Materiały źródłowe

• #1 Progressive Supranuclear Palsy (PSP) | National Institute of Neurological Disorders and Stroke

  https://www.ninds.nih.gov/health-information/disorders/progressive-supranuclear-palsy-psp

  Progressive supranuclear palsy (PSP) is a rare neurological disorder that affects body movements, walking and balance, and eye movements. PSP is caused by damage to nerve cells in areas of the brain that control thinking and body movements. […] The exact cause of PSP is unknown, but research suggests that it involves progressive damage to cells in a few specific areas in the brain, mainly in the brain stem. The death of brain cells in one of these areas, the substantia nigra, accounts in part for the motor symptoms that PSP and Parkinson’s have in common. The tau protein deposits that accumulate in nerve cells in PSP appear to spread to different regions of the brain and cause cells to stop working and die. This in turn destroys the neuronal connections that underlie healthy brain function.

• #2 Progressive supranuclear palsy | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/progressive-supranuclear-palsy?content_id=CON-20251943

  Deterioration of cells in the brainstem, cerebral cortex, cerebellum and basal ganglia a cluster of cells deep within your brain is what causes the coordination and movement issues of progressive supranuclear palsy. […] The cause of progressive supranuclear palsy isn’t known. Its symptoms result from the damage of cells in areas of the brain, especially areas that help you control body movements and thinking. […] Researchers have found that the damaged brain cells of people with progressive supranuclear palsy have excess amounts of a protein called tau. Clumps of tau also are found in other brain diseases, such as Alzheimer’s disease. […] Rarely, progressive supranuclear palsy occurs within a family. But a genetic link isn’t clear. Most people with progressive supranuclear palsy haven’t inherited the disorder.

• #3 Progressive Supranuclear Palsy (PSP): Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/6096-progressive-supranuclear-palsy

  Progressive supranuclear palsy (PSP) is a rare condition that affects certain areas of your brain. […] Scientists dont know the exact cause of progressive supranuclear palsy. However, experts know that a protein called tau is involved. Tau is an important protein for brain health. It helps preserve the normal structure of neurons (brain cells). […] When you have PSP, the tau in your brain clumps together (aggregates). The clumps then damage neurons. Researchers have several theories for why this might happen, some of which include: Random genetic mutations. […] An unknown chemical in the air, water or your food that slowly damages certain vulnerable areas of your brain. […] The main risk factor for PSP is age. Your risk for developing PSP increases as you age if youre 60 or older. The condition is slightly more common in males.

• #4 FloridaHealthFinder | Progressive supranuclear palsy | Health Encyclopedia | FloridaHealthFinder

  https://quality.healthfinder.fl.gov/health-encyclopedia/HIE/1/000767

  Progressive supranuclear palsy (PSP) is a movement disorder that occurs from damage to certain nerve cells in the brain. […] The cause of the damage to the brain cells is unknown. […] People with PSP have deposits in brain tissues that look like those found in people with Alzheimer disease. […] The disorder is most often seen in people over 60 years old, and is somewhat more common in men.

• #5 Progressive supranuclear palsy | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/progressive-supranuclear-palsy?content_id=CON-20251943

  Deterioration of cells in the brainstem, cerebral cortex, cerebellum and basal ganglia a cluster of cells deep within your brain is what causes the coordination and movement issues of progressive supranuclear palsy. […] The cause of progressive supranuclear palsy isn’t known. Its symptoms result from the damage of cells in areas of the brain, especially areas that help you control body movements and thinking. […] Researchers have found that the damaged brain cells of people with progressive supranuclear palsy have excess amounts of a protein called tau. Clumps of tau also are found in other brain diseases, such as Alzheimer’s disease. […] Rarely, progressive supranuclear palsy occurs within a family. But a genetic link isn’t clear. Most people with progressive supranuclear palsy haven’t inherited the disorder.

• #6 Cause of supranuclear palsy

  https://www.mayoclinic.org/diseases-conditions/progressive-supranuclear-palsy/multimedia/cause-of-supranuclear-palsy/img-20007737

  Deterioration of cells in the brainstem, cerebral cortex, cerebellum and basal ganglia a cluster of cells deep within your brain is what causes the coordination and movement issues of progressive supranuclear palsy.

• #7 Progressive Supranuclear Palsy

  https://neurosciences.ucsd.edu/centers-programs/movement-disorders/community/disease-overview/psp.html

  There is strong evidence that a protein known as „tau” is abnormally aggregated in multiple types of brain cells (neurons, glia) in the brain leading to gradual malfunctioning and death of these cells; the common term is „tauopathy.” […] The tau aggregation and loss of cells occurs in several areas of the brain to include the subthalamic nucleus, striatum, substantia nigra, pontine tegmentum, oculomotor nucleus, medulla and dentate nucleus. The most specific features for PSP are presence of star-shaped astrocytic tangles that can be seen with light microscopy and that are stained with antibodies to tau (figure). […] PSP is considered a sporadic (not hereditary) disease; however, it has some genetic component. Environmental causes are being investigated, particularly by Dr. Litvan, who leads a multicenter, NIH funded case-control study to determine whether there are occupational and environmental risk factors for PSP.

• #8 Progressive supranuclear palsy | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/progressive-supranuclear-palsy-1?lang=us

  Progressive supranuclear palsy (PSP), also known as the Steele-Richardson-Olszewski syndrome, comprises a group of related tauopathies and is considered a neurodegenerative disease with no efficacious treatment. […] PSP is a 4R-tauopathy in which tau accumulation occurs with astrogliosis and neuron loss.

• #9

  https://www.psp.org/iwanttolearn/progressive-supranuclear-palsy

  Progressive supranuclear palsy (PSP) is a rare neurodegenerative disorder that has no known cause or cure. […] The direct cause of PSP is not fully understood. However, we do know that it has to do with the clumps of a protein called tau. Tau is a normal protein found in brain cells and is currently thought to help maintain the microtubules, which are stiff rods that function as the brain cells internal transportation and skeletal system. In PSP, the tau seems to become abnormally folded, which causes it to stick together and become stuck inside the cell. In brains with PSP, the areas of the brain that have cells with tau inside of them show neuron impairment and neuronal death. The clumps technical name is neurofibrillary tangles. Alzheimers disease and frontotemporal dementia also involve misfolding of the tau protein. […] Genetic differences and exposure to certain chemicals are being explored as possible causes, but there is not currently an answer to why tau misfolds or people get PSP.

• #10 Progressive supranuclear palsy (PSP) | NHS inform

  https://www.nhsinform.scot/illnesses-and-conditions/brain-nerves-and-spinal-cord/progressive-supranuclear-palsy-psp/

  Progressive supranuclear palsy (PSP) is a neurological condition. Its caused by the loss of nerve cells in parts of the brain. […] Its believed that a protein called tau, which builds up in certain areas of the brain, forms into clumps. This causes damage to the nerve cells. Over time this build up can lead to someone experiencing the symptoms of PSP. […] The location of the clumps and the amount of tau varies between people with PSP. This also affects the type and severity of symptoms. […] PSP tends not to run in families and is not believed to be hereditary in most cases.

• #11 Orphanet: Progressive supranuclear palsy

  https://www.orpha.net/en/disease/detail/683

  A rare late-onset neurodegenerative disease characterized by ocular motor dysfunction, postural instability, akinesia-rigidity, and cognitive dysfunction. […] PSP is a 4R tauopathy composed of a preponderance of four-repeat (exon 10 positive) tau isoforms and a characteristic biochemical profile (doublet tau 64 and tau 69). The MAPT H1-clade specific sub-haplotype, H1c, is a risk factor for this disease. PSP is also characterized by deficits in several neurotransmitter systems (e.g., dopaminergic, cholinergic, GABAergic). The factors that initiate tau-neurodegeneration are unknown. […] PSP is a sporadic, non-hereditary disease.

• #12 Clinical Approach to Progressive Supranuclear Palsy

  https://www.e-jmd.org/journal/view.php?doi=10.14802/jmd.15060

  The cause of PSP is unknown. Advanced age is the only established risk factor. To date, head injury has not been established as a risk factor of PSP. A prevalence study found 24% of PSP cases had early histological evidence of chronic traumatic encephalopathy (CTE), a neurodegenerative consequence of repetitive head injury previously referred as dementia pugilistica in boxers. Whether the CTE-tau pathology began following the onset of PSP as a result of frequent falls is not known. Geographical clusters of patients with PSP-like syndrome on Guam and Guadeloupe have probable links to environmental causes. These neurodegenerative tauopathies have clinical features atypical to the classic RS and the Guam-parkinsonian dementia complex is pathologically distinct from PSP. Frontotemporal dementia with parkinsonism due to autosomal dominant mutations in the MAPT gene (FTDP-17T) is clinically and pathologically heterogeneous. Specifically, FTDP-17T due to exon 10 coding or splicing shares the most similarity to PSP, both clinically and pathologically. Although PSP is considered a sporadic condition, FTDP-17T probably provides the best clues as to the etiology of PSP. The strong association between the H1c haplotype and PSP was confirmed by a genome-wide study (GWAS) of PSP which identified the presence of independent association signals at the MAPT locus representing both the H1/H2 haplotypes and the rs242557 MAPT SNP, related to the H1c sub-haplotype. Non-MAPT risk factors associated with PSP, EIF2AK3, MOBP and STX-6, were also found in the PSP GWAS. The protein functions of these candidate genes provide insights to the biochemical basis of the pathophysiological mechanisms. The findings of seeding and spreading of transmissible tau neuropathology in transgenic mouse brains including PSP-tau support the notion of a cell-to-cell propagation mechanism of different strains of fibrillary tau leading to distinct patterns of neuronal and glial pathology which is disease- and neural network-specific.

• #13 Orphanet: Progressive supranuclear palsy

  https://www.orpha.net/en/disease/detail/683

  A rare late-onset neurodegenerative disease characterized by ocular motor dysfunction, postural instability, akinesia-rigidity, and cognitive dysfunction. […] PSP is a 4R tauopathy composed of a preponderance of four-repeat (exon 10 positive) tau isoforms and a characteristic biochemical profile (doublet tau 64 and tau 69). The MAPT H1-clade specific sub-haplotype, H1c, is a risk factor for this disease. PSP is also characterized by deficits in several neurotransmitter systems (e.g., dopaminergic, cholinergic, GABAergic). The factors that initiate tau-neurodegeneration are unknown. […] PSP is a sporadic, non-hereditary disease.

• #14 Environmental Risk Factors for Progressive Supranuclear Palsy

  https://www.e-jmd.org/journal/view.php?number=346

  Typically, progressive supranuclear palsy (PSP) is clinically characterized by vertical slowing of saccades or supranuclear gaze palsy, axial-predominant parkinsonism with poor response to levodopa therapy, bulbar symptoms, and cognitive impairment, specifically, frontal/executive dysfunction. PSP is a primary four-repeat tauopathy that is caused by abnormal accumulation of pathologically altered microtubule-associated protein tau (MAPT) in neurons and glia. […] The identification of modifiable risk factors for PSP may lead to an improved understanding of the pathophysiology and targeted prevention and therapeutic efforts. […] Over the past two decades, tremendous advancements in recognizing environmental and other modifiable risk factors for PSP have been made. Here, we review environmental and modifiable lifestyle risk factors for PSP, including dietary habits, residential areas, education, metal/chemical exposure, hypertension, and drugs.

• #15 Progressive supranuclear palsy (PSP) | NHS inform

  https://www.nhsinform.scot/illnesses-and-conditions/brain-nerves-and-spinal-cord/progressive-supranuclear-palsy-psp/

  Progressive supranuclear palsy (PSP) is a neurological condition. Its caused by the loss of nerve cells in parts of the brain. […] Its believed that a protein called tau, which builds up in certain areas of the brain, forms into clumps. This causes damage to the nerve cells. Over time this build up can lead to someone experiencing the symptoms of PSP. […] The location of the clumps and the amount of tau varies between people with PSP. This also affects the type and severity of symptoms. […] PSP tends not to run in families and is not believed to be hereditary in most cases.

