senior presentation
•Download as PPTX, PDF•
2 likes•156 views
The document discusses the similarities between Alzheimer's disease and prion disorders like Creutzfeldt-Jakob disease. It argues that Alzheimer's should be investigated as a prion disorder due to shared physiological characteristics like tau proteins and amyloid plaques. While some evidence suggests the prion precursor protein in Alzheimer's could develop into an active prion, the evidence is not yet conclusive. More research is needed to definitively determine whether Alzheimer's is a prion disease.
Report
Share
Report
Share
Recommended
Prion disease
Prion diseases are rare neurodegenerative disorders caused by misfolded prion proteins. They affect both humans and animals. In cattle it is known as bovine spongiform encephalopathy (BSE) or "mad cow disease", and in humans it is known as Creutzfeldt-Jakob disease (CJD). Prion diseases occur when normal prion proteins misfold and induce other prion proteins to also misfold, triggering a chain reaction that causes damage to neural cells. There is no cure for prion diseases and diagnosis is difficult since prion proteins are similar to normal forms.
PRIONS THE CLINICAL CURIOSITY
Prions are abnormal protein particles that can cause neurodegenerative diseases. They have a normal function in the body but can transform into an infectious form. When this happens, they induce other normal prion proteins to adopt the abnormal shape. Common prion diseases include scrapie in sheep, mad cow disease, and Creutzfeldt-Jakob disease in humans. There are currently no effective treatments for prion diseases, so prevention and diagnosis are important.
Cjdfgrcincinnati2009 7-19-090722141427-phpapp02
This document summarizes research on prion diseases like Creutzfeldt-Jakob disease (CJD). It discusses the causes of prion diseases including genetic mutations and spontaneous misfolding. The document outlines the clinical diagnosis and tests used to diagnose CJD. It also describes recent investigational treatments for CJD like quinacrine, pentosan polysulfate, and doxycycline. Finally, it provides an overview of resources for patients and their families dealing with prion diseases.
Proin diseases
This document provides an overview of prion diseases, also known as transmissible spongiform encephalopathies (TSEs). It defines prion diseases as progressive neurodegenerative disorders that can affect both humans and animals. The causative agent is an abnormal prion protein (PrPsc) which induces abnormal folding of normal prion proteins in neurons. Several human and animal prion diseases are described, including their signs, symptoms, transmission methods, and neuropathological features. The mechanism of prion propagation and the role of the normal (PrPc) and abnormal (PrPsc) prion proteins are explained.
Prion diseases
The document discusses prion diseases, which are neurodegenerative disorders caused by abnormal prion proteins. It covers the discovery of prions, the different forms of human and animal prion diseases, and the molecular pathogenesis involving the conversion of normal prion protein (PrPc) to abnormal disease-causing prion protein (PrPsc). Clinical features of various human prion diseases like CJD are described. Diagnosis involves neuroimaging, EEG, CSF analysis and detection of PrPsc in brain tissue. Histopathology shows vacuolation, gliosis and prion plaques. Variant CJD results from eating meat from cows with BSE.
Prion Protein
The human PRNP gene is located on chromosome 20 and encodes the prion protein (PrP). Mutations in this gene can cause neurodegenerative diseases like Creutzfeldt-Jakob disease. The protein's exact function is unknown but it may be involved in copper transport, neuroprotection, and communication between neurons. Alternatively spliced variants have been identified for this gene and the mouse protein shares high sequence identity with the human protein. Clinically relevant genetic variants in PRNP have been associated with prion diseases.
Prion diseases
This document discusses a case of a 70-year-old male presenting with rapidly progressive dementia. Clinical exams and tests showed signs of a neurodegenerative disease but no clear diagnosis. A positive 14-3-3 protein from CSF suggested Creutzfeldt-Jakob disease (CJD). The document then provides background on prion diseases like CJD, discussing their pathology, diagnosis, variants like variant CJD, and public health implications. It indicates the patient will be placed in CJD surveillance and further tests are pending to confirm diagnosis.
Creutzfeldt jakob disease
Creutzfeldt-Jakob disease (CJD) is a rare, fatal brain disorder that affects about one in every one million people per year. Symptoms usually appear later in life and the disease runs a rapid course, with 90% of patients dying within one year. CJD belongs to a family of diseases called transmissible spongiform encephalopathies caused by misfolded prion proteins, which are abnormal infectious protein particles that cause normal prion proteins to take on the misfolded shape. Prion diseases are difficult to treat as prions are difficult to kill and do not contain genetic material.
Recommended
Prion disease
Prion diseases are rare neurodegenerative disorders caused by misfolded prion proteins. They affect both humans and animals. In cattle it is known as bovine spongiform encephalopathy (BSE) or "mad cow disease", and in humans it is known as Creutzfeldt-Jakob disease (CJD). Prion diseases occur when normal prion proteins misfold and induce other prion proteins to also misfold, triggering a chain reaction that causes damage to neural cells. There is no cure for prion diseases and diagnosis is difficult since prion proteins are similar to normal forms.
PRIONS THE CLINICAL CURIOSITY
Prions are abnormal protein particles that can cause neurodegenerative diseases. They have a normal function in the body but can transform into an infectious form. When this happens, they induce other normal prion proteins to adopt the abnormal shape. Common prion diseases include scrapie in sheep, mad cow disease, and Creutzfeldt-Jakob disease in humans. There are currently no effective treatments for prion diseases, so prevention and diagnosis are important.
Cjdfgrcincinnati2009 7-19-090722141427-phpapp02
This document summarizes research on prion diseases like Creutzfeldt-Jakob disease (CJD). It discusses the causes of prion diseases including genetic mutations and spontaneous misfolding. The document outlines the clinical diagnosis and tests used to diagnose CJD. It also describes recent investigational treatments for CJD like quinacrine, pentosan polysulfate, and doxycycline. Finally, it provides an overview of resources for patients and their families dealing with prion diseases.
