scholarly journals Misfolded protein aggregation and altered cellular pathways in neurodegenerative diseases

STEMedicine ◽  
2020 ◽  
Vol 1 (4) ◽  
pp. e63
Author(s):  
Huifang Li ◽  
Zhenghong Yu ◽  
Wei Zhang
Keyword(s):  
Neurodegenerative Diseases ◽  
Neurological Disorders ◽  
Cell Types ◽  
World Health ◽  
Protein Homeostasis ◽  
Sensory Impairments ◽  
Cellular Pathways ◽  
Health Organization ◽  
Nonhuman Species ◽  
Human Death

Neurodegenerative diseases are estimated by the World Health Organization to be the second leading cause of human death by 2050. They actually are a group of chronic neurological disorders leading to motor, cognitive and sensory impairments in both human and nonhuman species. Despite different in clinical manifestation, prevalence, risk factors, cell types injured and genes hijacked, neurodegenerative disorders are usually associated with the misfolding and aggregation of a distinct protein that accumulates in diverse cellular locations including the nucleus, cytoplasm, plasma membrane and extracellular space. Here we intend to give an overview of the characters and features of several pathogenic protein aggregates in disease brains, and introduce some general signaling pathways involved in protein homeostasis with emphasis on their puzzling roles under the degenerative conditions.

scholarly journals Roles of Cannabidiol in Reversing Proteinopathies

2020 ◽  
Author(s):  
Raju Dash ◽  
Md. Chayan Ali ◽  
Israt Jahan ◽  
Yeasmin Akter Munni ◽  
Sarmistha Mitra ◽  
...  
Keyword(s):  
Neurodegenerative Diseases ◽  
Neurological Disorders ◽  
Protein Misfolding ◽  
Cannabis Sativa ◽  
Protein Homeostasis ◽  
Compelling Evidence ◽  
The Past ◽  
Clearing System ◽  
Central Nervous Systems ◽  
Preclinical And Clinical Studies

Cannabidiol is a well-known non-psychotropic phytocannabinoid from Cannabis sativa, which exerts a broad range of neuropharmacological activities in the central nervous systems. Over the past years, compelling evidence from preclinical and clinical studies support therapeutic potentials of cannabidiol in various neurological disorders, including neurodegenerative diseases. Neurodegenerative diseases are characterized by the accumulation of misfolded or aggregated protein due to the defective protein homeostasis or proteostasis network, termed as proteinopathies. Because of its role in the protein homeostasis network, cannabidiol could be a potent molecule to revert not only age-associated neurodegeneration but also other protein misfolding disorders. In this review, we discuss the potentiality of cannabidiol as a pharmacological modulator of the proteostasis network, highlighting its neuroprotective and aggregates clearing system inducing potentials in the neurodegenerative diseases.

scholarly journals The emerging spectrum of COVID-19 neurology: clinical, radiological and laboratory findings

Brain ◽  
2020 ◽  
Vol 143 (10) ◽  
pp. 3104-3120 ◽  
Author(s):  
Ross W Paterson ◽  
Rachel L Brown ◽  
Laura Benjamin ◽  
Ross Nortley ◽  
Sarah Wiethoff ◽  
...  
Keyword(s):  
Neurological Disorders ◽  
Early Recognition ◽  
Wide Spectrum ◽  
Acute Disseminated Encephalomyelitis ◽  
World Health ◽  
Brachial Plexopathy ◽  
Partial Recovery ◽  
Laboratory Findings ◽  
Health Organization