• #16 Progressive supranuclear palsy – Wikipedia

  https://en.wikipedia.org/wiki/Progressive_supranuclear_palsy

  Progressive supranuclear palsy (PSP) is a late-onset neurodegenerative disease involving the gradual deterioration and death of specific volumes of the brain. […] The cause of the condition is uncertain, but involves the accumulation of tau protein within the brain. […] The cause of PSP is unknown. Fewer than 1% of those with PSP have a family member with the same disorder. A variant in the gene for tau protein called the H1 haplotype, located on chromosome 17 (rs1800547), has been linked to PSP. […] Other genes, as well as environmental toxins, are being investigated as other possible contributors to the cause of PSP. […] Besides tauopathy, mitochondrial dysfunction seems to be a factor involved in PSP.

• #17

  https://www.brain.northwestern.edu/dementia/psps/symptoms-causes.html

  PSPS can be caused by a number of neurodegenerative diseases. […] Most cases of PSP are sporadic, which means there is no one specific genetic mutation causing the disease. […] In the exceedingly rare genetic form of PSP, disease is transmitted from one generation to the next by a mutation in the MAPT gene, which provide the brain with the instructions for making the protein called tau.

• #18 Progressive supranuclear palsy: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/progressive-supranuclear-palsy/

  Progressive supranuclear palsy is a brain disorder that affects movement, vision, speech, and thinking ability (cognition). […] In most cases, the genetic cause of progressive supranuclear palsy is unknown. Rarely, the disease results from mutations in the MAPT gene. Certain normal variations (polymorphisms) in the MAPT gene have also been associated with an increased risk of developing progressive supranuclear palsy. […] The signs and symptoms of progressive supranuclear palsy appear to be related to abnormalities in the tau protein. In people with MAPT gene mutations, genetic changes disrupt the protein’s normal structure and function. However, abnormal tau is also found in affected individuals without MAPT gene mutations. […] Researchers suspect that other genetic and environmental factors also contribute to progressive supranuclear palsy. For example, the disease has been linked to genetic changes on chromosome 1 and chromosome 11. However, the specific genes involved have not been identified.

• #19 Progressive supranuclear palsy | Monash Health

  https://monashhealth.org/services/movement-disorders-program/understanding-movement-disorders/progressive-supranuclear-palsy/

  Progressive supranuclear palsy (PSP) is a rare and progressive neurodegenerative disorder. […] The deterioration in brain cells in several areas of the brain leads to the symptoms found in PSP. In PSP there is an abnormal accumulation of the protein called tau in the brain. […] The cause of the tau clumping is not known. […] The cause of the brain cell degeneration is unknown. […] To date genetics has not been found to be the cause of most cases of PSP. In rare cases, the mutation of the MAPT gene is said to have caused PSP. […] No environmental or infective factors have been linked to PSP to date.

• #20 Progressive supranuclear palsy – Wikipedia

  https://en.wikipedia.org/wiki/Progressive_supranuclear_palsy

  Progressive supranuclear palsy (PSP) is a late-onset neurodegenerative disease involving the gradual deterioration and death of specific volumes of the brain. […] The cause of the condition is uncertain, but involves the accumulation of tau protein within the brain. […] The cause of PSP is unknown. Fewer than 1% of those with PSP have a family member with the same disorder. A variant in the gene for tau protein called the H1 haplotype, located on chromosome 17 (rs1800547), has been linked to PSP. […] Other genes, as well as environmental toxins, are being investigated as other possible contributors to the cause of PSP. […] Besides tauopathy, mitochondrial dysfunction seems to be a factor involved in PSP.

• #21 Progressive Supranuclear Palsy – EyeWiki

  https://eyewiki.org/Progressive_Supranuclear_Palsy

  Although most cases of PSP are sporadic, there have been reports of familial aggregation. Among these groups with familial PSP, a stronger association with the MAPT H1 haplotype was noted when compared to controls and patients with sporadic PSP. MAPT encodes the Tau protein and genetic mutations in this gene are common in tauopathies. The familial cases of PSP were confirmed by autopsy adding to the validity of the study. However, the presence of the MAPT H1 haplotype does not definitively predict development of PSP and can only be regarded as a mild predisposition for it. […] The major pathways affected by the pathology of PSP are the dopaminergic nigrostriatal, the GABAergic, cholinoceptive striatal neurons, and the cholinergic brainstem and basal forebrain nuclei.

• #22 Clinical Approach to Progressive Supranuclear Palsy

  https://www.e-jmd.org/journal/view.php?doi=10.14802/jmd.15060

  The cause of PSP is unknown. Advanced age is the only established risk factor. To date, head injury has not been established as a risk factor of PSP. A prevalence study found 24% of PSP cases had early histological evidence of chronic traumatic encephalopathy (CTE), a neurodegenerative consequence of repetitive head injury previously referred as dementia pugilistica in boxers. Whether the CTE-tau pathology began following the onset of PSP as a result of frequent falls is not known. Geographical clusters of patients with PSP-like syndrome on Guam and Guadeloupe have probable links to environmental causes. These neurodegenerative tauopathies have clinical features atypical to the classic RS and the Guam-parkinsonian dementia complex is pathologically distinct from PSP. Frontotemporal dementia with parkinsonism due to autosomal dominant mutations in the MAPT gene (FTDP-17T) is clinically and pathologically heterogeneous. Specifically, FTDP-17T due to exon 10 coding or splicing shares the most similarity to PSP, both clinically and pathologically. Although PSP is considered a sporadic condition, FTDP-17T probably provides the best clues as to the etiology of PSP. The strong association between the H1c haplotype and PSP was confirmed by a genome-wide study (GWAS) of PSP which identified the presence of independent association signals at the MAPT locus representing both the H1/H2 haplotypes and the rs242557 MAPT SNP, related to the H1c sub-haplotype. Non-MAPT risk factors associated with PSP, EIF2AK3, MOBP and STX-6, were also found in the PSP GWAS. The protein functions of these candidate genes provide insights to the biochemical basis of the pathophysiological mechanisms. The findings of seeding and spreading of transmissible tau neuropathology in transgenic mouse brains including PSP-tau support the notion of a cell-to-cell propagation mechanism of different strains of fibrillary tau leading to distinct patterns of neuronal and glial pathology which is disease- and neural network-specific.

• #23 Orphanet: Progressive supranuclear palsy

  https://www.orpha.net/en/disease/detail/683

  A rare late-onset neurodegenerative disease characterized by ocular motor dysfunction, postural instability, akinesia-rigidity, and cognitive dysfunction. […] PSP is a 4R tauopathy composed of a preponderance of four-repeat (exon 10 positive) tau isoforms and a characteristic biochemical profile (doublet tau 64 and tau 69). The MAPT H1-clade specific sub-haplotype, H1c, is a risk factor for this disease. PSP is also characterized by deficits in several neurotransmitter systems (e.g., dopaminergic, cholinergic, GABAergic). The factors that initiate tau-neurodegeneration are unknown. […] PSP is a sporadic, non-hereditary disease.

• #24 Clinical Approach to Progressive Supranuclear Palsy

  https://www.e-jmd.org/journal/view.php?doi=10.14802/jmd.15060

  The cause of PSP is unknown. Advanced age is the only established risk factor. To date, head injury has not been established as a risk factor of PSP. A prevalence study found 24% of PSP cases had early histological evidence of chronic traumatic encephalopathy (CTE), a neurodegenerative consequence of repetitive head injury previously referred as dementia pugilistica in boxers. Whether the CTE-tau pathology began following the onset of PSP as a result of frequent falls is not known. Geographical clusters of patients with PSP-like syndrome on Guam and Guadeloupe have probable links to environmental causes. These neurodegenerative tauopathies have clinical features atypical to the classic RS and the Guam-parkinsonian dementia complex is pathologically distinct from PSP. Frontotemporal dementia with parkinsonism due to autosomal dominant mutations in the MAPT gene (FTDP-17T) is clinically and pathologically heterogeneous. Specifically, FTDP-17T due to exon 10 coding or splicing shares the most similarity to PSP, both clinically and pathologically. Although PSP is considered a sporadic condition, FTDP-17T probably provides the best clues as to the etiology of PSP. The strong association between the H1c haplotype and PSP was confirmed by a genome-wide study (GWAS) of PSP which identified the presence of independent association signals at the MAPT locus representing both the H1/H2 haplotypes and the rs242557 MAPT SNP, related to the H1c sub-haplotype. Non-MAPT risk factors associated with PSP, EIF2AK3, MOBP and STX-6, were also found in the PSP GWAS. The protein functions of these candidate genes provide insights to the biochemical basis of the pathophysiological mechanisms. The findings of seeding and spreading of transmissible tau neuropathology in transgenic mouse brains including PSP-tau support the notion of a cell-to-cell propagation mechanism of different strains of fibrillary tau leading to distinct patterns of neuronal and glial pathology which is disease- and neural network-specific.

• #25 Progressive supranuclear palsy: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/progressive-supranuclear-palsy/

  Progressive supranuclear palsy is a brain disorder that affects movement, vision, speech, and thinking ability (cognition). […] In most cases, the genetic cause of progressive supranuclear palsy is unknown. Rarely, the disease results from mutations in the MAPT gene. Certain normal variations (polymorphisms) in the MAPT gene have also been associated with an increased risk of developing progressive supranuclear palsy. […] The signs and symptoms of progressive supranuclear palsy appear to be related to abnormalities in the tau protein. In people with MAPT gene mutations, genetic changes disrupt the protein’s normal structure and function. However, abnormal tau is also found in affected individuals without MAPT gene mutations. […] Researchers suspect that other genetic and environmental factors also contribute to progressive supranuclear palsy. For example, the disease has been linked to genetic changes on chromosome 1 and chromosome 11. However, the specific genes involved have not been identified.

• #26 Progressive Supranuclear Palsy – EyeWiki

  https://eyewiki.org/Progressive_Supranuclear_Palsy

  Although most cases of PSP are sporadic, there have been reports of familial aggregation. Among these groups with familial PSP, a stronger association with the MAPT H1 haplotype was noted when compared to controls and patients with sporadic PSP. MAPT encodes the Tau protein and genetic mutations in this gene are common in tauopathies. The familial cases of PSP were confirmed by autopsy adding to the validity of the study. However, the presence of the MAPT H1 haplotype does not definitively predict development of PSP and can only be regarded as a mild predisposition for it. […] The major pathways affected by the pathology of PSP are the dopaminergic nigrostriatal, the GABAergic, cholinoceptive striatal neurons, and the cholinergic brainstem and basal forebrain nuclei.

• #27 Clinical Approach to Progressive Supranuclear Palsy

  https://www.e-jmd.org/journal/view.php?doi=10.14802/jmd.15060

  The cause of PSP is unknown. Advanced age is the only established risk factor. To date, head injury has not been established as a risk factor of PSP. A prevalence study found 24% of PSP cases had early histological evidence of chronic traumatic encephalopathy (CTE), a neurodegenerative consequence of repetitive head injury previously referred as dementia pugilistica in boxers. Whether the CTE-tau pathology began following the onset of PSP as a result of frequent falls is not known. Geographical clusters of patients with PSP-like syndrome on Guam and Guadeloupe have probable links to environmental causes. These neurodegenerative tauopathies have clinical features atypical to the classic RS and the Guam-parkinsonian dementia complex is pathologically distinct from PSP. Frontotemporal dementia with parkinsonism due to autosomal dominant mutations in the MAPT gene (FTDP-17T) is clinically and pathologically heterogeneous. Specifically, FTDP-17T due to exon 10 coding or splicing shares the most similarity to PSP, both clinically and pathologically. Although PSP is considered a sporadic condition, FTDP-17T probably provides the best clues as to the etiology of PSP. The strong association between the H1c haplotype and PSP was confirmed by a genome-wide study (GWAS) of PSP which identified the presence of independent association signals at the MAPT locus representing both the H1/H2 haplotypes and the rs242557 MAPT SNP, related to the H1c sub-haplotype. Non-MAPT risk factors associated with PSP, EIF2AK3, MOBP and STX-6, were also found in the PSP GWAS. The protein functions of these candidate genes provide insights to the biochemical basis of the pathophysiological mechanisms. The findings of seeding and spreading of transmissible tau neuropathology in transgenic mouse brains including PSP-tau support the notion of a cell-to-cell propagation mechanism of different strains of fibrillary tau leading to distinct patterns of neuronal and glial pathology which is disease- and neural network-specific.