Proin diseases
This document provides an overview of prion diseases, also known as transmissible spongiform encephalopathies (TSEs). It defines prion diseases as progressive neurodegenerative disorders that can affect both humans and animals. The causative agent is an abnormal prion protein (PrPsc) which induces abnormal folding of normal prion proteins in neurons. Several human and animal prion diseases are described, including their signs, symptoms, transmission methods, and neuropathological features. The mechanism of prion propagation and the role of the normal (PrPc) and abnormal (PrPsc) prion proteins are explained.
Prion diseases
The document discusses prion diseases, which are neurodegenerative disorders caused by abnormal prion proteins. It covers the discovery of prions, the different forms of human and animal prion diseases, and the molecular pathogenesis involving the conversion of normal prion protein (PrPc) to abnormal disease-causing prion protein (PrPsc). Clinical features of various human prion diseases like CJD are described. Diagnosis involves neuroimaging, EEG, CSF analysis and detection of PrPsc in brain tissue. Histopathology shows vacuolation, gliosis and prion plaques. Variant CJD results from eating meat from cows with BSE.
Prion Protein
The human PRNP gene is located on chromosome 20 and encodes the prion protein (PrP). Mutations in this gene can cause neurodegenerative diseases like Creutzfeldt-Jakob disease. The protein's exact function is unknown but it may be involved in copper transport, neuroprotection, and communication between neurons. Alternatively spliced variants have been identified for this gene and the mouse protein shares high sequence identity with the human protein. Clinically relevant genetic variants in PRNP have been associated with prion diseases.
Prion diseases
This document discusses a case of a 70-year-old male presenting with rapidly progressive dementia. Clinical exams and tests showed signs of a neurodegenerative disease but no clear diagnosis. A positive 14-3-3 protein from CSF suggested Creutzfeldt-Jakob disease (CJD). The document then provides background on prion diseases like CJD, discussing their pathology, diagnosis, variants like variant CJD, and public health implications. It indicates the patient will be placed in CJD surveillance and further tests are pending to confirm diagnosis.
Creutzfeldt jakob disease
Creutzfeldt-Jakob disease (CJD) is a rare, fatal brain disorder that affects about one in every one million people per year. Symptoms usually appear later in life and the disease runs a rapid course, with 90% of patients dying within one year. CJD belongs to a family of diseases called transmissible spongiform encephalopathies caused by misfolded prion proteins, which are abnormal infectious protein particles that cause normal prion proteins to take on the misfolded shape. Prion diseases are difficult to treat as prions are difficult to kill and do not contain genetic material.
Prion diseases
Prion diseases, also known as transmissible spongiform encephalopathies, are rare progressive neurodegenerative disorders caused by prions. Prion diseases affect both humans and animals. There are several types of human prion diseases including Creutzfeldt-Jakob disease, variant Creutzfeldt-Jakob disease, kuru, and Gerstmann-Straussler-Scheinker syndrome. Prion diseases are caused by abnormal folding of the prion protein which forms plaques that damage the brain. There are sporadic, genetic, and acquired forms of prion diseases. Currently, there are no effective treatments for human prion diseases which are universally fatal.
Prion diseases
This document discusses prion diseases, also known as transmissible spongiform encephalopathies (TSEs). It notes that TSEs have long incubation periods and progressive clinical courses. They can be caused by conventional or unconventional agents and include diseases like Creutzfeldt-Jakob disease (CJD) in humans and bovine spongiform encephalopathy (BSE) in cattle. Prions, the infectious agents responsible for TSEs, are abnormal forms of proteins that can induce normal proteins to also take the abnormal form. They are resistant to heat, chemicals, and radiation. Diagnosis of prion diseases is difficult due to a lack of immune response or signs of infection. Currently, there
Prion disease: A Rare Phenomena
This document discusses prion diseases like Creutzfeldt-Jakob disease (CJD). It covers diagnosing CJD using tools like EEG, MRI, and spinal fluid tests. It also discusses managing patient care which involves symptom treatment, limiting overstimulation, and hospice support. The risks of CJD transmission are also addressed, noting standard precautions are usually sufficient. Future research aims to improve diagnosis and develop treatments for prion diseases.
Creutzfeld-Jakob Disease: Diagnosis and Management of Prion Diseases
I. Dr. Brian Appleby discusses diagnosing and managing Creutzfeldt-Jakob disease (CJD), including using EEG, MRI, CSF tests and brain biopsy for diagnosis. Symptomatic treatments aim to improve quality of life.
II. Risk of CJD transmission in clinical settings is low with standard precautions. Care involves managing symptoms, supporting patients and caregivers, and facilitating end-of-life planning.
III. Current research includes studies of bioassays, diagnostic factors, and treatments like art therapy, with the goal of better understanding and caring for those with prion diseases.
Clinical Assessment & Management of Prion Disease
This document summarizes information on diagnosing and managing prion disease:
- It describes tools used to diagnose prion disease such as CSF tests, EEGs, and brain MRIs showing abnormalities. The #1 rule is to consider alternative diagnoses first due to the consequences of missing treatable conditions.
- A case example is presented of a patient with ataxia, myoclonus and cognitive impairment, and brain MRI shows changes characteristic of prion disease.
- Experimental treatments for prion disease are discussed but evidence suggests they provide only temporary benefits at best, with no treatment stopping disease progression. Symptomatic treatments aim to manage symptoms like psychosis, myoclonus and seizures.
An Introduction of Prion protein
A prion is a protein that can fold in multiple, structurally distinct ways, at least one of which is transmissible to other prion proteins. It is this form of replication that leads to disease that is similar to viral infection. The word prion, coined in 1982 by Stanley B. Prusiner, is short for “proteinaceous infectious particle” derived from the words protein and infection, in reference to a prion's ability to self-propagate and transmit its conformation to other prions. While several yeast proteins have been identified as having prionogenic properties, the first prion protein was discovered in mammals and is referred to as the major prion protein (PrP). This infectious agent causes mammalian transmissible spongiform encephalopathies, including bovine spongiform encephalopathy (BSE, also known as "mad cow disease") and scrapie in sheep. In humans, PrP causes Creutzfeldt-Jakob Disease (CJD), variant Creutzfeldt-Jakob Disease (vCJD), Gerstmann–Sträussler–Scheinker syndrome, Fatal Familial Insomnia and kuru.
