristics and structural features. RESULTS: Volumetric analyses showed no significant difference in the subregion volume of the brainstem between ALS patients and healthy controls. In the shape analyses, ALS patients had a local abnormal surface contraction in the ventral medulla oblongata and ventral pons. Compared with healthy controls, ALS patients showed significantly lower fractional anisotropy (FA) in the left corticospinal tract (CST) and bilateral frontopontine tracts (FPT) at the brainstem level, and higher radial diffusivity (RD) in bilateral CST and left FPT at the brainstem level by ROI analysis in DTI. Correlation analysis showed that disease severity was positively associated with FA in left CST and left FPT. CONCLUSION: These findings suggest that the brainstem in ALS suffers atrophy, and degenerative processes in the brainstem may reflect disease severity in ALS. These findings may be helpful for further understanding of potential neural mechanisms in ALS.

14.
Front Pediatr ; 9: 662984, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34113588

RESUMO

The coronavirus (COVID-19) pandemic has impacted education systems globally, making digital devices common arrangements for adolescent learning. However, vision consequences of such behavioral changes are not well-understood. This study investigates the association between duration of daily digital screen engagement and myopic progression among 3,831 Chinese adolescents during the COVID-19 pandemic. Study subjects report an average of 2.70 (SD = 1.77), 3.88 (SD = 2.23), 3.58 (SD = 2.30), and 3.42 (SD = 2.49) hours of television, computer, and smartphone for digital learning use at home, respectively. Researchers analyzed the association between digital screen use and myopic symptoms using statistical tools, and find that every 1 h increase in daily digital screen use is associated with 1.26 OR [Odds Ratio] (95% CI [Confidence Interval: 1.21-1.31, p < 0.001]) higher risks of myopic progression. Using computers (OR = 1.813, 95% CI = 1.05-3.12, p = 0.032) and using smartphones (OR = 2.02, 95% CI = 1.19-3.43, p = 0.009) are shown to be associated with higher risks of myopic progression than television use. Results from additional sensitivity tests that included inverse probability weights which accounted for heterogeneous user profile across different device type categories confirm that these findings are robust. In conclusion, this study finds that daily digital screen use is positively associated with prevalence of myopic progression and holds serious vision health implications for adolescents.

15.
Healthcare (Basel) ; 9(5)2021 Apr 23.
Artigo em Inglês | MEDLINE | ID: mdl-33922486

RESUMO

The coronavirus disease 2019 (COVID-19) pandemic forced many education systems to consider alternative remote e-learning modalities, which have consequential behavioral and health implications for youth. In particular, increased e-learning engagement with digital screens and reduction in outdoor activities are two likely channels posing adverse risks for myopia development. This study investigated the association between e-learning screen use, outdoor activity, lighting condition, and myopia development among school-age children in China, during the first wave of the COVID-19 pandemic. Data were collected from 3405 school-age children attending primary, lower-secondary, and upper-secondary schools in China. Univariate parametric and nonparametric tests, and multivariate logistic regression analysis were used. Findings show that each diopter hour increase in daily e-learning screen use is significantly associated with progression of myopia symptoms (OR: 1.074, 95% CI: 1.058-1.089; p < 0.001), whereas engaging in outdoor exercise four to six times per week (OR: 0.745, 95% CI: 0.568-0.977; p = 0.034) and one to three times per week (OR: 0.829, 95% CI: 0.686-0.991; p = 0.048) is associated with a lower likelihood of myopia progression than none at all. In addition, we found that indoor lighting that is either "too dim" (OR: 1.686, 95% CI: 1.226-2.319; p = 0.001) or "too bright" (OR: 1.529, 95% CI: 1.007-2.366; p = 0.036) is significantly associated higher likelihood of myopic symptoms. Findings in this study uncover the less observable vision consequences of the COVID-19 pandemic on youths through digital online learning and highlight the importance of considering appropriate mitigation strategies to deal with this emerging public health challenge.