Abstract Preliminary clinical data indicate that severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection is associated with neurological and neuropsychiatric illness. Responding to this, a weekly virtual coronavirus disease 19 (COVID-19) neurology multi-disciplinary meeting was established at the National Hospital, Queen Square, in early March 2020 in order to discuss and begin to understand neurological presentations in patients with suspected COVID-19-related neurological disorders. Detailed clinical and paraclinical data were collected from cases where the diagnosis of COVID-19 was confirmed through RNA PCR, or where the diagnosis was probable/possible according to World Health Organization criteria. Of 43 patients, 29 were SARS-CoV-2 PCR positive and definite, eight probable and six possible. Five major categories emerged: (i) encephalopathies (n = 10) with delirium/psychosis and no distinct MRI or CSF abnormalities, and with 9/10 making a full or partial recovery with supportive care only; (ii) inflammatory CNS syndromes (n = 12) including encephalitis (n = 2, para- or post-infectious), acute disseminated encephalomyelitis (n = 9), with haemorrhage in five, necrosis in one, and myelitis in two, and isolated myelitis (n = 1). Of these, 10 were treated with corticosteroids, and three of these patients also received intravenous immunoglobulin; one made a full recovery, 10 of 12 made a partial recovery, and one patient died; (iii) ischaemic strokes (n = 8) associated with a pro-thrombotic state (four with pulmonary thromboembolism), one of whom died; (iv) peripheral neurological disorders (n = 8), seven with Guillain-Barré syndrome, one with brachial plexopathy, six of eight making a partial and ongoing recovery; and (v) five patients with miscellaneous central disorders who did not fit these categories. SARS-CoV-2 infection is associated with a wide spectrum of neurological syndromes affecting the whole neuraxis, including the cerebral vasculature and, in some cases, responding to immunotherapies. The high incidence of acute disseminated encephalomyelitis, particularly with haemorrhagic change, is striking. This complication was not related to the severity of the respiratory COVID-19 disease. Early recognition, investigation and management of COVID-19-related neurological disease is challenging. Further clinical, neuroradiological, biomarker and neuropathological studies are essential to determine the underlying pathobiological mechanisms that will guide treatment. Longitudinal follow-up studies will be necessary to ascertain the long-term neurological and neuropsychological consequences of this pandemic.

Learning Disability

2014 ◽  
Author(s):  
Maggie McGurgan ◽  
Holly Greer
Keyword(s):  
Intellectual Disability ◽  
General Population ◽  
World Health ◽  
Genetic Syndromes ◽  
Sensory Impairments ◽  
The World ◽  
People With Intellectual Disabilities ◽  
Health Organization ◽  
Additional Support ◽  
Complete History

Intellectual disability is defined by the World Health Organization (WHO) as: ‘a significantly reduced ability to understand new or complex infor­mation and to learn and apply new skills (impaired intelligence) resulting in a reduced ability to cope independently (impaired social functioning)’, and begins before adulthood, with a lasting effect on development. People with an intellectual disability can develop any of the mental ill­nesses common to the general population; however, they are up to three times more likely to develop a mental illness. This predisposition to psy­chiatric illness can occur due to a variety of reasons, including associated genetic syndromes, brain injury, and sensory impairments. People with an intellectual disability are also more likely to have negative psychoso­cial experiences, such as deprivation, abuse, separation/loss events, low self-esteem, and financial disadvantage, and consequently the ensuing effects of these can affect their mental health. The psychiatric assessment of a person with an intellectual disability broadly covers the same as that of the general population; however, a different approach at times is needed to adapt to the individual’s com­munication skills. It may be necessary to complete history taking from a family member or carer, and an MSE may even have to be completed solely on observable behaviours. It is also more pertinent to focus on any co-existing medical conditions, such as epilepsy which is present in 25–30% of people with an intellectual disability. The WHO states that the true prevalence of intellectual disability is close to 3%. The vast majority of these people (85%) have mild intel­lectual disability defined as an IQ of 50–69 points. Many of these peo­ple can and do access mainstream services (with or without additional support). In whichever service you work, doctors and medical students will encounter people with intellectual disabilities, and an awareness of their needs is essential.

Correlation of glucose consumption and tumor cell density in astrocytomas

1993 ◽  
Vol 79 (6) ◽  
pp. 853-858 ◽  
Author(s):  
Karl Herholz ◽  
Uwe Pietrzyk ◽  
Jürgen Voges ◽  
Roland Schröder ◽  
Marco Halber ◽  
...  
Keyword(s):  
Tumor Cell ◽  
Cell Density ◽  
Diagnostic Yield ◽  
Glucose Consumption ◽  
Cell Types ◽  
World Health ◽  
Content Type ◽  
Positron Emission ◽  
Stereotactic Frame ◽  
Health Organization