• #28 Progressive Supranuclear Palsy – EyeWiki

  https://eyewiki.org/Progressive_Supranuclear_Palsy

  Although most cases of PSP are sporadic, there have been reports of familial aggregation. Among these groups with familial PSP, a stronger association with the MAPT H1 haplotype was noted when compared to controls and patients with sporadic PSP. MAPT encodes the Tau protein and genetic mutations in this gene are common in tauopathies. The familial cases of PSP were confirmed by autopsy adding to the validity of the study. However, the presence of the MAPT H1 haplotype does not definitively predict development of PSP and can only be regarded as a mild predisposition for it. […] The major pathways affected by the pathology of PSP are the dopaminergic nigrostriatal, the GABAergic, cholinoceptive striatal neurons, and the cholinergic brainstem and basal forebrain nuclei.

• #29

  https://www.nhs.uk/conditions/progressive-supranuclear-palsy-psp/

  Progressive supranuclear palsy (PSP) is a rare neurological condition that can cause problems with balance, movement, vision, speech and swallowing. […] It’s caused by increasing numbers of brain cells becoming damaged over time. […] PSP occurs when brain cells in certain parts of the brain are damaged as a result of a build-up of a protein called tau. […] In people with PSP, it isn’t broken down properly and forms harmful clumps in brain cells. […] The condition has been linked to changes in certain genes, but these genetic faults aren’t inherited and the risk to other family members, including the children or siblings of someone with PSP, is very low.

• #30 Progressive supranuclear palsy (PSP) | nidirect

  https://www.nidirect.gov.uk/conditions/progressive-supranuclear-palsy-psp

  PSP occurs when brain cells in certain parts of the brain are damaged as a result of a build-up of a protein called tau. […] In people with PSP, it isn’t broken down properly and forms harmful clumps in brain cells. […] The condition has been linked to changes in certain genes, but these genetic faults are not inherited and the risk to other family members, including children or siblings of someone with PSP, is low.

• #31 Progressive Supranuclear Palsy (PSP) | National Institute of Neurological Disorders and Stroke

  https://www.ninds.nih.gov/health-information/disorders/progressive-supranuclear-palsy-psp

  PSP is usually sporadic, meaning that it occurs randomly without a known cause. In very few cases, the disease results from mutations in the MAPT gene. This mutation provides faulty instructions for making tau in brain cells. For most people with PSP, no known genetic factor is found. […] Several theories suggest that PSP might be caused by: Random genetic changes that occur in particular cells or genes in a specific combination that injures these cells. Exposure to some unknown environmental factor (such as chemicals in food, air, or water), which slowly damages certain vulnerable areas of the brain. Cellular damage caused by free radicals, which are reactive molecules produced continuously by all cells during normal metabolism. Although the body has built-in ways to clear free radicals from the body, scientists suspect that under certain circumstances free radicals can react with and damage other molecules.

• #32 Environmental Risk Factors for Progressive Supranuclear Palsy

  https://www.e-jmd.org/journal/view.php?number=346

  Typically, progressive supranuclear palsy (PSP) is clinically characterized by vertical slowing of saccades or supranuclear gaze palsy, axial-predominant parkinsonism with poor response to levodopa therapy, bulbar symptoms, and cognitive impairment, specifically, frontal/executive dysfunction. PSP is a primary four-repeat tauopathy that is caused by abnormal accumulation of pathologically altered microtubule-associated protein tau (MAPT) in neurons and glia. […] The identification of modifiable risk factors for PSP may lead to an improved understanding of the pathophysiology and targeted prevention and therapeutic efforts. […] Over the past two decades, tremendous advancements in recognizing environmental and other modifiable risk factors for PSP have been made. Here, we review environmental and modifiable lifestyle risk factors for PSP, including dietary habits, residential areas, education, metal/chemical exposure, hypertension, and drugs.

• #33 Progressive Supranuclear Palsy – EyeWiki

  https://eyewiki.org/Progressive_Supranuclear_Palsy

  Progressive supranuclear palsy (PSP) is a sporadic disease and the exact cause is not known. Potential hypotheses include: […] There have been three geographical clusters of illnesses similar to PSP in Guam, Guadeloupe, and northern France. The first two locations revealed a strong association with PSP and cycad neurotoxins in Guam, and consumption of traditional medicines containing annonacin in Guadeloupe. However, the cluster in France is the most compelling case for environmental etiology, because the cases in Guam and Guadeloupe were not classic cases of PSP. The study in northern France found an increased prevalence of arsenic in the soil of chemical plants near the towns with increases in classic PSP diagnosis. […] There are 10 different MAPT mutations that have been identified in PSP patients including the R5L mutation in exon 1. The remaining variants are in exon 10 and its splicing region. These genetic abnormalities lead to neurofibrillary tangles and are currently under investigation as causative agents.

• #34 Heavy metals contaminating the environment of a progressive supranuclear palsy cluster induce tau accumulation and cell death in cultured neurons | Scientific Reports

  https://www.nature.com/articles/s41598-019-56930-w

  Progressive supranuclear palsy (PSP) is a neurodegenerative disorder characterized by the presence of intracellular aggregates of tau protein and neuronal loss leading to cognitive and motor impairment. […] Although the etiopathology of PSP is unknown, mutations in the MAPT/tau gene and exposure to environmental toxins can increase the risk of PSP. […] The mechanisms underlying tau pathology and neuronal death in PSP are largely unknown. […] It has also been reported that the exposure to environmental toxins increases the risk of sporadic PSP. […] Therefore, although molecular genetic analysis had not been performed, the Wattrelos cluster seemed unlikely to be due to hereditary or genetic causes. Rather, the authors speculated that the presence of the PSP cluster could be related to environmental exposure to those heavy metals from improper disposal of industrial waste in residential areas.

• #35 Heavy metals contaminating the environment of a progressive supranuclear palsy cluster induce tau accumulation and cell death in cultured neurons | Scientific Reports

  https://www.nature.com/articles/s41598-019-56930-w

  The chronic exposure to Cr, Ni and Cd is toxic for humans and has been associated with different neurodegenerative diseases, including tauopathies such as Alzheimers disease. […] Together, these results suggest that Cr and Ni exposure, but not other heavy metals such as Al, could be potentially associated with the sporadic PSP cluster found in Wattrelos and support previous studies suggesting that Al and Cd exposure is associated with some neurodegenerative diseases such as AD, but not with PSP. […] Our results showed that the exposure to three heavy metal (Cr, Ni and Cd) contaminating a geographic region associated with a cluster of sporadic PSP patients induced dose-dependent neurotoxicity in vitro. […] Additionally, we showed that neurotoxic doses of Cr and Ni increased the levels and phosphorylation status of tau protein in vitro, linking heavy metal exposure with the development of tauopathies such as PSP.

• #36 Environmental Risk Factors for Progressive Supranuclear Palsy

  https://www.e-jmd.org/journal/view.php?number=346

  Chronic exposure to the Annonaceae family, such as Annona muricata (corossol, soursop, guanabana and graviola), Annona squamosa (pomme cannelle, sweepsop, sugar apple, and cherimoya), and Annona reticulata (cachiman, custard-apple and mamon), was reported to be associated with an increased risk of developing PSP-like features. […] Annonacin may play a role in the pathomechanism as a mitochondrial inhibitor and a toxin to induce tau pathology and neurodegeneration. […] Metal exposure is associated with PSP. […] Observational and case-control studies suggested that hypertension is associated with PSP. […] The use of beta blockers showed an association with PSP, while other drugs, such as calcium channel blockers, diuretics, and angiotensin-converting enzyme inhibitors/angiotensin receptor blockers, did not show any association with PSP in the univariate analysis of the ENGENE-PSP study.

• #37 Progressive Supranuclear Palsy: Causes, Symptoms, Diagnosis And Treatment

  https://www.netmeds.com/health-library/post/progressive-supranuclear-palsy-causes-symptoms-diagnosis-and-treatment?srsltid=AfmBOop1XJvF1f6AfnKma7BpOon8ZoWrWmVgQQUNHgEq_6kp0DpqDCbb

  The cause of progressive supranuclear palsy is unknown but most likely the disease occurs when brain cells in certain parts of the brain are damaged because of a build-up of a protein called tau. […] Abnormalities in tau protein lead to damage in both cortical (the outer layer of the cerebrum) as well as subcortical areas of the brain. […] The reason for this intrinsically disordered protein malfunction can be an infectious agent, a random genetic mutation, or a chemical present in the food, air, or water. […] A common risk factor for this illness is generally identified as age. […] Moreover, environmental factors such as air pollution and pesticides and industrial solvents could increase the risk of this neurological, degenerative disorder.

• #38 Progressive Supranuclear Palsy | Causes and Signs | CogniFit

  https://yourbrain.health/progressive-supranuclear-palsy/

  Another line of thinking is that PSP and other neurological diseases may be triggered by continued exposure to environmental toxins, such as carbon monoxide or pesticides. […] Some evidence supports this theory. […] For example, researchers in France identified a high number of PSP cases in an industrial town. […] Additionally, scientists spent many years researching neurological diseases on the Pacific Island of Guam.

• #39 Environmental Risk Factors for Progressive Supranuclear Palsy

  https://www.e-jmd.org/journal/view.php?number=346

  Chronic exposure to the Annonaceae family, such as Annona muricata (corossol, soursop, guanabana and graviola), Annona squamosa (pomme cannelle, sweepsop, sugar apple, and cherimoya), and Annona reticulata (cachiman, custard-apple and mamon), was reported to be associated with an increased risk of developing PSP-like features. […] Annonacin may play a role in the pathomechanism as a mitochondrial inhibitor and a toxin to induce tau pathology and neurodegeneration. […] Metal exposure is associated with PSP. […] Observational and case-control studies suggested that hypertension is associated with PSP. […] The use of beta blockers showed an association with PSP, while other drugs, such as calcium channel blockers, diuretics, and angiotensin-converting enzyme inhibitors/angiotensin receptor blockers, did not show any association with PSP in the univariate analysis of the ENGENE-PSP study.

• #40 Environmental Risk Factors for Progressive Supranuclear Palsy

  https://www.e-jmd.org/journal/view.php?number=346

  Chronic exposure to the Annonaceae family, such as Annona muricata (corossol, soursop, guanabana and graviola), Annona squamosa (pomme cannelle, sweepsop, sugar apple, and cherimoya), and Annona reticulata (cachiman, custard-apple and mamon), was reported to be associated with an increased risk of developing PSP-like features. […] Annonacin may play a role in the pathomechanism as a mitochondrial inhibitor and a toxin to induce tau pathology and neurodegeneration. […] Metal exposure is associated with PSP. […] Observational and case-control studies suggested that hypertension is associated with PSP. […] The use of beta blockers showed an association with PSP, while other drugs, such as calcium channel blockers, diuretics, and angiotensin-converting enzyme inhibitors/angiotensin receptor blockers, did not show any association with PSP in the univariate analysis of the ENGENE-PSP study.

• #41 Environmental Risk Factors for Progressive Supranuclear Palsy

  https://www.e-jmd.org/journal/view.php?number=346

  Repeated stressful events may increase the risk for PSP. […] This extensive literature review about environmental and modifiable factors in PSP shows that specific dietary habits, such as high consumption of Annonaceae family fruits, metal/chemical exposure, hypertension, and stress, are risk factors for PSP. High educational level and statin use may be protective.

• #42 Environmental Risk Factors for Progressive Supranuclear Palsy

  https://www.e-jmd.org/journal/view.php?number=346

  Repeated stressful events may increase the risk for PSP. […] This extensive literature review about environmental and modifiable factors in PSP shows that specific dietary habits, such as high consumption of Annonaceae family fruits, metal/chemical exposure, hypertension, and stress, are risk factors for PSP. High educational level and statin use may be protective.