16.
Neuroreport ; 32(1): 23-28, 2021 01 06.
Artigo em Inglês | MEDLINE | ID: mdl-33252476

RESUMO

OBJECTIVE: The aim of this study was to explore the upper motor neurons (UMN) and lower motor neurons (LMN) degeneration in amyotrophic lateral sclerosis (ALS) from the perspective of the clinical neurological examination and MRI-electromyography manifold detection, respectively. METHODS: The clinical data, cortical thickness of corresponding areas in different body regions in MRI and electromyography data were collected from 108 classical ALS patients. RESULTS: The kappa value of UMN and LMN involvement signs in the bulbar region (0.31) was higher than that of the left upper limb (-0.13), right upper limb (-0.27), left lower limb (-0.05) and right lower limb (-0.08). The cortical thickness in the positive LMN damage group was thinner than that of the negative LMN damage group in the left head-face area (P < 0.05; Cohen's d = 0.84); however, cortical thickness showed no significant differences in the right head-face, bilateral tongue-larynx, upper-limb, trunk and lower-limb areas between LMN-damage-positive and LMN-damage-negative groups. CONCLUSION: The degeneration of motor neuron could be independent through UMN and LMN levels. The degenerative process was not only confined to UMN and LMN levels but can also expand to white matter fiber tracts. Thus, the degeneration of UMN and LMN might be independent of the motor system's three-dimensional anatomy.


Assuntos
Esclerose Lateral Amiotrófica/diagnóstico , Esclerose Lateral Amiotrófica/patologia , Neurônios Motores/patologia , Degeneração Neural/diagnóstico , Degeneração Neural/patologia , Adulto , Idoso , Eletromiografia/métodos , Feminino , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Exame Neurológico/métodos
17.
BMJ Open ; 10(12): e042603, 2020 12 04.
Artigo em Inglês | MEDLINE | ID: mdl-33277290

RESUMO

INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is a representative rare disease characterised by progressive, fatal motor neuron degeneration. Due to the unknown aetiology and variability of the phenotypes, there are no accurate reports concerning the epidemiology or clinical characteristics of the disease. The low prevalence, as previously reported, makes it difficult to carry out studies with large samples. The aim of this study was to explore the natural history and clinical features of ALS in mainland China through a multicentre, prospective cohort study. The findings will both offer a better understanding of ALS and also support the development of a model to study other rare diseases. METHODS AND ANALYSIS: Patients from 88 representative hospitals in different parts of mainland China will be recruited through a specially designed online data system (http://www.chalsr.net/). We aim to recruit 4752 ALS patients over a 3-year period. Baseline data will be recorded, and follow-up data will be collected every 3 months. The primary outcome is effective survival. Overall survival and indices of disease progression will be measured as the secondary outcomes. ETHICS AND DISSEMINATION: Ethical approval has been obtained from the ethics committee of Peking University Third Hospital (M2019388). Informed written consent will be obtained from each participant. Dissemination of the study protocol and data will take place primarily through a specially designed online data system (http://www.chalsr.net/). The collective results of the study will be published in peer-reviewed journals and shared in scientific presentations. TRIAL REGISTRATION NUMBER: NCT04328675.


Assuntos
Esclerose Lateral Amiotrófica , Esclerose Lateral Amiotrófica/epidemiologia , China/epidemiologia , Progressão da Doença , Humanos , Estudos Multicêntricos como Assunto , Estudos Prospectivos , Sistema de Registros
18.
Sci Rep ; 9(1): 20059, 2019 12 27.
Artigo em Inglês | MEDLINE | ID: mdl-31882886

RESUMO

The aim of this study was to localize the anatomic distribution of upper motor neuron (UMN) loss through examining cortical thickness at the clinical onset of amyotrophic lateral sclerosis (ALS) and explore motor manifestation in functionally impaired body region attribute to impairment of lower motor neuron (LMN) or UMN or mixed LMN and UMN? The clinical features, cortical thickness of corresponding areas from different body regions in MRI and electromyography (EMG) data were collected from 108 classical ALS patients. The cortical thickness was thinner in ALS group than control group in bilateral head-face and upper-limb areas (p < 0.05). In head-face area, the cortical thickness of bulbar-onset group was significantly lower than that of control groups (p < 0.05). In upper-limb areas, the cortical thickness of cervical-onset group was significantly thinner than that of control group. Notably, the bulbar ALSFRS-R subscore was correlated with cortical thickness in bilateral head-face areas (p < 0.05). The bulbar ALSFRS-R subscore of the positive LMN damage group was lower compared to that of the negative LMN damage group (P < 0.001). The limb ALSFRS-R subscore correlated with compound muscle action potential (CMAP) amplitudes of median, ulnar, peroneal, and tibial nerves (P < 0.001), but was not related to cortical thickness. In conclusion, the UMN degeneration in ALS was derived from focal initiation, bulbar- and cervical-onset may date from head-face and upper-limb areas in motor homunculus cortex, respectively. The bulbar dysfunction was resulted from the mixed UMN and LMN impairment, while limb dysfunction derived mostly from LMN loss.