CJD Project final 4.16.15
Creutzfeldt-Jakob disease (CJD) is a fatal brain disorder caused by prion proteins. Prions are abnormal proteins that induce normal proteins to fold abnormally, damaging the brain. CJD symptoms include rapidly progressive dementia and neurological problems. There are four main types - sporadic, inherited, iatrogenic from medical treatment, and variant CJD from "mad cow disease." Currently there are no effective treatments and CJD is always fatal. Researchers are working to better understand prion diseases and develop diagnostic tests and treatments.
Finalprojectmadcow v cjd amaliamartinez
Mad Cow Disease, also known as Bovine Spongiform Encephalopathy (BSE), is a fatal neurodegenerative disease affecting cattle. It belongs to a group of diseases called Transmissible Spongiform Encephalopathies (TSEs) that can affect both animals and humans. A related human disease is variant Creutzfeldt-Jakob disease (vCJD), which is believed to be caused by eating beef infected with BSE. Both diseases are caused by an abnormal prion protein that changes the shape of normal prion proteins in the brain, causing neurodegeneration. There is currently no treatment for either disease, so prevention focuses on restricting animal feed and meat imports.
2007 CJD Presentation - Graham Steel
This document discusses the formation and roles of support groups for transmissible spongiform encephalopathies (TSEs) like Creutzfeldt-Jakob disease (CJD) over time. It outlines the timeline of when major support groups around the world were established from 1992 to present. It also discusses the challenges faced in understanding and finding treatments for these fatal neurodegenerative diseases.
Diseases caused by prions
Misfolded proteins which catalyses the refolding of the native protein into the misfolded pathogenic forms are called as Prions. They are responsible for fatal neurodegenerative diseases. Some of the neurodegenerative diseases such as Scrapie, BSE, CWD of animals and Kuru, CJD, Fatal familial insomnia of human beings are included in the presentation.
Creutzfeldt–Jakob disease
This patient, a 65-year-old male, presented with 6 months of gradually worsening gait difficulties and incoordination. Over the past 4 months, he developed forgetfulness, hallucinations, and sensorium changes. Exams found rigidity, myoclonic jerks, and a non-reactive semi-conscious state. Imaging and tests were suggestive of Creutzfeldt-Jakob disease (CJD), a prion disease characterized by spongiform changes in the brain. A definitive diagnosis requires brain biopsy or autopsy.
Creutzfeld-Jakob's Disease Research
Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disorder caused by prions, which are abnormally folded proteins that promote the misfolding of normal prion proteins in the brain. CJD leads to rapid mental deterioration and death within a year. There are several types of CJD including sporadic, familial, and variant CJD linked to mad cow disease. Currently there is no cure for CJD and diagnosis can only be confirmed by brain biopsy. Research continues to study prion transmission and potential treatments.
Cjd
This article discusses Creutzfeldt-Jakob disease (CJD), a rare and fatal neurological disorder caused by misfolded prion proteins in the brain. It describes the clinical presentation of CJD, which includes rapidly progressive dementia, personality changes, involuntary movements, and death usually within 6 months of symptom onset. The article outlines different types of CJD, discusses the pathogenesis involving prion transmission and brain degeneration, and summarizes diagnostic studies and management approaches, which are largely supportive since there is no cure. Nursing care focuses on preventing injury, promoting comfort, and providing psychological support to patients and families.
Stem cell research in Parkinson's Disease
Stem cell research shows promise for treating Parkinson's disease. Studies have found that transplanting embryonic stem cells or neural stem cells into animal models of Parkinson's can reduce symptoms by generating new dopamine-producing cells. However, many of these transplanted cells have failed to maintain their function over time or have caused tumor formation. A clinical trial in Peru found that transplanting bone marrow-derived stem cells into the brains of Parkinson's patients improved their symptoms, but long-term data is still needed. Future research aims to develop safer and more effective stem cell therapies by improving protocols and understanding how to support long-term cell survival and specialization without tumor risks.
Prion disease
This document discusses several neurodegenerative diseases including prion diseases, Alzheimer's disease, and Parkinson's disease. Prion diseases are a group of rare progressive conditions caused by prions, which are abnormal folded proteins. Common types include Creutzfeldt-Jakob disease and variant CJD. Alzheimer's disease is characterized by beta-amyloid plaques and neurofibrillary tangles leading to dementia. Parkinson's disease results from degeneration of dopaminergic neurons and is associated with Lewy bodies containing alpha-synuclein.
The brain eater (Creutzfeldt Jakob Disease)
Creutzfeldt-Jakob disease (CJD) is a rapidly progressive neurodegenerative brain disorder caused by prions. Symptoms include dementia, muscle stiffness, and myoclonus. It is always fatal, with death usually occurring within 1 year of symptom onset. While there is no cure, management focuses on symptom relief. CJD can occur sporadically, through infection or genetic mutation, and was first described in the early 1920s.
new gene and new target in alzheimer disease
This review summarizes recent advances in understanding the genetic underpinnings of Alzheimer's disease (AD). While mutations in amyloid precursor protein and presenilin 1 and 2 genes cause early-onset familial AD, the Apolipoprotein E ε4 allele and other common gene variants confer small increased risks for late-onset AD. New genetic studies are identifying additional novel AD risk genes to improve disease understanding and identify potential therapeutic targets. Current drug development focuses on modifying amyloid β production or clearance through vaccines, monoclonal antibodies, and inhibitors of β- and γ-secretase, but most candidates have so far shown limited efficacy in clinical trials.
Diagnosis of Creutzfeldt-Jakob Disease
This document provides information on diagnosing Creutzfeldt-Jakob Disease (CJD). It discusses the importance of considering other treatable causes of rapidly progressive dementias before diagnosing CJD. It outlines the diagnostic criteria for probable sporadic CJD, including having at least two clinical signs and one positive diagnostic test result. The document discusses various diagnostic tests used for CJD like CSF 14-3-3, EEG, and brain MRI findings. It notes that conditions other than CJD can produce positive 14-3-3 results and that genetic mutations may have unknown or age-related penetrance. Finally, it provides a case report example of a patient initially responding to psychiatric treatment before being found to have a
Kelly agudelo priones molecular
1. Prion diseases such as Creutzfeldt-Jacob disease can be transmitted from mother sheep to offspring and may be spread through airborne transmission.