• #43 Progressive Supranuclear Palsy – EyeWiki

  https://eyewiki.org/Progressive_Supranuclear_Palsy

  Progressive supranuclear palsy (PSP) is a sporadic disease and the exact cause is not known. Potential hypotheses include: […] There have been three geographical clusters of illnesses similar to PSP in Guam, Guadeloupe, and northern France. The first two locations revealed a strong association with PSP and cycad neurotoxins in Guam, and consumption of traditional medicines containing annonacin in Guadeloupe. However, the cluster in France is the most compelling case for environmental etiology, because the cases in Guam and Guadeloupe were not classic cases of PSP. The study in northern France found an increased prevalence of arsenic in the soil of chemical plants near the towns with increases in classic PSP diagnosis. […] There are 10 different MAPT mutations that have been identified in PSP patients including the R5L mutation in exon 1. The remaining variants are in exon 10 and its splicing region. These genetic abnormalities lead to neurofibrillary tangles and are currently under investigation as causative agents.

• #44 Clinical Approach to Progressive Supranuclear Palsy

  https://www.e-jmd.org/journal/view.php?doi=10.14802/jmd.15060

  The cause of PSP is unknown. Advanced age is the only established risk factor. To date, head injury has not been established as a risk factor of PSP. A prevalence study found 24% of PSP cases had early histological evidence of chronic traumatic encephalopathy (CTE), a neurodegenerative consequence of repetitive head injury previously referred as dementia pugilistica in boxers. Whether the CTE-tau pathology began following the onset of PSP as a result of frequent falls is not known. Geographical clusters of patients with PSP-like syndrome on Guam and Guadeloupe have probable links to environmental causes. These neurodegenerative tauopathies have clinical features atypical to the classic RS and the Guam-parkinsonian dementia complex is pathologically distinct from PSP. Frontotemporal dementia with parkinsonism due to autosomal dominant mutations in the MAPT gene (FTDP-17T) is clinically and pathologically heterogeneous. Specifically, FTDP-17T due to exon 10 coding or splicing shares the most similarity to PSP, both clinically and pathologically. Although PSP is considered a sporadic condition, FTDP-17T probably provides the best clues as to the etiology of PSP. The strong association between the H1c haplotype and PSP was confirmed by a genome-wide study (GWAS) of PSP which identified the presence of independent association signals at the MAPT locus representing both the H1/H2 haplotypes and the rs242557 MAPT SNP, related to the H1c sub-haplotype. Non-MAPT risk factors associated with PSP, EIF2AK3, MOBP and STX-6, were also found in the PSP GWAS. The protein functions of these candidate genes provide insights to the biochemical basis of the pathophysiological mechanisms. The findings of seeding and spreading of transmissible tau neuropathology in transgenic mouse brains including PSP-tau support the notion of a cell-to-cell propagation mechanism of different strains of fibrillary tau leading to distinct patterns of neuronal and glial pathology which is disease- and neural network-specific.

• #45 Progressive Supranuclear Palsy – EyeWiki

  https://eyewiki.org/Progressive_Supranuclear_Palsy

  Progressive supranuclear palsy (PSP) is a sporadic disease and the exact cause is not known. Potential hypotheses include: […] There have been three geographical clusters of illnesses similar to PSP in Guam, Guadeloupe, and northern France. The first two locations revealed a strong association with PSP and cycad neurotoxins in Guam, and consumption of traditional medicines containing annonacin in Guadeloupe. However, the cluster in France is the most compelling case for environmental etiology, because the cases in Guam and Guadeloupe were not classic cases of PSP. The study in northern France found an increased prevalence of arsenic in the soil of chemical plants near the towns with increases in classic PSP diagnosis. […] There are 10 different MAPT mutations that have been identified in PSP patients including the R5L mutation in exon 1. The remaining variants are in exon 10 and its splicing region. These genetic abnormalities lead to neurofibrillary tangles and are currently under investigation as causative agents.

• #46 Progressive Supranuclear Palsy – EyeWiki

  https://eyewiki.org/Progressive_Supranuclear_Palsy

  Progressive supranuclear palsy (PSP) is a sporadic disease and the exact cause is not known. Potential hypotheses include: […] There have been three geographical clusters of illnesses similar to PSP in Guam, Guadeloupe, and northern France. The first two locations revealed a strong association with PSP and cycad neurotoxins in Guam, and consumption of traditional medicines containing annonacin in Guadeloupe. However, the cluster in France is the most compelling case for environmental etiology, because the cases in Guam and Guadeloupe were not classic cases of PSP. The study in northern France found an increased prevalence of arsenic in the soil of chemical plants near the towns with increases in classic PSP diagnosis. […] There are 10 different MAPT mutations that have been identified in PSP patients including the R5L mutation in exon 1. The remaining variants are in exon 10 and its splicing region. These genetic abnormalities lead to neurofibrillary tangles and are currently under investigation as causative agents.

• #47 What Causes PSP?

  https://www.triadfamilyhealth.com/conditions/progressive-supranuclear-palsy-psp/what-causes-psp

  There are, however, several theories about PSP’s cause. One possibility is that an unconventional virus-like agent infects the body and takes years or decades to start producing visible effects. Another possibility is that random genetic mutations, of the kind that occur in all of us all the time, happen to occur in particular cells or certain genes, in just the right combination to injure these cells. A third possibility is that there is exposure to some unknown chemical in the food, air, or water which slowly damages certain vulnerable areas of the brain. This theory stems from a clue found on the Pacific island of Guam, where a common neurological disease occurring only there and on a few neighboring islands shares some of the characteristics of PSP, Alzheimer’s disease, Parkinson’s disease, and amyotrophic lateral sclerosis (Lou Gehrig’s disease). Its cause is thought to be a dietary factor or toxic substance found only in that area.

• #48 Progressive Supranuclear Palsy – EyeWiki

  https://eyewiki.org/Progressive_Supranuclear_Palsy

  Progressive supranuclear palsy (PSP) is a sporadic disease and the exact cause is not known. Potential hypotheses include: […] There have been three geographical clusters of illnesses similar to PSP in Guam, Guadeloupe, and northern France. The first two locations revealed a strong association with PSP and cycad neurotoxins in Guam, and consumption of traditional medicines containing annonacin in Guadeloupe. However, the cluster in France is the most compelling case for environmental etiology, because the cases in Guam and Guadeloupe were not classic cases of PSP. The study in northern France found an increased prevalence of arsenic in the soil of chemical plants near the towns with increases in classic PSP diagnosis. […] There are 10 different MAPT mutations that have been identified in PSP patients including the R5L mutation in exon 1. The remaining variants are in exon 10 and its splicing region. These genetic abnormalities lead to neurofibrillary tangles and are currently under investigation as causative agents.

• #49 Progressive Supranuclear Palsy – EyeWiki

  https://eyewiki.org/Progressive_Supranuclear_Palsy

  Progressive supranuclear palsy (PSP) is a sporadic disease and the exact cause is not known. Potential hypotheses include: […] There have been three geographical clusters of illnesses similar to PSP in Guam, Guadeloupe, and northern France. The first two locations revealed a strong association with PSP and cycad neurotoxins in Guam, and consumption of traditional medicines containing annonacin in Guadeloupe. However, the cluster in France is the most compelling case for environmental etiology, because the cases in Guam and Guadeloupe were not classic cases of PSP. The study in northern France found an increased prevalence of arsenic in the soil of chemical plants near the towns with increases in classic PSP diagnosis. […] There are 10 different MAPT mutations that have been identified in PSP patients including the R5L mutation in exon 1. The remaining variants are in exon 10 and its splicing region. These genetic abnormalities lead to neurofibrillary tangles and are currently under investigation as causative agents.

• #50 Progressive Supranuclear Palsy (PSP) | National Institute of Neurological Disorders and Stroke

  https://www.ninds.nih.gov/health-information/disorders/progressive-supranuclear-palsy-psp

  PSP is usually sporadic, meaning that it occurs randomly without a known cause. In very few cases, the disease results from mutations in the MAPT gene. This mutation provides faulty instructions for making tau in brain cells. For most people with PSP, no known genetic factor is found. […] Several theories suggest that PSP might be caused by: Random genetic changes that occur in particular cells or genes in a specific combination that injures these cells. Exposure to some unknown environmental factor (such as chemicals in food, air, or water), which slowly damages certain vulnerable areas of the brain. Cellular damage caused by free radicals, which are reactive molecules produced continuously by all cells during normal metabolism. Although the body has built-in ways to clear free radicals from the body, scientists suspect that under certain circumstances free radicals can react with and damage other molecules.

• #51 What Causes PSP?

  https://www.triadfamilyhealth.com/conditions/progressive-supranuclear-palsy-psp/what-causes-psp

  There are, however, several theories about PSP’s cause. One possibility is that an unconventional virus-like agent infects the body and takes years or decades to start producing visible effects. Another possibility is that random genetic mutations, of the kind that occur in all of us all the time, happen to occur in particular cells or certain genes, in just the right combination to injure these cells. A third possibility is that there is exposure to some unknown chemical in the food, air, or water which slowly damages certain vulnerable areas of the brain. This theory stems from a clue found on the Pacific island of Guam, where a common neurological disease occurring only there and on a few neighboring islands shares some of the characteristics of PSP, Alzheimer’s disease, Parkinson’s disease, and amyotrophic lateral sclerosis (Lou Gehrig’s disease). Its cause is thought to be a dietary factor or toxic substance found only in that area.

• #52 Progressive supranuclear palsy – Wikipedia

  https://en.wikipedia.org/wiki/Progressive_supranuclear_palsy

  Progressive supranuclear palsy (PSP) is a late-onset neurodegenerative disease involving the gradual deterioration and death of specific volumes of the brain. […] The cause of the condition is uncertain, but involves the accumulation of tau protein within the brain. […] The cause of PSP is unknown. Fewer than 1% of those with PSP have a family member with the same disorder. A variant in the gene for tau protein called the H1 haplotype, located on chromosome 17 (rs1800547), has been linked to PSP. […] Other genes, as well as environmental toxins, are being investigated as other possible contributors to the cause of PSP. […] Besides tauopathy, mitochondrial dysfunction seems to be a factor involved in PSP.

• #53 Progressive Supranuclear Palsy (PSP) Information Page » Norman Fixel Institute for Neurological Diseases » University of Florida

  https://fixel.ufhealth.org/getting-care/centers-of-excellence/progressive-supranuclear-palsy-atypical-parkinsonism-center-of-excellence/progressive-supranuclear-palsy-psp-information-page/

  Understanding the process of how tau goes from a good to a bad protein and contributes different neurodegenerative diseases is a major area of research. […] Other factors such as neuro-inflammation and mitochondrial dysfunction also play a role in the disease and may also be amenable for therapeutics in the future. […] There is hope! Research on PSP and related disorders has exploded in recent years. We know now more about the pathology of PSP than ever thanks to the generosity of patients. However, the cause of this devastating disease remains unclear. There is active research though in many areas looking at the pathology and role of tau protein in disease, protein processing, genetics, environmental risk factors, biomarkers, brain imaging, and therapeutics for PSP.

• #54 Progressive Supranuclear Palsy: 4 Stages, Symptoms & Care | Plexus

  https://plexusnc.com/progressive-supranuclear-palsy-an-overview/

  Progressive Supranuclear Palsy (PSP) is a rare yet life-altering neurological disorder that affects movement, balance, vision, and thinking. […] Caused by the abnormal buildup of tau proteins in specific parts of the brain, PSP gradually damages nerve cells, impacting a persons ability to walk, speak, swallow, and express emotions. […] Emerging research suggests that inflammation in the brain may play a key role in worsening PSP. Chronic inflammation may contribute to the damage caused by tau protein accumulation, accelerating neuron loss. […] Scientists are exploring anti-inflammatory therapies to slow this process an exciting area of research that could redefine future treatment protocols. […] PSP progresses faster, affects eye movement more severely, and impacts cognitive abilities earlier than Parkinsons. It also typically responds poorly to medications used for Parkinsons.