Assuntos
Esclerose Lateral Amiotrófica/fisiopatologia , Neurônios Motores/patologia , Adulto , Idoso , Esclerose Lateral Amiotrófica/diagnóstico por imagem , Esclerose Lateral Amiotrófica/patologia , Estudos de Casos e Controles , Eletromiografia , Feminino , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade
19.
Sci Rep ; 9(1): 17786, 2019 11 28.
Artigo em Inglês | MEDLINE | ID: mdl-31780761

RESUMO

To explore differential diagnosis value of dissociated lower-limb muscle impairment, we performed a retrospective analysis of clinical and electrophysiological features in 141 lower-limb involved ALS patients, 218 normal controls, 67 disease controls, and 32 lumbar spondylosis disease patients. The dissociated lower-limb muscle impairment was quantified by plantar flexion and dorsiflexion strength, compound muscle action potentials ratio of peroneal and tibial nerves (split index, SI) and semi-quantitative scoring scale of denervation potential. Clinical features: the proportion of decreased dorsiflexion was higher than decreased planter flexor strength in lower-limb involved ALS (77.2%vs 38.3%). Electrophysiological features: (1) SI in ALS was the lowest among four groups (Test statistic = 40.57, p < 0.001). (2) Percentage of positive denervation potential was higher in tibialis anterior than gastrocnemius muscle (χ2 = 87.12, p < 0.001). ROC curve: the SI cutoff was 0.52 and 0.33 respectively to differentiate ALS from lumbar spondylosis disease and peripheral neuropathy. Lower-limb involved ALS patients exhibited "split leg" phenomenon. The SI value could be used as an electrophysiological marker to differentiate ALS from lumbar spondylosis disease and peripheral neuropathy.


Assuntos
Esclerose Lateral Amiotrófica/diagnóstico , Esclerose Lateral Amiotrófica/fisiopatologia , Diagnóstico Diferencial , Eletromiografia , Feminino , Humanos , Extremidade Inferior/fisiopatologia , Masculino , Pessoa de Meia-Idade , Músculo Esquelético/fisiopatologia , Doenças do Sistema Nervoso Periférico/diagnóstico , Doenças do Sistema Nervoso Periférico/fisiopatologia , Espondilose/diagnóstico , Espondilose/fisiopatologia
20.
J Clin Neurosci ; 69: 15-20, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31526678

RESUMO

Photosensitive is probably caused by multiple factors including gender, familiar, etc. We aim to study the clinical and EEG features of Chinese Han patients with photosensitivity. A total of 5482 consecutive patients with possible epilepsy from 3 center in China. Of the 73 patients with PPR to IPS, 48 were female. 69.9% patients were evoked by frequency ranged 8 Hz-25 Hz, with accompanying seizures in 13 patients. 6 of 9 patients with eyes closure sensitivity experienced epileptic seizures during IPS. We found some new features: 1) The patients with eyes closure sensitivity apt to experience electro-clinical seizures provoked by IPS; 2) Female epilepsy patients with PPR and ECS maybe difficult to be seizure free. Preventive measures for related seizures should be performed to the patients with generalized PPR, upper threshold evoking frequency, and eyes closure sensitivity when they received the IPS.


Assuntos
Epilepsia Reflexa/epidemiologia , Epilepsia Reflexa/etiologia , Estimulação Luminosa/efeitos adversos , Adolescente , Adulto , Povo Asiático , Criança , China/epidemiologia , Eletroencefalografia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos
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