2. Fatal familial insomnia is a prion disease characterized by progressively worsening insomnia and autonomic dysfunction that ultimately leads to death.
3. Definitive diagnosis of prion diseases requires brain biopsy or autopsy, though neuroimaging and cerebrospinal fluid analysis can provide supportive evidence for suspected cases.
Neuron Glial Interactions
The dance between Glia and Neurons is critical to the development and maintenance to CNS. Did you know: • up to 90% of the cells in the vertebrate nervous system are “not neurons” • make > 50% of the brain volume. This is a great overview to anyone that wants to learn more.
Concussions and brain health
The document discusses various topics related to brain health and trauma, including:
- The human brain contains hundreds of billions of neurons and synaptic connections that can process information rapidly.
- Trauma and certain disorders can cause permanent changes to brain structure and function, including conditions like Alzheimer's, Parkinson's, depression, and post-traumatic stress.
- The hippocampus plays a role in many functions including pleasure, rage, emotions, and is involved in mood disorders like depression and bipolar disorder. It has been found to be smaller in people with these conditions.
- Repeated head injuries increase the risk of chronic traumatic encephalopathy, while injuries to the upper neck can cause symptoms of multiple sclerosis and Parkinson's disease
Alzheimers disease
The document discusses the etiopathogenesis, assessment, diagnosis, management and recent advances in Alzheimer's disease. It provides details on the history, epidemiology, risk factors, pathophysiology involving amyloid plaques and neurofibrillary tangles, assessment tools including cognitive tests, and the involvement of genetics. The management focuses on pharmacological and non-pharmacological approaches to address the cognitive and behavioral symptoms of Alzheimer's disease.
More Related Content
What's hot
Prion diseases
Prion diseases, also known as transmissible spongiform encephalopathies, are rare progressive neurodegenerative disorders caused by prions. Prion diseases affect both humans and animals. There are several types of human prion diseases including Creutzfeldt-Jakob disease, variant Creutzfeldt-Jakob disease, kuru, and Gerstmann-Straussler-Scheinker syndrome. Prion diseases are caused by abnormal folding of the prion protein which forms plaques that damage the brain. There are sporadic, genetic, and acquired forms of prion diseases. Currently, there are no effective treatments for human prion diseases which are universally fatal.
Prion diseases
This document discusses prion diseases, also known as transmissible spongiform encephalopathies (TSEs). It notes that TSEs have long incubation periods and progressive clinical courses. They can be caused by conventional or unconventional agents and include diseases like Creutzfeldt-Jakob disease (CJD) in humans and bovine spongiform encephalopathy (BSE) in cattle. Prions, the infectious agents responsible for TSEs, are abnormal forms of proteins that can induce normal proteins to also take the abnormal form. They are resistant to heat, chemicals, and radiation. Diagnosis of prion diseases is difficult due to a lack of immune response or signs of infection. Currently, there
Prion disease: A Rare Phenomena
This document discusses prion diseases like Creutzfeldt-Jakob disease (CJD). It covers diagnosing CJD using tools like EEG, MRI, and spinal fluid tests. It also discusses managing patient care which involves symptom treatment, limiting overstimulation, and hospice support. The risks of CJD transmission are also addressed, noting standard precautions are usually sufficient. Future research aims to improve diagnosis and develop treatments for prion diseases.
Creutzfeld-Jakob Disease: Diagnosis and Management of Prion Diseases
I. Dr. Brian Appleby discusses diagnosing and managing Creutzfeldt-Jakob disease (CJD), including using EEG, MRI, CSF tests and brain biopsy for diagnosis. Symptomatic treatments aim to improve quality of life.
II. Risk of CJD transmission in clinical settings is low with standard precautions. Care involves managing symptoms, supporting patients and caregivers, and facilitating end-of-life planning.
III. Current research includes studies of bioassays, diagnostic factors, and treatments like art therapy, with the goal of better understanding and caring for those with prion diseases.
Clinical Assessment & Management of Prion Disease
This document summarizes information on diagnosing and managing prion disease:
- It describes tools used to diagnose prion disease such as CSF tests, EEGs, and brain MRIs showing abnormalities. The #1 rule is to consider alternative diagnoses first due to the consequences of missing treatable conditions.
- A case example is presented of a patient with ataxia, myoclonus and cognitive impairment, and brain MRI shows changes characteristic of prion disease.
- Experimental treatments for prion disease are discussed but evidence suggests they provide only temporary benefits at best, with no treatment stopping disease progression. Symptomatic treatments aim to manage symptoms like psychosis, myoclonus and seizures.
An Introduction of Prion protein
A prion is a protein that can fold in multiple, structurally distinct ways, at least one of which is transmissible to other prion proteins. It is this form of replication that leads to disease that is similar to viral infection. The word prion, coined in 1982 by Stanley B. Prusiner, is short for “proteinaceous infectious particle” derived from the words protein and infection, in reference to a prion's ability to self-propagate and transmit its conformation to other prions. While several yeast proteins have been identified as having prionogenic properties, the first prion protein was discovered in mammals and is referred to as the major prion protein (PrP). This infectious agent causes mammalian transmissible spongiform encephalopathies, including bovine spongiform encephalopathy (BSE, also known as "mad cow disease") and scrapie in sheep. In humans, PrP causes Creutzfeldt-Jakob Disease (CJD), variant Creutzfeldt-Jakob Disease (vCJD), Gerstmann–Sträussler–Scheinker syndrome, Fatal Familial Insomnia and kuru.
CJD Project final 4.16.15
Creutzfeldt-Jakob disease (CJD) is a fatal brain disorder caused by prion proteins. Prions are abnormal proteins that induce normal proteins to fold abnormally, damaging the brain. CJD symptoms include rapidly progressive dementia and neurological problems. There are four main types - sporadic, inherited, iatrogenic from medical treatment, and variant CJD from "mad cow disease." Currently there are no effective treatments and CJD is always fatal. Researchers are working to better understand prion diseases and develop diagnostic tests and treatments.