✓ To determine histological correlates of the variability of glucose consumption in astrocytomas, the authors performed positron emission tomography (PET) with 18F-2-fluoro-2-deoxy-D-glucose (FDG) and matched the PET scans three-dimensionally with computerized tomography scans obtained in a stereotactic frame before biopsy. Ten patients with astrocytomas of World Health Organization Grade 2 or 3 were studied; patients with glioblastomas, oligodendrogliomas, or oligoastrocytomas were excluded from the study to avoid any confounding effects of different cell types and necroses. In samples of pure tumor, glucose consumption correlated significantly with cell density, but not with nuclear polymorphism. It is concluded that tumor cell density is a major determinant of glucose consumption in astrocytomas. The use of PET with FDG may help to locate the highest cell density and thus improve the diagnostic yield of stereotactic biopsy.

scholarly journals The importance of smell and taste in everyday life: Dysfunction in COVID-19 patients

2021 ◽  
Vol 72 (3) ◽  
pp. 37-48
Author(s):  
Olivera Stanojlović
Keyword(s):  
Virus Disease ◽  
Cranial Nerves ◽  
Young Patients ◽  
Basic Research ◽  
Cell Types ◽  
Lung Damage ◽  
World Health ◽  
Specific Cell ◽  
Basal Cells ◽  
Health Organization

Human-to-human transmission of coronavirus (SARS-CoV-2) - COVID-19 (corona virus disease 2019) - is characterized by a pandemic exponential rate and the patients with mild to moderate infection have odor and taste problems that represent a new atypical disease. A new viral syndrome of acute anosmia or "new loss of taste or smell" without rhinitis and nasal obstruction or rhinorrhea has been placed on the list of symptoms that may occur 2 to 14 days after exposure to the COVID-19 virus. Two months after declaring the COVID-19 pandemic in May 2020, the World Health Organization (WHO) has recognized changes in the perception of smell and taste as symptoms of this disease. The described cardinal symptoms are more common in the population of young patients and able-bodied people which facilitates the spread of disease. Significantly higher prevalence of patients with COVID-19 who have lost their taste and smell is treated at home (rare hospitalization), lung damage is rare, as well as oxygen therapy with mild lymphopenia. Different scenarios of SARS-CoV-2 viral infection can be assumed: it is probable that the virus does not enter directly into olfactory sensory neurons (they do not have ACE2 and TMPRSS2 receptors), but it is localized to vascular pericytes and causes inflammatory processes and vasculopathies. On the other hand, direct infection of non-neuronal cells which contain said receptors is possible. Those are specific cell types in the olfactory epithelium such as sustentacular, horizontal basal cells, as well as Bowman's glands, which leads to massive degeneration and loss of olfactory neurons. The sense of taste is a complex sensation that is the result of the interaction of smell, taste, temperature and texture of food. The virus damages cranial nerves, epithelial receptors and blood vessels leading to taste damage (ageusia or dysgeusia). A multidisciplinary approach with epidemiological, clinical and basic research is needed to elucidate the mechanism of sensorineural odor and taste loss caused by coronavirus.

Stem cells hold key to tackling brain disease

Impact ◽  
2020 ◽  
Vol 2020 (7) ◽  
pp. 67-69
Author(s):  
Yun-Bae Kim ◽  
Ehn-Kyoung Choi ◽  
Hye-Rim Seong ◽  
Sangryong Park
Keyword(s):  
Stem Cells ◽  
Stem Cell ◽  
Neurodegenerative Diseases ◽  
Stem Cell Research ◽  
Central Research Institute ◽  
World Health ◽  
Central Research ◽  
Ache Inhibitors ◽  
The World ◽  
Health Organization

A rapidly ageing global society means a higher prevalence of neurodegenerative diseases. Looking at dementia, for example, the most common form of which is Alzheimer's disease (AD), the World Health Organization estimates that worldwide, around 50 million people have dementia, and there are nearly 10 million new cases every year. And, it's predicted that this number could rise to 150 million people by the year 2050. It's clear that consistent and continued efforts to explore methods of prevention and treatments are required. Existing treatments include acetylcholinesterase (AChE) inhibitors, antibodies to Aβ and anti-inflammatory agents, but these are not without their drawbacks and limitations and new avenues must be explored. A group of Korean scientists believes that stem cells and regenerative medicine hold the key to alleviating the global burden of neurodegenerative diseases and this is where they are focusing their efforts. The innovative stem cell research being undertaken by Designed Cells Co., Ltd, which is based in Korea, performs important stem cell research geared towards improving life – enabling people to age gracefully and maintain wellbeing – and making treatments for neurodegenerative diseases available to all who need them. Professor James Yun-Bae Kim is CEO of the company and Dr Ehn-Kyoung Choi is Director of the company's Central Research Institute. Through their work, the researchers are collaborating with numerous companies spanning different fields, the team is keen to work with global partners in any field to further expand their work to develop treatments for AD and more widely demonstrate the effectiveness of their research.

