Progressive Supranuclear Palsy

Lubarsky, Michael; Juncos, Jorge L.

doi:10.1097/nrl.0b013e31815cffc9

Cited by 53 publications

(60 citation statements)

References 75 publications

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“…These syndromes can be distinguished by their clinical pictures in the first 2 years but there is a clinical overlap, and after 6 years of follow up, the clinical phenomenology might become similar. The patients with PSP-P have less severe tau pathology than those with RS 3,7,10 . PSP-pure akinesia with gait freezing (PSP-PAGF): This PSP-phenotype is highly predictive of PSP-tau pathology.…”

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confidence: 90%

“…Tauopathies refers generally to neurodegenerative disorders with prominent tau pathology in the neuronal or glial cells. Tau is a microtubule-associated protein expressed in neurons, which in tauopathies forms abnormal, fibrillar structures of ag-gregated, hyperphosphorylated and ubiquinated tau 3,4 . There are an increasing number of tauopathies described, including Alzheimer's disease, corticobasal degeneration, postencephalitic parkinsonism, Parkinson-dementia complex of Guam, Guadeloupian parkinsonism, frontotemporal dementia with parkinsonism linked to chromosome 17, Pick disease and Niemann-Pick disease type C [2][3][4] .…”

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confidence: 99%

“…Guadeloupian parkinsonism), complicating the pathological diagnosis of PSP. The most specific features are star-shaped astrocytic tufts and neurofibrillary tangles that can be seen with light microscopy and that immunostain strongly with antibodies to tau 3,4 .…”

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confidence: 99%

“…Most patients become dependent on others for care within 3 to 4 years of diagnosis and the common causes of death are aspiration pneumonia, primary neurogenic respiratory failure and pulmonary emboli. RS consists of diffuse tau pathology and the most severely affected regions are subthalamic nucleus and substantia nigra 3,4,7,10 .…”

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confidence: 99%

“…The tau-pathology in these individuals is described as pallido-nigro-luysial. The restricted distribution of pathology in PAGF is thought to be the cause of clinical differences and better prognosis 3,10 .…”

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confidence: 99%

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Progressive supranuclear palsy: new concepts

Barsottini

Felı́cio

Aquino

et al. 2010

Arq. Neuro-Psiquiatr.

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Progressive supranuclear palsy (PSP) is a distinctive form of neurodegenerative disease which affects the brainstem and basal ganglia. Patients present supranuclear ophthalmoplegia, postural instability and mild dementia. PSP is defined neuropathologically by the accumulation of neurofibrillary tangles in the subthalamic nucleus, pallidum, red nucleus, substantia nigra, striatum, pontine tegmentum, oculomotor nucleus, medulla and dentate nucleus. Over the last decade many lines of investigations have helped refine PSP in many aspects and it is the purpose of this review to help neurologists identify PSP, to better understand its pathophysiology and to provide a more focused, symptom-based treatment approach. Key words: progressive supranuclear palsy, atypical parkinsonism, tauopathy.Paralisia supranuclear progressiva: conceitos atuais RESUMO A paralisia supranuclear progressiva (PSP) é uma doença neurodegenerativa, que afeta principalmente o tronco cerebral e os núcleos da base. O quadro clínico se caracteriza por oftalmoparesia supranuclear, instabilidade postural e demência . Do ponto de vista anátomo-patológico, a PSP se caracteriza por acúmulo de emaranhados neurofibrilares no núcleo subtalâmico, globo pálido, núcleo rubro, substância negra, estriado, tegumento da ponte, núcleos oculomotores, bulbo e núcleo denteado. Nas últimas décadas, muitas linhas de pesquisa têm colaborado para redefinir a PSP em muitos aspectos. Os objetivos dessa revisão são auxiliar o neurologista geral na identificação da doença, compreensão da sua fisiopatologia, além de apresentar alternativas para seu tratamento sintomático. Palavras-chave: paralisia supranuclear progressiva, parkinsonismo atípico, taupatias.

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confidence: 99%

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confidence: 99%

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confidence: 99%

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Progressive supranuclear palsy: new concepts

Barsottini

Felı́cio

Aquino

et al. 2010

Arq. Neuro-Psiquiatr.