Finalprojectmadcow v cjd amaliamartinez
Mad Cow Disease, also known as Bovine Spongiform Encephalopathy (BSE), is a fatal neurodegenerative disease affecting cattle. It belongs to a group of diseases called Transmissible Spongiform Encephalopathies (TSEs) that can affect both animals and humans. A related human disease is variant Creutzfeldt-Jakob disease (vCJD), which is believed to be caused by eating beef infected with BSE. Both diseases are caused by an abnormal prion protein that changes the shape of normal prion proteins in the brain, causing neurodegeneration. There is currently no treatment for either disease, so prevention focuses on restricting animal feed and meat imports.
2007 CJD Presentation - Graham Steel
This document discusses the formation and roles of support groups for transmissible spongiform encephalopathies (TSEs) like Creutzfeldt-Jakob disease (CJD) over time. It outlines the timeline of when major support groups around the world were established from 1992 to present. It also discusses the challenges faced in understanding and finding treatments for these fatal neurodegenerative diseases.
Diseases caused by prions
Misfolded proteins which catalyses the refolding of the native protein into the misfolded pathogenic forms are called as Prions. They are responsible for fatal neurodegenerative diseases. Some of the neurodegenerative diseases such as Scrapie, BSE, CWD of animals and Kuru, CJD, Fatal familial insomnia of human beings are included in the presentation.
Creutzfeldt–Jakob disease
This patient, a 65-year-old male, presented with 6 months of gradually worsening gait difficulties and incoordination. Over the past 4 months, he developed forgetfulness, hallucinations, and sensorium changes. Exams found rigidity, myoclonic jerks, and a non-reactive semi-conscious state. Imaging and tests were suggestive of Creutzfeldt-Jakob disease (CJD), a prion disease characterized by spongiform changes in the brain. A definitive diagnosis requires brain biopsy or autopsy.
Creutzfeld-Jakob's Disease Research
Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disorder caused by prions, which are abnormally folded proteins that promote the misfolding of normal prion proteins in the brain. CJD leads to rapid mental deterioration and death within a year. There are several types of CJD including sporadic, familial, and variant CJD linked to mad cow disease. Currently there is no cure for CJD and diagnosis can only be confirmed by brain biopsy. Research continues to study prion transmission and potential treatments.
Cjd
This article discusses Creutzfeldt-Jakob disease (CJD), a rare and fatal neurological disorder caused by misfolded prion proteins in the brain. It describes the clinical presentation of CJD, which includes rapidly progressive dementia, personality changes, involuntary movements, and death usually within 6 months of symptom onset. The article outlines different types of CJD, discusses the pathogenesis involving prion transmission and brain degeneration, and summarizes diagnostic studies and management approaches, which are largely supportive since there is no cure. Nursing care focuses on preventing injury, promoting comfort, and providing psychological support to patients and families.
Stem cell research in Parkinson's Disease
Stem cell research shows promise for treating Parkinson's disease. Studies have found that transplanting embryonic stem cells or neural stem cells into animal models of Parkinson's can reduce symptoms by generating new dopamine-producing cells. However, many of these transplanted cells have failed to maintain their function over time or have caused tumor formation. A clinical trial in Peru found that transplanting bone marrow-derived stem cells into the brains of Parkinson's patients improved their symptoms, but long-term data is still needed. Future research aims to develop safer and more effective stem cell therapies by improving protocols and understanding how to support long-term cell survival and specialization without tumor risks.
Prion disease
This document discusses several neurodegenerative diseases including prion diseases, Alzheimer's disease, and Parkinson's disease. Prion diseases are a group of rare progressive conditions caused by prions, which are abnormal folded proteins. Common types include Creutzfeldt-Jakob disease and variant CJD. Alzheimer's disease is characterized by beta-amyloid plaques and neurofibrillary tangles leading to dementia. Parkinson's disease results from degeneration of dopaminergic neurons and is associated with Lewy bodies containing alpha-synuclein.
The brain eater (Creutzfeldt Jakob Disease)
Creutzfeldt-Jakob disease (CJD) is a rapidly progressive neurodegenerative brain disorder caused by prions. Symptoms include dementia, muscle stiffness, and myoclonus. It is always fatal, with death usually occurring within 1 year of symptom onset. While there is no cure, management focuses on symptom relief. CJD can occur sporadically, through infection or genetic mutation, and was first described in the early 1920s.
new gene and new target in alzheimer disease
This review summarizes recent advances in understanding the genetic underpinnings of Alzheimer's disease (AD). While mutations in amyloid precursor protein and presenilin 1 and 2 genes cause early-onset familial AD, the Apolipoprotein E ε4 allele and other common gene variants confer small increased risks for late-onset AD. New genetic studies are identifying additional novel AD risk genes to improve disease understanding and identify potential therapeutic targets. Current drug development focuses on modifying amyloid β production or clearance through vaccines, monoclonal antibodies, and inhibitors of β- and γ-secretase, but most candidates have so far shown limited efficacy in clinical trials.
Diagnosis of Creutzfeldt-Jakob Disease
This document provides information on diagnosing Creutzfeldt-Jakob Disease (CJD). It discusses the importance of considering other treatable causes of rapidly progressive dementias before diagnosing CJD. It outlines the diagnostic criteria for probable sporadic CJD, including having at least two clinical signs and one positive diagnostic test result. The document discusses various diagnostic tests used for CJD like CSF 14-3-3, EEG, and brain MRI findings. It notes that conditions other than CJD can produce positive 14-3-3 results and that genetic mutations may have unknown or age-related penetrance. Finally, it provides a case report example of a patient initially responding to psychiatric treatment before being found to have a
Kelly agudelo priones molecular
1. Prion diseases such as Creutzfeldt-Jacob disease can be transmitted from mother sheep to offspring and may be spread through airborne transmission.
2. Fatal familial insomnia is a prion disease characterized by progressively worsening insomnia and autonomic dysfunction that ultimately leads to death.
3. Definitive diagnosis of prion diseases requires brain biopsy or autopsy, though neuroimaging and cerebrospinal fluid analysis can provide supportive evidence for suspected cases.