• #55 Progressive Supranuclear Palsy (PSP) Information Page » Norman Fixel Institute for Neurological Diseases » University of Florida

  https://fixel.ufhealth.org/getting-care/centers-of-excellence/progressive-supranuclear-palsy-atypical-parkinsonism-center-of-excellence/progressive-supranuclear-palsy-psp-information-page/

  Understanding the process of how tau goes from a good to a bad protein and contributes different neurodegenerative diseases is a major area of research. […] Other factors such as neuro-inflammation and mitochondrial dysfunction also play a role in the disease and may also be amenable for therapeutics in the future. […] There is hope! Research on PSP and related disorders has exploded in recent years. We know now more about the pathology of PSP than ever thanks to the generosity of patients. However, the cause of this devastating disease remains unclear. There is active research though in many areas looking at the pathology and role of tau protein in disease, protein processing, genetics, environmental risk factors, biomarkers, brain imaging, and therapeutics for PSP.

• #56 Progressive Supranuclear Palsy (PSP) | National Institute of Neurological Disorders and Stroke

  https://www.ninds.nih.gov/health-information/disorders/progressive-supranuclear-palsy-psp

  PSP is usually sporadic, meaning that it occurs randomly without a known cause. In very few cases, the disease results from mutations in the MAPT gene. This mutation provides faulty instructions for making tau in brain cells. For most people with PSP, no known genetic factor is found. […] Several theories suggest that PSP might be caused by: Random genetic changes that occur in particular cells or genes in a specific combination that injures these cells. Exposure to some unknown environmental factor (such as chemicals in food, air, or water), which slowly damages certain vulnerable areas of the brain. Cellular damage caused by free radicals, which are reactive molecules produced continuously by all cells during normal metabolism. Although the body has built-in ways to clear free radicals from the body, scientists suspect that under certain circumstances free radicals can react with and damage other molecules.

• #57 What Causes PSP?

  https://www.triadfamilyhealth.com/conditions/progressive-supranuclear-palsy-psp/what-causes-psp

  Another possible cause of PSP is cellular damage caused by free radicals, reactive molecules produced continuously by all cells during normal metabolism. Although the body has built-in mechanisms for clearing free radicals from the system, scientists suspect that, under certain circumstances, free radicals can react with and damage other molecules. A great deal of research is directed at understanding the role of free radical damage in human diseases.

• #58 Progressive Supranuclear Palsy | Causes and Signs | CogniFit

  https://yourbrain.health/progressive-supranuclear-palsy/

  Progressive supranuclear palsy (PSP) is a serious disease of the brain. […] The symptoms of PSP are caused by harmful buildups of a protein called tau, the same protein which is present in Alzheimer’s disease. […] Tau occurs naturally in the brain, however, in people with progressive supranuclear palsy it is not broken down properly and forms clumps in brain cells, mostly at the base of the brain. […] However, the exact reason why this happens remains unknown. […] There is a hypothesis that neurodegenerative diseases such as PSP develop because when abnormal protein clumps form in a cell, they affect nearby connected cells, allowing the disease to progress through the brain. […] Other theories regarding the cause of PSP include genetic mutations, bacterial infections entering the nervous system, or free radicals which cause cell malfunction in the brain.

• #59 Clinical Approach to Progressive Supranuclear Palsy

  https://www.e-jmd.org/journal/view.php?doi=10.14802/jmd.15060

  The cause of PSP is unknown. Advanced age is the only established risk factor. To date, head injury has not been established as a risk factor of PSP. A prevalence study found 24% of PSP cases had early histological evidence of chronic traumatic encephalopathy (CTE), a neurodegenerative consequence of repetitive head injury previously referred as dementia pugilistica in boxers. Whether the CTE-tau pathology began following the onset of PSP as a result of frequent falls is not known. Geographical clusters of patients with PSP-like syndrome on Guam and Guadeloupe have probable links to environmental causes. These neurodegenerative tauopathies have clinical features atypical to the classic RS and the Guam-parkinsonian dementia complex is pathologically distinct from PSP. Frontotemporal dementia with parkinsonism due to autosomal dominant mutations in the MAPT gene (FTDP-17T) is clinically and pathologically heterogeneous. Specifically, FTDP-17T due to exon 10 coding or splicing shares the most similarity to PSP, both clinically and pathologically. Although PSP is considered a sporadic condition, FTDP-17T probably provides the best clues as to the etiology of PSP. The strong association between the H1c haplotype and PSP was confirmed by a genome-wide study (GWAS) of PSP which identified the presence of independent association signals at the MAPT locus representing both the H1/H2 haplotypes and the rs242557 MAPT SNP, related to the H1c sub-haplotype. Non-MAPT risk factors associated with PSP, EIF2AK3, MOBP and STX-6, were also found in the PSP GWAS. The protein functions of these candidate genes provide insights to the biochemical basis of the pathophysiological mechanisms. The findings of seeding and spreading of transmissible tau neuropathology in transgenic mouse brains including PSP-tau support the notion of a cell-to-cell propagation mechanism of different strains of fibrillary tau leading to distinct patterns of neuronal and glial pathology which is disease- and neural network-specific.

• #60 What Causes PSP?

  https://www.triadfamilyhealth.com/conditions/progressive-supranuclear-palsy-psp/what-causes-psp

  There are, however, several theories about PSP’s cause. One possibility is that an unconventional virus-like agent infects the body and takes years or decades to start producing visible effects. Another possibility is that random genetic mutations, of the kind that occur in all of us all the time, happen to occur in particular cells or certain genes, in just the right combination to injure these cells. A third possibility is that there is exposure to some unknown chemical in the food, air, or water which slowly damages certain vulnerable areas of the brain. This theory stems from a clue found on the Pacific island of Guam, where a common neurological disease occurring only there and on a few neighboring islands shares some of the characteristics of PSP, Alzheimer’s disease, Parkinson’s disease, and amyotrophic lateral sclerosis (Lou Gehrig’s disease). Its cause is thought to be a dietary factor or toxic substance found only in that area.

• #61 The facts about Progressive Supranuclear Palsy.

  https://www.gbhealthwatch.com/progressivesupranuclearpalsy-details.php

  Progressive supranuclear palsy (PSP) is a rare brain disorder that causes serious and progressive problems with control of gait and balance, along with complex eye movement and thinking problems. […] We know that the symptoms of PSP are caused by a gradual deterioration of brain cells in a few specific areas in the brain, mainly in the region called the brainstem. One of these areas, the substantia nigra, is also affected in Parkinson’s disease, and damage to this region of the brain accounts in part for the motor symptoms that PSP and Parkinson’s have in common. […] Scientists do not fully know what causes these brain cells to degenerate, but it is known that a hallmark of the disease is the accumulation of an abnormal protein called tau. […] There are, however, several theories about PSP’s cause. One possibility is that an unconventional virus-like agent infects the body and takes years or decades to start producing visible effects. Another possibility is that random genetic mutations, of the kind that occur in all of us all the time, happen to occur in particular cells or certain genes, in just the right combination to injure these cells. A third possibility is that there is exposure to some unknown chemical in the food, air, or water which slowly damages certain vulnerable areas of the brain. […] Another possible cause of PSP is cellular damage caused by free radicals, reactive molecules produced continuously by all cells during normal metabolism.

• #62 Progressive Supranuclear Palsy (PSP) – TheraSpecs

  https://www.theraspecs.com/blog/progressive-supranuclear-palsy-psp/?srsltid=AfmBOorxGA4lVl39bi-jwOeYFLJhwRFxwqSxZRoEqrxlS7BU_FGyEAag

  PSP symptoms are caused by slow deterioration of brain cells in some specific areas of the brain, mainly in the brainstem. […] Although scientists dont completely understand why brain cells deteriorate, people with PSP have an accumulation of tau, which is an abnormal protein. […] Genetic factors have not been identified in most people; there is also no evidence it is contagious. […] Several factors have been speculated to cause PSP, though none have been proven. […] Speculated causes include a virus-like illness that does not produce symptoms for years, a random genetic mutation, exposure to some unknown chemical that slowly damages implicated areas of the brain, or damage from free radicals.

• #63 Progressive Supranuclear Palsy

  https://neurosciences.ucsd.edu/centers-programs/movement-disorders/community/disease-overview/psp.html

  There is strong evidence that a protein known as „tau” is abnormally aggregated in multiple types of brain cells (neurons, glia) in the brain leading to gradual malfunctioning and death of these cells; the common term is „tauopathy.” […] The tau aggregation and loss of cells occurs in several areas of the brain to include the subthalamic nucleus, striatum, substantia nigra, pontine tegmentum, oculomotor nucleus, medulla and dentate nucleus. The most specific features for PSP are presence of star-shaped astrocytic tangles that can be seen with light microscopy and that are stained with antibodies to tau (figure). […] PSP is considered a sporadic (not hereditary) disease; however, it has some genetic component. Environmental causes are being investigated, particularly by Dr. Litvan, who leads a multicenter, NIH funded case-control study to determine whether there are occupational and environmental risk factors for PSP.

• #64 Progressive supranuclear palsy (PSP) | Paris Brain Institute

  https://parisbraininstitute.org/disease-files/progressive-supranuclear-palsy-psp

  In progressive supranuclear palsy (PSP), there is an abnormal accumulation of a protein known as the tau protein in neurons, causing them to degenerate. PSP is described as tauopathy. The exact cause of PSP is still unknown. The hypothesis that PSP is a multifactorial disease has been put forward; a genetic predisposition combined with environmental influences is the likely cause of the condition, but it is not hereditary. In just a few tens of families worldwide, a mutation has been identified in a gene coding for a microtubule protein. […] The neuronal death associated with the accumulation of the tau protein affects all regions of the brain, but has a greater impact on the basal ganglia and brain stem. The basal ganglia are brain structures that initiate and harmonise voluntary movements and control changes in posture. The brain stem regulates vital functions such as breathing, heart rate and swallowing.

• #65 Progressive supranuclear palsy (PSP) | Paris Brain Institute

  https://parisbraininstitute.org/disease-files/progressive-supranuclear-palsy-psp

  In progressive supranuclear palsy (PSP), there is an abnormal accumulation of a protein known as the tau protein in neurons, causing them to degenerate. PSP is described as tauopathy. The exact cause of PSP is still unknown. The hypothesis that PSP is a multifactorial disease has been put forward; a genetic predisposition combined with environmental influences is the likely cause of the condition, but it is not hereditary. In just a few tens of families worldwide, a mutation has been identified in a gene coding for a microtubule protein. […] The neuronal death associated with the accumulation of the tau protein affects all regions of the brain, but has a greater impact on the basal ganglia and brain stem. The basal ganglia are brain structures that initiate and harmonise voluntary movements and control changes in posture. The brain stem regulates vital functions such as breathing, heart rate and swallowing.

• #66 Progressive supranuclear palsy (PSP) | Paris Brain Institute

  https://parisbraininstitute.org/disease-files/progressive-supranuclear-palsy-psp

  In progressive supranuclear palsy (PSP), there is an abnormal accumulation of a protein known as the tau protein in neurons, causing them to degenerate. PSP is described as tauopathy. The exact cause of PSP is still unknown. The hypothesis that PSP is a multifactorial disease has been put forward; a genetic predisposition combined with environmental influences is the likely cause of the condition, but it is not hereditary. In just a few tens of families worldwide, a mutation has been identified in a gene coding for a microtubule protein. […] The neuronal death associated with the accumulation of the tau protein affects all regions of the brain, but has a greater impact on the basal ganglia and brain stem. The basal ganglia are brain structures that initiate and harmonise voluntary movements and control changes in posture. The brain stem regulates vital functions such as breathing, heart rate and swallowing.