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Mass spectrometry‐based proteomics analysis of human globus pallidus from progressive supranuclear palsy patients discovers multiple disease pathways

Jang

Thuraisamy

Redding‐Ochoa

et al. 2022

Clinical & Translational Med

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Background Progressive supranuclear palsy (PSP) is a neurodegenerative disorder clinically characterized by progressive postural instability, supranuclear gaze palsy, parkinsonism, and cognitive decline caused by degeneration in specific areas of the brain including globus pallidus (GP), substantia nigra, and subthalamic nucleus. However, the pathogenetic mechanism of PSP remains unclear to date.Unbiased global proteome analysis of patients' brain samples is an important step toward understanding PSP pathogenesis, as proteins serve as workhorses and building blocks of the cell. Methods In this study, we conducted unbiased mass spectrometry‐based global proteome analysis of GP samples from 15 PSP patients, 15 Parkinson disease (PD) patients, and 15 healthy control (HC) individuals. To analyze 45 samples, we conducted 5 batches of 11‐plex isobaric tandem mass tag (TMT)‐based multiplexing experiments. The identified proteins were subjected to statistical analysis, such as a permutation‐based statistical analysis in the significance analysis of microarray (SAM) method and bootstrap receiver operating characteristic curve (ROC)‐based statistical analysis. Subsequently, we conducted bioinformatics analyses using gene set enrichment analysis, Search Tool for the Retrieval of Interacting Genes/Proteins (STRING) protein‐protein interaction (PPI) analysis, and weighted gene co‐expression network analysis (WGCNA). Results We have identified 10,231 proteins with ∼1,000 differentially expressed proteins. The gene set enrichment analysis results showed that the PD pathway was the most highly enriched, followed by pathways for oxidative phosphorylation, Alzheimer disease, Huntington disease, and non‐alcoholic fatty liver disease (NAFLD) when PSP was compared to HC or PD. Most of the proteins enriched in the gene set enrichment analysis were mitochondrial proteins such as cytochrome c oxidase, NADH dehydrogenase, acyl carrier protein, succinate dehydrogenase, ADP/ATP translocase, cytochrome b‐c1 complex, and/or ATP synthase. Strikingly, all of the enriched mitochondrial proteins in the PD pathway were downregulated in PSP compared to both HC and PD. The subsequent STRING PPI analysis and the WGCNA further supported that the mitochondrial proteins were the most highly enriched in PSP. Conclusion Our study showed that the mitochondrial respiratory electron transport chain complex was the key proteins that were dysregulated in GP of PSP, suggesting that the mitochondrial respiratory electron transport chain complex could potentially be involved in the pathogenesis of PSP. This is the first global proteome analysis of human GP from PSP patients, and this study paves the way to understanding the mechanistic pathogenesis of PSP.

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Clinical Features of Patients with Concomitant Parkinson's Disease and Progressive Supranuclear Palsy Pathology

Rigby

Dugger

Hentz

et al. 2014

Movement Disord Clin Pract

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The pathologic changes of Parkinson's disease (PD) and Progressive Supranuclear Palsy (PSP) have been reported to coexist, but whether PSP pathology modifies the clinical course of those individuals is unknown. The aim of this study was to determine whether clinical features of pathologically confirmed PD subjects with concomitant PSP pathology differ from those with PD alone. Subjects enrolled in the Arizona Study of Aging and Neurodegenerative Disorders had annual movement and cognitive evaluations from enrollment until death/autopsy. All cases between 1997 and 2014 with a final clinicopathological diagnosis of PD with or without PSP at autopsy were analyzed. Overall, 12 of the 125 cases with pathologically confirmed PD had coexisting PSP pathology (9.6%). Those with PD-PSP had more-prominent postural instability, body bradykinesia, difficulty arising from a chair, and falls; asymmetric onset was less common in this group. Downgaze palsy and square wave jerks were infrequent in both groups. Gender, age at death, disease duration, rate of dementia, and presence of rest tremor did not differ between groups. Only 58% of subjects in the PD-PSP group were correctly given a final diagnosis in life of PD, compared to 91% of those with PD alone. The combination of PD and PSP pathology yields a heterogeneous clinical syndrome that often resembles PD, but may be more symmetric at onset and have more-prominent postural instability and falls. Our observations suggest that coexisting PSP pathology may be an important factor contributing to the clinical heterogeneity in PD and a potential confounder in diagnosis.

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Progressive Supranuclear Palsy

Cited by 53 publications

References 75 publications

Progressive supranuclear palsy: new concepts

Progressive supranuclear palsy: new concepts

Mass spectrometry‐based proteomics analysis of human globus pallidus from progressive supranuclear palsy patients discovers multiple disease pathways

Clinical Features of Patients with Concomitant Parkinson's Disease and Progressive Supranuclear Palsy Pathology

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