What's hot (19)
Creutzfeld-Jakob Disease: Diagnosis and Management of Prion Diseases
Creutzfeld-Jakob Disease: Diagnosis and Management of Prion Diseases
Similar to senior presentation
Neuron Glial Interactions
The dance between Glia and Neurons is critical to the development and maintenance to CNS. Did you know: • up to 90% of the cells in the vertebrate nervous system are “not neurons” • make > 50% of the brain volume. This is a great overview to anyone that wants to learn more.
Concussions and brain health
The document discusses various topics related to brain health and trauma, including:
- The human brain contains hundreds of billions of neurons and synaptic connections that can process information rapidly.
- Trauma and certain disorders can cause permanent changes to brain structure and function, including conditions like Alzheimer's, Parkinson's, depression, and post-traumatic stress.
- The hippocampus plays a role in many functions including pleasure, rage, emotions, and is involved in mood disorders like depression and bipolar disorder. It has been found to be smaller in people with these conditions.
- Repeated head injuries increase the risk of chronic traumatic encephalopathy, while injuries to the upper neck can cause symptoms of multiple sclerosis and Parkinson's disease
Alzheimers disease
The document discusses the etiopathogenesis, assessment, diagnosis, management and recent advances in Alzheimer's disease. It provides details on the history, epidemiology, risk factors, pathophysiology involving amyloid plaques and neurofibrillary tangles, assessment tools including cognitive tests, and the involvement of genetics. The management focuses on pharmacological and non-pharmacological approaches to address the cognitive and behavioral symptoms of Alzheimer's disease.
Brighten your brain & optimize your mind and body for peak performance
This is the lecture presented by Dr. Cady at SWIRCA - (Southwestern Indiana Regional Council on Aging) on May 7, 2013.
ageing final.pptx
The document discusses normal aging and provides definitions and theories related to the aging process. It addresses topics like the mechanisms of aging, theories of aging, hallmarks of aging, and changes that occur in the brain with aging. It also discusses mental health and personality in older adults, coping strategies, and factors involved in successful aging. The document provides an overview of many aspects of the normal aging process from a biological, psychological, and social perspective.
ROLE OF TELOMERE AND IN CELL AGEING
Telomeres play an important role in cellular aging. Telomeres are nucleoprotein structures located at the end of chromosomes that protect chromosomal DNA from deterioration. With each cell division, telomeres shorten due to the end-replication problem. When telomeres become too short, cells enter a permanent non-dividing state called senescence. Telomere shortening has thus been proposed as a molecular clock that counts the number of cell divisions and limits the replicative capacity of cells, contributing to aging at the cellular and organismal level. Telomerase is an enzyme that can add telomeric repeats to chromosome ends and counteract telomere shortening. Dysfunctional telomeres and telomerase
Thesis defense
The study examined differences in brain activity and its relationship to depressive symptoms between adolescent boys and girls. It found that boys and girls showed opposite patterns of activation in the posterior cingulate cortex (PCC) and dorsolateral prefrontal cortex (dlPFC) that correlated with their reported depressive symptoms and difficulties with emotion regulation. Specifically, boys showed increased activation in these areas associated with higher symptoms, while girls showed decreased activation. This suggests a potential neurobiological mechanism underlying differences in depression risk between adolescent males and females.
How and Why Pesticides Affect Our Health
Dr. Myrto Ashe, MD, MPH presents scientific evidence that pesticides affect the health of our children. She explains the mechanism of how pesticides disrupt the health of children.
Recent advances in the treatment of psychoses
This presentation deals with atypical antipsychotics & new drugs in the pipeline (Clozapine, Risperidone, Olanzapine, Quetiapine, Ziprasidone, Aripiprazole, Second generation antipsychotics, atypical antipsychotics, Aspirin, Minocycline, Raloxifene, Estrogen, N-acetylcysteine). Introduction
Major psychiatric disorders Psychoses & affective disorders
Psychoses Disorders in which patients exhibit gross disturbances in their comprehension of reality as evidenced by false perception (hallucinations), false beliefs (delusions) and loss of contact with reality; schizophrenia most common form of psychosis (+ve n –ve symptoms)
Mostly concerned Abt –ve because poor prognosis , more difficult to treat, persist after positive symptoms have resolved
Mesolimbic Dopamine travels from the midbrain tegmental area to the nucleus accumbens. Increased activity in this pathway may cause delusions, hallucinations, and other so-called positive symptoms of schizophrenia.
Mesocortical pathways Decreased activity in the pathway that goes from the midbrain to the prefrontal lobe cortex can cause apathy, withdrawal, lack of motivation and pleasure, and other so-called negative symptoms of schizophrenia. Mesocortical dysfunction also disinhibits the mesolimbic pathway.
Nigrostriatal pathway from the substantia nigra to the striatum is involved in the coordination of body movements. Inhibition of this pathway causes the extrapyramidal side effects of antipsychotic drugs.
Tuberoinfundibular pathway from the hypothalamus to the pituitary inhibits the release of prolactin. Inhibition of this pathway leads to elevated serum prolactin levels.
2nd generation antipsychotics Clozapine, Risperidone, Olanazapine, Quetiapine, Ziprasidone, Aripiprazole (MOA, Dose, Brand name & A/E and receptor affinities)
Why do 1st gen. antipsychotics cause EPS & 2nd gen. do not? “Hit and run” hypothesis
Inflammation & schizophrenia
New drugs in pipeline Aspirin, Minocycline, Raloxifene, Estrogen, N-acetylcysteine (MOA and rationale of use)
Potential future targets of schizophrenia
Luisetto m et al (2017) immune shock chronologic event in some brain patho...