• #67 Progressive Supranuclear Palsy (PSP) – Brain, Spinal Cord, and Nerve Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/brain-spinal-cord-and-nerve-disorders/movement-disorders/progressive-supranuclear-palsy-psp

  Progressive supranuclear palsy, which is much rarer than Parkinson disease, affects many parts of the brain, particularly the basal ganglia and the brain stem. […] Brain cells in these areas degenerate, but why they do is usually unknown. […] The diagnosis of progressive supranuclear palsy is based on symptoms and a doctor’s evaluation. […] Magnetic resonance imaging (MRI) is usually done to check for other disorders that may be causing the symptoms. […] In people with advanced progressive supranuclear palsy, MRI shows that the upper part of the brain stem (the midbrain) has shrunk and is smaller than normal. […] There is no cure for progressive supranuclear palsy. […] Because progressive supranuclear palsy is fatal, people with this disorder should prepare advance directives, indicating what kind of medical care they want at the end of life.

• #68 Progressive Supranuclear Palsy: Symptoms, Causes, Treatments

  https://www.webmd.com/parkinsons-disease/progressive-supranuclear-palsy-psp

  Progressive supranuclear palsy causes PSP develops because of the deterioration of brain cells in a few small but very important areas at the base of the brain. The most important affected area is the substantia nigra. When this area of the brain is affected by the disease, a lot of the palsy’s symptoms become more visible. Researchers are still looking into why the brain cells degenerate. […] There is no cure for PSP. Care for this condition focuses on creating the best quality of life for as long as possible. If you have PSP, you can expect to live for about 5 to 7 years, although many things can affect how the disease progresses.

• #69 What Causes PSP?

  https://www.triadfamilyhealth.com/conditions/progressive-supranuclear-palsy-psp/what-causes-psp

  We know that the symptoms of PSP are caused by a gradual deterioration of brain cells in a few specific areas in the brain, mainly in the region called the brainstem. One of these areas, the substantia nigra, is also affected in Parkinson’s disease, and damage to this region of the brain accounts in part for the motor symptoms that PSP and Parkinson’s have in common. […] Scientists do not fully know what causes these brain cells to degenerate, but it is known that a hallmark of the disease is the accumulation of an abnormal protein called tau. There is no evidence that PSP is contagious, and genetic factors have not been implicated in most individuals. No ethnic or racial groups have been affected more often than any others, and PSP is no more likely to occur in some geographic areas than in others.

• #70 Progressive Supranuclear Palsy

  https://neurosciences.ucsd.edu/centers-programs/movement-disorders/community/disease-overview/psp.html

  There is strong evidence that a protein known as „tau” is abnormally aggregated in multiple types of brain cells (neurons, glia) in the brain leading to gradual malfunctioning and death of these cells; the common term is „tauopathy.” […] The tau aggregation and loss of cells occurs in several areas of the brain to include the subthalamic nucleus, striatum, substantia nigra, pontine tegmentum, oculomotor nucleus, medulla and dentate nucleus. The most specific features for PSP are presence of star-shaped astrocytic tangles that can be seen with light microscopy and that are stained with antibodies to tau (figure). […] PSP is considered a sporadic (not hereditary) disease; however, it has some genetic component. Environmental causes are being investigated, particularly by Dr. Litvan, who leads a multicenter, NIH funded case-control study to determine whether there are occupational and environmental risk factors for PSP.

• #71 Progressive Supranuclear Palsy

  https://neurosciences.ucsd.edu/centers-programs/movement-disorders/community/disease-overview/psp.html

  There is strong evidence that a protein known as „tau” is abnormally aggregated in multiple types of brain cells (neurons, glia) in the brain leading to gradual malfunctioning and death of these cells; the common term is „tauopathy.” […] The tau aggregation and loss of cells occurs in several areas of the brain to include the subthalamic nucleus, striatum, substantia nigra, pontine tegmentum, oculomotor nucleus, medulla and dentate nucleus. The most specific features for PSP are presence of star-shaped astrocytic tangles that can be seen with light microscopy and that are stained with antibodies to tau (figure). […] PSP is considered a sporadic (not hereditary) disease; however, it has some genetic component. Environmental causes are being investigated, particularly by Dr. Litvan, who leads a multicenter, NIH funded case-control study to determine whether there are occupational and environmental risk factors for PSP.

• #72 Progressive Supranuclear Palsy – EyeWiki

  https://eyewiki.org/Progressive_Supranuclear_Palsy

  Although most cases of PSP are sporadic, there have been reports of familial aggregation. Among these groups with familial PSP, a stronger association with the MAPT H1 haplotype was noted when compared to controls and patients with sporadic PSP. MAPT encodes the Tau protein and genetic mutations in this gene are common in tauopathies. The familial cases of PSP were confirmed by autopsy adding to the validity of the study. However, the presence of the MAPT H1 haplotype does not definitively predict development of PSP and can only be regarded as a mild predisposition for it. […] The major pathways affected by the pathology of PSP are the dopaminergic nigrostriatal, the GABAergic, cholinoceptive striatal neurons, and the cholinergic brainstem and basal forebrain nuclei.

• #73 Progressive Supranuclear Palsy

  https://neurosciences.ucsd.edu/centers-programs/movement-disorders/community/disease-overview/psp.html

  There is strong evidence that a protein known as „tau” is abnormally aggregated in multiple types of brain cells (neurons, glia) in the brain leading to gradual malfunctioning and death of these cells; the common term is „tauopathy.” […] The tau aggregation and loss of cells occurs in several areas of the brain to include the subthalamic nucleus, striatum, substantia nigra, pontine tegmentum, oculomotor nucleus, medulla and dentate nucleus. The most specific features for PSP are presence of star-shaped astrocytic tangles that can be seen with light microscopy and that are stained with antibodies to tau (figure). […] PSP is considered a sporadic (not hereditary) disease; however, it has some genetic component. Environmental causes are being investigated, particularly by Dr. Litvan, who leads a multicenter, NIH funded case-control study to determine whether there are occupational and environmental risk factors for PSP.

• #74 Progressive Supranuclear Palsy

  https://neurosciences.ucsd.edu/centers-programs/movement-disorders/community/disease-overview/psp.html

  There is strong evidence that a protein known as „tau” is abnormally aggregated in multiple types of brain cells (neurons, glia) in the brain leading to gradual malfunctioning and death of these cells; the common term is „tauopathy.” […] The tau aggregation and loss of cells occurs in several areas of the brain to include the subthalamic nucleus, striatum, substantia nigra, pontine tegmentum, oculomotor nucleus, medulla and dentate nucleus. The most specific features for PSP are presence of star-shaped astrocytic tangles that can be seen with light microscopy and that are stained with antibodies to tau (figure). […] PSP is considered a sporadic (not hereditary) disease; however, it has some genetic component. Environmental causes are being investigated, particularly by Dr. Litvan, who leads a multicenter, NIH funded case-control study to determine whether there are occupational and environmental risk factors for PSP.

• #75 Parkinson’s disease and Progressive Supranuclear Palsy (PSP) | Hull University Teaching Hospitals NHS Trust

  https://www.hey.nhs.uk/patient-leaflet/parkinsons-disease-and-progressive-supranuclear-palsy-psp/

  Parkinsons Disease and Progressive Supranuclear Palsy (PSP) are similar conditions in which there is a premature deterioration of nerve cells in the midbrain. Onset of both diseases is in late middle age with men more likely to be affected by both conditions. PSP occurs less frequently than Parkinsons disease. […] Both conditions are caused by damage to a specific area of the brain called the Substantia Nigra. As a result both conditions have similar symptoms. […] Parkinsons disease is caused by a loss of cells in a specific region of the brainstem (called the substantia nigra) deep within the brain. This leads to a reduction in a chemical called dopamine which is responsible for sending messages to the body to help control movements. […] PSP occurs when cells in an area of the brain called the brainstem are damaged as a result of a build-up of a protein called Tau. Tau occurs naturally in the brain and is usually broken down before it reaches high levels. In people with PSP it is not broken down properly which prevents information passing between nerve cells.

• #76 Progressive Supranuclear Palsy

  https://neurosciences.ucsd.edu/centers-programs/movement-disorders/community/disease-overview/psp.html

  There is strong evidence that a protein known as „tau” is abnormally aggregated in multiple types of brain cells (neurons, glia) in the brain leading to gradual malfunctioning and death of these cells; the common term is „tauopathy.” […] The tau aggregation and loss of cells occurs in several areas of the brain to include the subthalamic nucleus, striatum, substantia nigra, pontine tegmentum, oculomotor nucleus, medulla and dentate nucleus. The most specific features for PSP are presence of star-shaped astrocytic tangles that can be seen with light microscopy and that are stained with antibodies to tau (figure). […] PSP is considered a sporadic (not hereditary) disease; however, it has some genetic component. Environmental causes are being investigated, particularly by Dr. Litvan, who leads a multicenter, NIH funded case-control study to determine whether there are occupational and environmental risk factors for PSP.

• #77 Progressive Supranuclear Palsy – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK526098/

  The cause of progressive supranuclear palsy is unknown. Advanced age and environmental factors such as exposure to toxins are theorized causes. The tau protein aggregates may be due to an unconventional infectious agent, random genetic mutations, or some unknown chemical in the food, air, or water which slowly damages certain vulnerable areas of the brain. […] Since then, different phenotypes have been characterized and linked to the severity of abnormal tau accumulation and neuronal loss in various brain regions. Different progressive supranuclear palsies, regardless of clinical characteristics, share similar neuropathologic features.

• #78 Progressive supranuclear palsy (PSP) | NHS inform

  https://www.nhsinform.scot/illnesses-and-conditions/brain-nerves-and-spinal-cord/progressive-supranuclear-palsy-psp/

  Progressive supranuclear palsy (PSP) is a neurological condition. Its caused by the loss of nerve cells in parts of the brain. […] Its believed that a protein called tau, which builds up in certain areas of the brain, forms into clumps. This causes damage to the nerve cells. Over time this build up can lead to someone experiencing the symptoms of PSP. […] The location of the clumps and the amount of tau varies between people with PSP. This also affects the type and severity of symptoms. […] PSP tends not to run in families and is not believed to be hereditary in most cases.

• #79 Progressive Supranuclear Palsy (PSP): Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/6096-progressive-supranuclear-palsy

  Progressive supranuclear palsy (PSP) is a rare condition that affects certain areas of your brain. […] Scientists dont know the exact cause of progressive supranuclear palsy. However, experts know that a protein called tau is involved. Tau is an important protein for brain health. It helps preserve the normal structure of neurons (brain cells). […] When you have PSP, the tau in your brain clumps together (aggregates). The clumps then damage neurons. Researchers have several theories for why this might happen, some of which include: Random genetic mutations. […] An unknown chemical in the air, water or your food that slowly damages certain vulnerable areas of your brain. […] The main risk factor for PSP is age. Your risk for developing PSP increases as you age if youre 60 or older. The condition is slightly more common in males.

• #80 Progressive Supranuclear Palsy: Understanding This Rare Condition

  https://www.healthline.com/health/progressive-supranuclear-palsy

  Progressive supranuclear palsy is a rare condition that affects the brain. The condition is also called Steele-Richardson-Olszewski syndrome. […] Progressive supranuclear palsy causes the brain cells that control movement and motor skills to deteriorate. […] Its not clear what causes progressive supranuclear palsy, but researchers have found that people with supranuclear palsy have excess amounts of a protein called tau in their brains. […] Researchers arent sure what the link between this protein and brain cell deterioration is. […] No other possible causes have been identified. There are some cases of progressive supranuclear palsy occurring more than once in the same family, but no genetic link has been found. […] The only known risk factor is age. People in their 60s and 70s are the most commonly diagnosed.

• #81 Progressive Supranuclear Palsy (PSP): Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/6096-progressive-supranuclear-palsy

  Progressive supranuclear palsy (PSP) is a rare condition that affects certain areas of your brain. […] Scientists dont know the exact cause of progressive supranuclear palsy. However, experts know that a protein called tau is involved. Tau is an important protein for brain health. It helps preserve the normal structure of neurons (brain cells). […] When you have PSP, the tau in your brain clumps together (aggregates). The clumps then damage neurons. Researchers have several theories for why this might happen, some of which include: Random genetic mutations. […] An unknown chemical in the air, water or your food that slowly damages certain vulnerable areas of your brain. […] The main risk factor for PSP is age. Your risk for developing PSP increases as you age if youre 60 or older. The condition is slightly more common in males.