The clinical use of quality-of-life scales in neurological disorders

2013 ◽  
Vol 25 (4) ◽  
pp. 193-205 ◽  
Author(s):  
Francesco Corallo ◽  
Rosaria De Luca ◽  
Roberta Leonardi ◽  
Simona De Salvo ◽  
Placido Bramanti ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Neurological Disorders ◽  
Methodological Approach ◽  
Well Being ◽  
World Health ◽  
Global Evaluation ◽  
Life Itself ◽  
Health Organization ◽  
The Impact

ObjectiveQuality of life (QoL) is a growing issue in medicine, particularly in the evaluation of rehabilitative care. The concept of QoL is included in and expands the definition of health given by the WHO (World Health Organization) and comprises complete physical, mental, and social well-being. It expresses the degree of satisfaction in various areas as a result of the opportunities that arise during one's lifetime despite the restrictions and impediments that life itself puts forth. The last decade has exponentially increased the number of studies on QoL, although they are still limited.MethodsWe performed a literature review on the QoL scales used in patients with neurological disorders.ResultsRecent studies have shown the importance of QoL assessment because standard treatments do not assess the treatment impact felt by the patient. In fact, by understanding the impact of treatment on survival and QoL, one can make a clearer interpretation of the health of the patient.ConclusionThis review has adopted an innovative holistic methodological approach, which allowed a global evaluation of the comfort reported by the patients. The scales applied in this study allowed to choose the most suitable therapeutic strategies and programme individual therapeutic treatment.

scholarly journals Ribosomal Protein S27/Metallopanstimulin-1 (RPS27) in Glioma—A New Disease Biomarker?

Cancers ◽  
2020 ◽  
Vol 12 (5) ◽  
pp. 1085 ◽  
Author(s):  
Jonas Feldheim ◽  
Almuth F. Kessler ◽  
Dominik Schmitt ◽  
Ellaine Salvador ◽  
Camelia M. Monoranu ◽  
...  
Keyword(s):  
Tumor Cells ◽  
Cell Types ◽  
World Health ◽  
Malignant Neoplasms ◽  
Idh Mutation ◽  
Fluorescence Staining ◽  
Who Grade ◽  
The Central Nervous System ◽  
Grade Ii ◽  
Health Organization

Despite its significant overexpression in several malignant neoplasms, the expression of RPS27 in the central nervous system (CNS) is widely unknown. We identified the cell types expressing RPS27 in the CNS under normal and disease conditions. We acquired specimens of healthy brain (NB), adult pilocytic astrocytoma (PA) World Health Organization (WHO) grade I, anaplastic PA WHO grade III, gliomas WHO grade II/III with or without isocitrate dehydrogenase (IDH) mutation, and glioblastoma multiforme (GBM). RPS27 protein expression was examined by immunohistochemistry and double-fluorescence staining and its mRNA expression quantified by RT-PCR. Patients’ clinical and tumor characteristics were collected retrospectively. RPS27 protein was specifically expressed in tumor cells and neurons, but not in healthy astrocytes. In tumor tissue, most macrophages were positive, while this was rarely the case in inflamed tissue. Compared to NB, RPS27 mRNA was in mean 6.2- and 8.8-fold enhanced in gliomas WHO grade II/III with (p < 0.01) and without IDH mutation (p = 0.01), respectively. GBM displayed a 4.6-fold increased mean expression (p = 0.02). Although RPS27 expression levels did not affect the patients’ survival, their association with tumor cells and tumor-associated macrophages provides a rationale for a future investigation of a potential function during gliomagenesis and tumor immune response.

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