Luisetto m et al (2017) immune shock chronologic event in some brain patho...M. Luisetto Pharm.D.Spec. Pharmacology
Elevated levels of the cytokine interleukin-6 (IL-6) have been found in the brains and cerebrospinal fluid of individuals with autism spectrum disorder. A review of literature found that IL-6 plays an important role in central nervous system development and is normally expressed at low levels, but chronic over-expression of IL-6 in mice causes neurological alterations. Studies also found IL-6 was significantly increased in the cerebellum of autistic patients compared to controls. Additionally, over-expressing IL-6 in cerebellar granule cells in vitro impaired cell adhesion and migration and stimulated excessive formation of excitatory synapses, suggesting elevated IL-6 may contribute to autism pathogenesis by disrupting neuralRylan
This document proposes developing a novel PET radioligand to detect amyloid beta oligomers for early diagnosis of Alzheimer's disease. It describes 3 aims: 1) Develop and characterize radioligands to detect oligomers in transgenic rat models via microPET scans. 2) Determine the relationship between oligomer binding and AD pathology in rats. 3) Develop an algorithm to identify at-risk individuals and conduct toxicity screening and early human PET trials. The goals are to facilitate early diagnosis before symptom onset to allow for more effective treatment development.
ADHD, Autism, Depression, Schizophrenia& Neuroinflammation
Subtitle: The Moral Imperative of Integrative Medicine
This presentation, two hours in length, was delivered to the A4m MMI Audience in their Frontiers of Neurology - Module 3.
The following topics are reviewed:
- ADHD, Autism, Depression, Schizophrenia
- the impact of neuroinflammation on all of these.
- confounding factors and the ways to mitigate them: Omega6/Omega 3 imbalance in the Western diet, MTHFR polymorphism, the use of elemental lithium, the presence of intestinal dysbiosis and the role of gluten/dairy IgG Food allergies.
- pharmacogenomic testing
Alzheimer's, Parkinson's, ALS, MS,
This document discusses common pathologies and prevention strategies for neurodegenerative diseases such as Alzheimer's, Parkinson's, ALS, MS, and Fibromyalgia. It outlines that these diseases share common causes such as viral and bacterial infections, inflammation, poor diet, lack of exercise, and stress. Maintaining a healthy lifestyle and microbiome through diet, exercise, stress management, and laughter can help prevent neurodegeneration.
Long-lasting alterations to DNA methylation and ncRNAs could underlie the eff...
Fetal alcohol spectrum disorders (FASDs) are characterized by life-long changes in gene expression, neurodevelopment and behavior. What mechanisms initiate and maintain these changes are not known, but current research suggests a role for alcohol-induced epigenetic changes. We assessed alterations to adult mouse brain tissue by assaying DNA cytosine methylation and small noncoding RNA (ncRNA) expression, specifically the microRNA (miRNA) and small nucleolar RNA (snoRNA) subtypes. We found long-lasting alterations in DNA methylation as a result of fetal alcohol exposure, specifically in the imprinted regions of the genome harboring ncRNAs and sequences interacting with regulatory proteins. The findings of this study help to expand on the mechanisms behind the long-lasting changes in the brain transcriptome of FASD individuals.
Webinar Link: http://www.youtube.com/watch?v=fzdc0GIdCnA
Crofton McKim Conf Thyroid QSAR Talk 10-17-2008
This document summarizes a presentation about using in vitro methods to study thyroid-mediated central nervous system dysfunction. It discusses the thyroid system's role in development and homeostasis. Targets for disruption include thyroperoxidase, the iodine symporter, deiodinases, thyroid receptors, and hepatic nuclear receptors. In vitro assays exist for many of these targets, including assays measuring the iodine symporter, thyroperoxidase, deiodinases, thyroid receptors using reporter gene cell lines, and assays measuring thyroid hormone-dependent cell proliferation. The goal is to test chemicals in these in vitro assays to generate dose-response data that can be used to populate quantitative structure-activity relationship models.
Etiology of TAU & PLAQUE protein in Alzheimer's Disease
Details of Alzheimer's Disease and Etiology of Protein.
Under the guidance of
Mr. Nilanjan Adhikari
Assistant professor,Department of Pharmacology
P.G INSTITUTE OF MEDICAL SCIENCES
Luisetto m, ahmed y r, behzad ni, farhan a k, ghulam r m. immune shock chron...
Luisetto m, ahmed y r, behzad ni, farhan a k, ghulam r m. immune shock chron...M. Luisetto Pharm.D.Spec. Pharmacology
1. The document reviews literature on the relationship between immune system factors and brain development in autism disorder.
2. It finds that pro-inflammatory cytokines like IL-6 have been associated with autism and can impair neural cell adhesion/migration and synapse formation when overexpressed.
3. The timing of immune system disturbances and brain development may provide insights into autism pathogenesis.Alziemer 1-converted,anti alzheimer drugs and experimental drugs,laboratory t...
#introduction
#factors
#pathophysiology
#treatments
#anti alzheimer drugs
#experimenta drugs
#laboratory tests
Virtual lecture: 25 Must-Know Facts to Harness Neuroplasticity & Technology F...
Slides from the April 21st, 2016 virtual lecture where three well-recognized experts and pioneers–UCLA’s Dr. Bob Bilder, Emotiv’s Tan Le, and SharpBrains’ Alvaro Fernandez–discussed 25 fundamental facts around neuroplasticity, Alzheimer’s prevention, brain training, meditation, neurofeedback, neurogenesis, brain supplements, and more. Available online from anywhere with an Internet connection, this virtual lecture provided participants with the must-know foundation to understand the value and the limitations of emerging brain science and related technologies, empowering them to navigate the growing stream of news articles, research reports and marketing claims.
Similar to senior presentation (20)
Brighten your brain & optimize your mind and body for peak performance
Brighten your brain & optimize your mind and body for peak performance
Luisetto m et al (2017) immune shock chronologic event in some brain patho...
Luisetto m et al (2017) immune shock chronologic event in some brain patho...
ADHD, Autism, Depression, Schizophrenia& Neuroinflammation
ADHD, Autism, Depression, Schizophrenia& Neuroinflammation
Long-lasting alterations to DNA methylation and ncRNAs could underlie the eff...
Long-lasting alterations to DNA methylation and ncRNAs could underlie the eff...
Etiology of TAU & PLAQUE protein in Alzheimer's Disease
Etiology of TAU & PLAQUE protein in Alzheimer's Disease
Luisetto m, ahmed y r, behzad ni, farhan a k, ghulam r m. immune shock chron...
Luisetto m, ahmed y r, behzad ni, farhan a k, ghulam r m. immune shock chron...