• #82 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Progressive-Supranuclear-Palsy-(PSP)-Causes.aspx

  Progressive supranuclear palsy (PSP) is a rare brain disorder that affects approximately 6 in 100,000 people worldwide. […] The mechanism that causes PSP is not fully known. Most cases seem to appear spontaneously with no previous family history of neurodegenerative disorders. […] PSP (and other neurodegenerative disorders) has been linked to mutations in the MAPT gene, which encodes for a type of protein called tau. […] Interestingly, pathological tau protein is also found in PSP sufferers who don’t exhibit MAPT mutations. This has led researchers to believe that environmental factors play a role in the development of the disorder. […] The only risk factor established so far for PSP is advanced age. […] However, there is some evidence to show that certain chemicals in the environment or diet may contribute to PSP. […] Additionally, exposure to significant life stressors may be associated with the development of PSP. […] No conclusive evidence has been found so far to classify PSP as a genetic disease.

• #83 FloridaHealthFinder | Progressive supranuclear palsy | Health Encyclopedia | FloridaHealthFinder

  https://quality.healthfinder.fl.gov/health-encyclopedia/HIE/1/000767

  Progressive supranuclear palsy (PSP) is a movement disorder that occurs from damage to certain nerve cells in the brain. […] The cause of the damage to the brain cells is unknown. […] People with PSP have deposits in brain tissues that look like those found in people with Alzheimer disease. […] The disorder is most often seen in people over 60 years old, and is somewhat more common in men.

• #84 Parkinson’s disease and Progressive Supranuclear Palsy (PSP) | Hull University Teaching Hospitals NHS Trust

  https://www.hey.nhs.uk/patient-leaflet/parkinsons-disease-and-progressive-supranuclear-palsy-psp/

  Parkinsons Disease and Progressive Supranuclear Palsy (PSP) are similar conditions in which there is a premature deterioration of nerve cells in the midbrain. Onset of both diseases is in late middle age with men more likely to be affected by both conditions. PSP occurs less frequently than Parkinsons disease. […] Both conditions are caused by damage to a specific area of the brain called the Substantia Nigra. As a result both conditions have similar symptoms. […] Parkinsons disease is caused by a loss of cells in a specific region of the brainstem (called the substantia nigra) deep within the brain. This leads to a reduction in a chemical called dopamine which is responsible for sending messages to the body to help control movements. […] PSP occurs when cells in an area of the brain called the brainstem are damaged as a result of a build-up of a protein called Tau. Tau occurs naturally in the brain and is usually broken down before it reaches high levels. In people with PSP it is not broken down properly which prevents information passing between nerve cells.

• #85 Progressive supranuclear palsy – Symptoms, diagnosis and treatment | BMJ Best Practice US

  https://bestpractice.bmj.com/topics/en-us/3000330

  Progressive supranuclear palsy (PSP) is a neurodegenerative disorder that classically presents with oculomotor dysfunction (abnormal vertical eye movements), postural instability and falls, parkinsonism, and cognitive (mainly executive) dysfunction. […] PSP is caused by abnormal accumulation of tau protein, which is a constituent of neural structural microtubules and is abundantly present in neurons. […] PSP can present with multiple clinical phenotypes depending on the site of more severe pathology and the pattern of pathology spread in the brain. […] Risk factors include increasing age (40 years) and exposure to chromate and phosphate.

• #86 Progressive Supranuclear Palsy: Causes, Symptoms, Diagnosis And Treatment

  https://www.netmeds.com/health-library/post/progressive-supranuclear-palsy-causes-symptoms-diagnosis-and-treatment?srsltid=AfmBOop1XJvF1f6AfnKma7BpOon8ZoWrWmVgQQUNHgEq_6kp0DpqDCbb

  The cause of progressive supranuclear palsy is unknown but most likely the disease occurs when brain cells in certain parts of the brain are damaged because of a build-up of a protein called tau. […] Abnormalities in tau protein lead to damage in both cortical (the outer layer of the cerebrum) as well as subcortical areas of the brain. […] The reason for this intrinsically disordered protein malfunction can be an infectious agent, a random genetic mutation, or a chemical present in the food, air, or water. […] A common risk factor for this illness is generally identified as age. […] Moreover, environmental factors such as air pollution and pesticides and industrial solvents could increase the risk of this neurological, degenerative disorder.

• #87 Environmental Risk Factors for Progressive Supranuclear Palsy

  https://www.e-jmd.org/journal/view.php?number=346

  Chronic exposure to the Annonaceae family, such as Annona muricata (corossol, soursop, guanabana and graviola), Annona squamosa (pomme cannelle, sweepsop, sugar apple, and cherimoya), and Annona reticulata (cachiman, custard-apple and mamon), was reported to be associated with an increased risk of developing PSP-like features. […] Annonacin may play a role in the pathomechanism as a mitochondrial inhibitor and a toxin to induce tau pathology and neurodegeneration. […] Metal exposure is associated with PSP. […] Observational and case-control studies suggested that hypertension is associated with PSP. […] The use of beta blockers showed an association with PSP, while other drugs, such as calcium channel blockers, diuretics, and angiotensin-converting enzyme inhibitors/angiotensin receptor blockers, did not show any association with PSP in the univariate analysis of the ENGENE-PSP study.

• #88 Environmental Risk Factors for Progressive Supranuclear Palsy

  https://www.e-jmd.org/journal/view.php?number=346

  Repeated stressful events may increase the risk for PSP. […] This extensive literature review about environmental and modifiable factors in PSP shows that specific dietary habits, such as high consumption of Annonaceae family fruits, metal/chemical exposure, hypertension, and stress, are risk factors for PSP. High educational level and statin use may be protective.

• #89 Environmental Risk Factors for Progressive Supranuclear Palsy

  https://www.e-jmd.org/journal/view.php?number=346

  Chronic exposure to the Annonaceae family, such as Annona muricata (corossol, soursop, guanabana and graviola), Annona squamosa (pomme cannelle, sweepsop, sugar apple, and cherimoya), and Annona reticulata (cachiman, custard-apple and mamon), was reported to be associated with an increased risk of developing PSP-like features. […] Annonacin may play a role in the pathomechanism as a mitochondrial inhibitor and a toxin to induce tau pathology and neurodegeneration. […] Metal exposure is associated with PSP. […] Observational and case-control studies suggested that hypertension is associated with PSP. […] The use of beta blockers showed an association with PSP, while other drugs, such as calcium channel blockers, diuretics, and angiotensin-converting enzyme inhibitors/angiotensin receptor blockers, did not show any association with PSP in the univariate analysis of the ENGENE-PSP study.

• #90 Environmental Risk Factors for Progressive Supranuclear Palsy

  https://www.e-jmd.org/journal/view.php?number=346

  Repeated stressful events may increase the risk for PSP. […] This extensive literature review about environmental and modifiable factors in PSP shows that specific dietary habits, such as high consumption of Annonaceae family fruits, metal/chemical exposure, hypertension, and stress, are risk factors for PSP. High educational level and statin use may be protective.

• #91 Progressive Supranuclear Palsy (PSP) Information Page » Norman Fixel Institute for Neurological Diseases » University of Florida

  https://fixel.ufhealth.org/getting-care/centers-of-excellence/progressive-supranuclear-palsy-atypical-parkinsonism-center-of-excellence/progressive-supranuclear-palsy-psp-information-page/

  Understanding the process of how tau goes from a good to a bad protein and contributes different neurodegenerative diseases is a major area of research. […] Other factors such as neuro-inflammation and mitochondrial dysfunction also play a role in the disease and may also be amenable for therapeutics in the future. […] There is hope! Research on PSP and related disorders has exploded in recent years. We know now more about the pathology of PSP than ever thanks to the generosity of patients. However, the cause of this devastating disease remains unclear. There is active research though in many areas looking at the pathology and role of tau protein in disease, protein processing, genetics, environmental risk factors, biomarkers, brain imaging, and therapeutics for PSP.

• #92 Progressive Supranuclear Palsy | Penn State Health

  https://www.pennstatehealth.org/services-treatments/progressive-supranuclear-palsy

  Currently, the exact causes of PSP are unknown. Like many other diseases, it may not have a single cause, but could be caused by a combination of genetic, environmental and behavioral factors. […] Along with researchers around the world, the clinicians and scientists at Penn State College of Medicines Translational Brain Research Center are devoted to identifying the causes of PSP and developing better diagnostic tools through partnership with the National Institute of Neurological Disorders and Stroke (NINDS) Parkinsons Disease Biomarkers Program and clinical trials.

• #93 Progressive Supranuclear Palsy (PSP) Information Page » Norman Fixel Institute for Neurological Diseases » University of Florida

  https://fixel.ufhealth.org/getting-care/centers-of-excellence/progressive-supranuclear-palsy-atypical-parkinsonism-center-of-excellence/progressive-supranuclear-palsy-psp-information-page/

  Understanding the process of how tau goes from a good to a bad protein and contributes different neurodegenerative diseases is a major area of research. […] Other factors such as neuro-inflammation and mitochondrial dysfunction also play a role in the disease and may also be amenable for therapeutics in the future. […] There is hope! Research on PSP and related disorders has exploded in recent years. We know now more about the pathology of PSP than ever thanks to the generosity of patients. However, the cause of this devastating disease remains unclear. There is active research though in many areas looking at the pathology and role of tau protein in disease, protein processing, genetics, environmental risk factors, biomarkers, brain imaging, and therapeutics for PSP.

• #94 Case Report Discusses Symptoms and Diagnosis of Progressive Supranuclear Palsy | AFTD

  https://www.theaftd.org/posts/1ftd-in-the-news/blog-psp-case-report/

  A case report published earlier this year in the journal Radiology Case Reports discusses the symptoms and features of progressive supranuclear palsy (PSP) and explores different ways to diagnose it. […] PSP is divided into various subtypes based on the presenting symptoms and the underlying pathological mechanisms driving the disease, with its most common type, PSP-Richardson-Steele (PSP-RS), making up more than half of cases. […] The report highlights the progress in molecular imaging and its diagnostic potential for PSP, such as recent research suggesting that FDG-PET scans have diagnostic potential for PSP. […] While there is no cure for PSP, some of its symptoms — such as abnormal eye movements, depression, anxiety, or “Parkinsonian” symptoms — can be addressed with medicines and other interventions.

• #95 Case Report Discusses Symptoms and Diagnosis of Progressive Supranuclear Palsy | AFTD

  https://www.theaftd.org/posts/1ftd-in-the-news/blog-psp-case-report/

  A case report published earlier this year in the journal Radiology Case Reports discusses the symptoms and features of progressive supranuclear palsy (PSP) and explores different ways to diagnose it. […] PSP is divided into various subtypes based on the presenting symptoms and the underlying pathological mechanisms driving the disease, with its most common type, PSP-Richardson-Steele (PSP-RS), making up more than half of cases. […] The report highlights the progress in molecular imaging and its diagnostic potential for PSP, such as recent research suggesting that FDG-PET scans have diagnostic potential for PSP. […] While there is no cure for PSP, some of its symptoms — such as abnormal eye movements, depression, anxiety, or “Parkinsonian” symptoms — can be addressed with medicines and other interventions.

• #96 Progressive Supranuclear Palsy: 4 Stages, Symptoms & Care | Plexus

  https://plexusnc.com/progressive-supranuclear-palsy-an-overview/

  Most cases are sporadic, meaning they occur without any family history. However, researchers continue to study whether certain genetic markers may increase susceptibility. […] Yes. Clinical trials are ongoing for therapies that target tau protein buildup, reduce inflammation, and even regenerate brain cells. At Plexus, we are pioneering autologous cell therapy a promising approach showing early signs of improvement in motor and cognitive symptoms.

• #97 Progressive Supranuclear Palsy (PSP) Information Page » Norman Fixel Institute for Neurological Diseases » University of Florida

  https://fixel.ufhealth.org/getting-care/centers-of-excellence/progressive-supranuclear-palsy-atypical-parkinsonism-center-of-excellence/progressive-supranuclear-palsy-psp-information-page/

  Understanding the process of how tau goes from a good to a bad protein and contributes different neurodegenerative diseases is a major area of research. […] Other factors such as neuro-inflammation and mitochondrial dysfunction also play a role in the disease and may also be amenable for therapeutics in the future. […] There is hope! Research on PSP and related disorders has exploded in recent years. We know now more about the pathology of PSP than ever thanks to the generosity of patients. However, the cause of this devastating disease remains unclear. There is active research though in many areas looking at the pathology and role of tau protein in disease, protein processing, genetics, environmental risk factors, biomarkers, brain imaging, and therapeutics for PSP.

• #98 Environmental Risk Factors for Progressive Supranuclear Palsy

  https://www.e-jmd.org/journal/view.php?number=346

  Typically, progressive supranuclear palsy (PSP) is clinically characterized by vertical slowing of saccades or supranuclear gaze palsy, axial-predominant parkinsonism with poor response to levodopa therapy, bulbar symptoms, and cognitive impairment, specifically, frontal/executive dysfunction. PSP is a primary four-repeat tauopathy that is caused by abnormal accumulation of pathologically altered microtubule-associated protein tau (MAPT) in neurons and glia. […] The identification of modifiable risk factors for PSP may lead to an improved understanding of the pathophysiology and targeted prevention and therapeutic efforts. […] Over the past two decades, tremendous advancements in recognizing environmental and other modifiable risk factors for PSP have been made. Here, we review environmental and modifiable lifestyle risk factors for PSP, including dietary habits, residential areas, education, metal/chemical exposure, hypertension, and drugs.

• #99 Progressive Supranuclear Palsy: 4 Stages, Symptoms & Care | Plexus

  https://plexusnc.com/progressive-supranuclear-palsy-an-overview/

  Progressive Supranuclear Palsy (PSP) is a rare yet life-altering neurological disorder that affects movement, balance, vision, and thinking. […] Caused by the abnormal buildup of tau proteins in specific parts of the brain, PSP gradually damages nerve cells, impacting a persons ability to walk, speak, swallow, and express emotions. […] Emerging research suggests that inflammation in the brain may play a key role in worsening PSP. Chronic inflammation may contribute to the damage caused by tau protein accumulation, accelerating neuron loss. […] Scientists are exploring anti-inflammatory therapies to slow this process an exciting area of research that could redefine future treatment protocols. […] PSP progresses faster, affects eye movement more severely, and impacts cognitive abilities earlier than Parkinsons. It also typically responds poorly to medications used for Parkinsons.

• #100 Progressive Supranuclear Palsy: 4 Stages, Symptoms & Care | Plexus

  https://plexusnc.com/progressive-supranuclear-palsy-an-overview/

  Most cases are sporadic, meaning they occur without any family history. However, researchers continue to study whether certain genetic markers may increase susceptibility. […] Yes. Clinical trials are ongoing for therapies that target tau protein buildup, reduce inflammation, and even regenerate brain cells. At Plexus, we are pioneering autologous cell therapy a promising approach showing early signs of improvement in motor and cognitive symptoms.

• #101

  https://111.wales.nhs.uk/encyclopaedia/p/article/progressivesupranuclearpalsy/

  Progressive supranuclear palsy (PSP) is a rare neurological condition that can cause problems with balance, movement, vision, speech and swallowing. […] It’s caused by increasing numbers of brain cells becoming damaged over time. […] PSP occurs when brain cells in certain parts of the brain are damaged as a result of a build-up of a protein called tau. […] The condition has been linked to changes in certain genes, but these genetic faults aren’t inherited and the risk to other family members, including the children or siblings of someone with PSP, is very low. […] There have been several trials of different medications that aim to help symptoms of PSP or slow its progression. […] Further research into treatments, scans and causes of the condition is ongoing.

• #102 Progressive Supranuclear Palsy (PSP) | National Institute of Neurological Disorders and Stroke

  https://www.ninds.nih.gov/health-information/disorders/progressive-supranuclear-palsy-psp

  Progressive supranuclear palsy (PSP) is a rare neurological disorder that affects body movements, walking and balance, and eye movements. PSP is caused by damage to nerve cells in areas of the brain that control thinking and body movements. […] The exact cause of PSP is unknown, but research suggests that it involves progressive damage to cells in a few specific areas in the brain, mainly in the brain stem. The death of brain cells in one of these areas, the substantia nigra, accounts in part for the motor symptoms that PSP and Parkinson’s have in common. The tau protein deposits that accumulate in nerve cells in PSP appear to spread to different regions of the brain and cause cells to stop working and die. This in turn destroys the neuronal connections that underlie healthy brain function.

• #103 What causes PSP? | Paris Brain Institute

  https://parisbraininstitute.org/disease-files/progressive-supranuclear-palsy-psp/what-causes-psp

  The causes of progressive supranuclear paralysis (PSP) are still unknown. This pathology is said to be multifactorial with probably a genetic predisposition coupled with an environmental factor. […] It is not a hereditary disease and the genetic component is very possibly based on several genetic variants not identified to date. A toxic environmental origin is a hypothesis that has been investigated, such as exposure to arsenic or chromium in the textile industries, but has never been formally proven. […] PSP is described as a taupathy because abnormal toxic accumulation of the abnormally phosphorylated TAU protein in neurons leads to their degeneration. Other brain cells such as astrocytes and oligodendrocytes, which protect and nourish neurons, are also affected.

• #104 What causes PSP? | Paris Brain Institute

  https://parisbraininstitute.org/disease-files/progressive-supranuclear-palsy-psp/what-causes-psp

  The causes of progressive supranuclear paralysis (PSP) are still unknown. This pathology is said to be multifactorial with probably a genetic predisposition coupled with an environmental factor. […] It is not a hereditary disease and the genetic component is very possibly based on several genetic variants not identified to date. A toxic environmental origin is a hypothesis that has been investigated, such as exposure to arsenic or chromium in the textile industries, but has never been formally proven. […] PSP is described as a taupathy because abnormal toxic accumulation of the abnormally phosphorylated TAU protein in neurons leads to their degeneration. Other brain cells such as astrocytes and oligodendrocytes, which protect and nourish neurons, are also affected.

• #105 Progressive Supranuclear Palsy (PSP) | ParkinsonsDisease.netShare to Facebookprint pageBookmark for latercommentcaret iconcaret iconmore actionsmore actionsFollow us on facebookFollow us on facebookFollow us on linkedincaret icon

  https://parkinsonsdisease.net/clinical/progressive-supranuclear-palsy

  Progressive supranuclear palsy (PSP) is not Parkinson’s disease (PD), but is a Parkinsonian-like syndrome. […] Like Parkinson’s, PSP has no known cause, no cure and is not life-threatening. […] First described in 1964 by three scientists, it has also been known by their names as Steele-Richardson-Olszewski syndrome. PSP is considered a sporadic neurodegenerative disease, one that develops by chance. Build up of the Tau protein in the brain causes cellular damage and thus affects the normal function of neurons. PSP is considered a tauopathy as is Alzheimer’s and other frontotemporal brain disorders. […] There is no known specific cause of PD but it is considered to be a combination of genetic and environmental factors just like PSP.

• #106 PSP Progressive Supranuclear Palsy | Definitions, Symptoms, Help

  https://www.pspassociation.org.uk/information-and-support/what-is-psp/

  Progressive Supranuclear Palsy (PSP) is a rare neurodegenerative disease caused by the progressive death of nerve cells in certain parts of the brain. […] Specifically, PSP is associated with the overproduction of a protein called tau, which accumulates in and damages brain cells. […] Researchers are still working to understand what exactly triggers this overproduction of tau, but it likely involves a combination of genetic susceptibility factors and unknown environmental exposures or triggers.

• #107 Orphanet: Progressive supranuclear palsy

  https://www.orpha.net/en/disease/detail/683

  A rare late-onset neurodegenerative disease characterized by ocular motor dysfunction, postural instability, akinesia-rigidity, and cognitive dysfunction. […] PSP is a 4R tauopathy composed of a preponderance of four-repeat (exon 10 positive) tau isoforms and a characteristic biochemical profile (doublet tau 64 and tau 69). The MAPT H1-clade specific sub-haplotype, H1c, is a risk factor for this disease. PSP is also characterized by deficits in several neurotransmitter systems (e.g., dopaminergic, cholinergic, GABAergic). The factors that initiate tau-neurodegeneration are unknown. […] PSP is a sporadic, non-hereditary disease.

• #108 Progressive Supranuclear Palsy (PSP) | National Institute of Neurological Disorders and Stroke

  https://www.ninds.nih.gov/health-information/disorders/progressive-supranuclear-palsy-psp

  PSP is usually sporadic, meaning that it occurs randomly without a known cause. In very few cases, the disease results from mutations in the MAPT gene. This mutation provides faulty instructions for making tau in brain cells. For most people with PSP, no known genetic factor is found. […] Several theories suggest that PSP might be caused by: Random genetic changes that occur in particular cells or genes in a specific combination that injures these cells. Exposure to some unknown environmental factor (such as chemicals in food, air, or water), which slowly damages certain vulnerable areas of the brain. Cellular damage caused by free radicals, which are reactive molecules produced continuously by all cells during normal metabolism. Although the body has built-in ways to clear free radicals from the body, scientists suspect that under certain circumstances free radicals can react with and damage other molecules.

• #109 Progressive supranuclear palsy (PSP) | Paris Brain Institute

  https://parisbraininstitute.org/disease-files/progressive-supranuclear-palsy-psp

  In progressive supranuclear palsy (PSP), there is an abnormal accumulation of a protein known as the tau protein in neurons, causing them to degenerate. PSP is described as tauopathy. The exact cause of PSP is still unknown. The hypothesis that PSP is a multifactorial disease has been put forward; a genetic predisposition combined with environmental influences is the likely cause of the condition, but it is not hereditary. In just a few tens of families worldwide, a mutation has been identified in a gene coding for a microtubule protein. […] The neuronal death associated with the accumulation of the tau protein affects all regions of the brain, but has a greater impact on the basal ganglia and brain stem. The basal ganglia are brain structures that initiate and harmonise voluntary movements and control changes in posture. The brain stem regulates vital functions such as breathing, heart rate and swallowing.

• #110 Progressive Supranuclear Palsy (PSP) Information Page » Norman Fixel Institute for Neurological Diseases » University of Florida

  https://fixel.ufhealth.org/getting-care/centers-of-excellence/progressive-supranuclear-palsy-atypical-parkinsonism-center-of-excellence/progressive-supranuclear-palsy-psp-information-page/

  Understanding the process of how tau goes from a good to a bad protein and contributes different neurodegenerative diseases is a major area of research. […] Other factors such as neuro-inflammation and mitochondrial dysfunction also play a role in the disease and may also be amenable for therapeutics in the future. […] There is hope! Research on PSP and related disorders has exploded in recent years. We know now more about the pathology of PSP than ever thanks to the generosity of patients. However, the cause of this devastating disease remains unclear. There is active research though in many areas looking at the pathology and role of tau protein in disease, protein processing, genetics, environmental risk factors, biomarkers, brain imaging, and therapeutics for PSP.

• #111

  https://111.wales.nhs.uk/encyclopaedia/p/article/progressivesupranuclearpalsy/

  Progressive supranuclear palsy (PSP) is a rare neurological condition that can cause problems with balance, movement, vision, speech and swallowing. […] It’s caused by increasing numbers of brain cells becoming damaged over time. […] PSP occurs when brain cells in certain parts of the brain are damaged as a result of a build-up of a protein called tau. […] The condition has been linked to changes in certain genes, but these genetic faults aren’t inherited and the risk to other family members, including the children or siblings of someone with PSP, is very low. […] There have been several trials of different medications that aim to help symptoms of PSP or slow its progression. […] Further research into treatments, scans and causes of the condition is ongoing.

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