Alziemer 1-converted,anti alzheimer drugs and experimental drugs,laboratory t...
Alziemer 1-converted,anti alzheimer drugs and experimental drugs,laboratory t...
Virtual lecture: 25 Must-Know Facts to Harness Neuroplasticity & Technology F...
Virtual lecture: 25 Must-Know Facts to Harness Neuroplasticity & Technology F...
senior presentation
- 1. Alzheimer’s and Prion Disease By: Charles Gillespie
- 2. Thesis • Alzheimer’s Disease is linked to prion disorders and should be investigated as such.
- 3. Audience Psychologists who have an understanding of psychological terms but are less familiar with physiological literature
- 4. Terms I had to look up • SH-SY5Y • Ntera-2 • Conditioned Medium • Amyloid Beta • Beta Secretase • Alpha Secretase • Cytokines • Microglia • Alanine • Valine • Neurofibrillary tangles • Amyloid Precursor Protein • IL-1ß, IL-6, TNF α • sAPPß • Neurite • Neuroblastoma • Western-Blot Analysis • Reverse-transcriptase polymerase reaction • PrPC vs. PrPSc
- 5. Three Groups • Abnormal • Developmental • Physiological
- 6. Abnormal Psychology Alzheimer’s Disease • Memory Impairment • One of more of the following: Aphasia Apraxia Agnosia Disturbance in executive function • Impairment in social or occupational functioning • Deficits do not occur exclusively during the course of a delirium Creutzfeldt-Jakob Disease • Memory Impairment • One of more of the following: Aphasia Apraxia Agnosia Disturbance in executive function • Impairment in social or occupational functioning • Deficits do not occur exclusively during the course of a delirium
- 7. Differences Alzheimer’s Disease • The course is characterized by gradual onset and continuing cognitive decline • The disturbance is not better accounted for by another Axis 1 disorder Creutzfeldt-Jakob Disease • Evidence that disturbance is direct physiological consequence of a general medical condition other than Alzheimer’s or cardiovascular disease. (American Psychiatric Association, 2000)
- 8. Developmental Psychology • Prions are epigenetically linked (Saijo et al., 2013) • Late age of onset of disease suggests epigenetics (Grolleau-Julius, Ray, & Yung, 2009) • Alzheimer’s is genetically related (Wang et al., 2015) • Genetic influence in Alzheimer’s is susceptible to environmental influence (Yildiz, Pekel, Kilic, Tolgay, & Tufan, 2015)
- 9. Physiological Psychology • Shared physiology of CJD and AD Tau Proteins Amyloid Plaques (Rohan, Smetakova, Kukal, Rusina, & Matej, 2015) • Existences of prion precursor in Alzheimer’s (Chrobak & Adamek, 2014) • Can AD precursor develop into a prion?
- 10. Feasibility PrPc (precursor found in AD) as active prion • Amyloid beta peptides and Amyloid precursor protein inserted into healthy cells did not influence stem cell viability • Micro-glia and micro-glia derived neurotoxins (save one) increased tau expression (Lee, McGeer, & McGeer, 2015)
- 11. What this means • This article strongly indicated the micro-glia instead of the prion precursor proteins as the mode of transportation for the disease. • However…
- 12. The flip side • Micro-glia shown to form after amyloid plaques. (Jung, Keppler, Steinbach, Blazquez-Llorca, & Herms, 2015)
- 13. Conclusion • Alzheimer’s and Creutzfeldt-Jakob disease are very similar • Alzheimer's shows many characteristics of a prion disorder • The physiological testing has not yet been conclusive • More testing is necessary for a definitive answer
- 14. Sources • American Psychiatric Association. (2000). Diagnostic and statistical manual of mental disorders (4th ed., text rev.). doi:10.1176/appi.books.9780890423349. • Chrobak, A., & Adamek, D. (2014). New light on prions: Putative role of co-operation of PrPc and Aβ proteins in cognition. Folia Neuropathologica, 52(1), 1-9. doi:10.5114/fn.2014.41739 • Grolleau-Julius, A., Ray, D., & Yung, R. (2009). The Role of Epigenetics in Aging and Autoimmunity. Clinical Reviews in Allergy & Immunology, 39(1), 42-50. doi:10.1007/s12016-009-8169-3. • Jung, C., Keppler, K., Steinbach, S., Blazquez-Llorca, L., & Herms, J. (2015). Fibrillar Amyloid Plaque Formation Precedes Microglial Activation. PLOS ONE, 10(3), 1-10. doi:10.1371/journal.pone.0119768 • Lee, M., Mcgeer, E., & Mcgeer, P. (2015). Activated human microglia stimulate neuroblastoma cells to upregulate production of beta amyloid protein and tau: Implications for Alzheimer's disease pathogenesis. Neurobiology of Aging, 36, 42-52. doi:10.1016/j.neurobiolaging.2014.07.024 • Rohan, Z., Smetakova, M., Kukal, J., Rusina, R., & Matej, R. (2015). Proteinase-activated receptor 2 and disease biomarkers in cerebrospinal fluid in cases with autopsy-confirmed prion diseases and other neurodegenerative diseases. BMC Neurology, 15(50), 1-6. doi:10.1186/s12883-015-0300-x • Saijo, E., Kang, H., Bian, J., Bowling, K., Browning, S., Kim, S., Telling, G. (2013). Epigenetic Dominance of Prion Conformers. PLOS Pathogens, 9(10), 1-12. • Wang, W., Mandel, J., Bouaziz, J., Commenges, D., Nabirotchkine, S., Chumakov, I., Guedj, M. (2015). A Multi- Marker Genetic Association Test Based on the Rasch Model Applied to Alzheimer’s disease. PLOS ONE, 10(9), 1-19. doi:10.1371/journal.pone.0138223 • Yildiz, D., Pekel, N., Kiliç, A., Tolgay, E., & Tufan, F. (2015). Malnutrition is associated with dementia severity and geriatric syndromes in patients with Alzheimer disease. TURKISH JOURNAL OF MEDICAL SCIENCES, 45, 1078-1081. doi:10.3906/sag-1406-76