Progressive supranuclear palsy
View article: Paralisia Supranuclear Progressiva (PSP) no contexto do Parkinson plus
Paralisia Supranuclear Progressiva (PSP) no contexto do Parkinson plus Open
Introdução: A Paralisia Supranuclear Progressiva (PSP) é uma doença neurodegenerativa rara do grupo Parkinson Plus, marcada pela deposição anômala de proteína tau em neurônios e glia. Essa tauopatia primária causa degeneração do tronco enc…
View article: Noninvasive imaging of amyloid-beta and tau in rodent and nonhuman primate models
Noninvasive imaging of amyloid-beta and tau in rodent and nonhuman primate models Open
Neurodegenerative diseases are characterized by the aberrant accumulation of protein aggregates. The development of noninvasive imaging techniques for detecting pathological hallmarks, amyloid‐beta plaques and tau tangles in Alzheimer's di…
View article: "CLINICAL FEATURES AND DIAGNOSTIC APPROACHES IN MAJOR NEURODEGENERATIVE DISEASES: AN UPDATED REVIEW"
"CLINICAL FEATURES AND DIAGNOSTIC APPROACHES IN MAJOR NEURODEGENERATIVE DISEASES: AN UPDATED REVIEW" Open
There are numerous clinical questions that do not have simple answers, even for well-trained medical professionals. The diagnostic instrument frequently used to evaluate cognitive impairment in neurodegenerative diseases is founded on esta…
View article: "CLINICAL FEATURES AND DIAGNOSTIC APPROACHES IN MAJOR NEURODEGENERATIVE DISEASES: AN UPDATED REVIEW"
"CLINICAL FEATURES AND DIAGNOSTIC APPROACHES IN MAJOR NEURODEGENERATIVE DISEASES: AN UPDATED REVIEW" Open
There are numerous clinical questions that do not have simple answers, even for well-trained medical professionals. The diagnostic instrument frequently used to evaluate cognitive impairment in neurodegenerative diseases is founded on esta…
View article: Unlocking the Sugar Code: Implications and Consequences of Glycosylation in Alzheimer’s Disease and Other Tauopathies
Unlocking the Sugar Code: Implications and Consequences of Glycosylation in Alzheimer’s Disease and Other Tauopathies Open
Alzheimer’s disease (AD) is the most prevalent cause of dementia, characterized by progressive cognitive decline, amyloid-β (Aβ) plaques, and neurofibrillary tangles composed of hyperphosphorylated tau protein. Other tauopathies, including…
View article: Inflammation PET and plasma neurofilament light predict survival in people with progressive supranuclear palsy
Inflammation PET and plasma neurofilament light predict survival in people with progressive supranuclear palsy Open
Progressive Supranuclear Palsy (PSP) is a primary tauopathy characterised by atrophy and neuroinflammation of the brainstem, basal ganglia and, to a lesser degree of the cortex. This study investigates the association of regional atrophy (…
View article: Molecular features of human pathological Tau distinguish tauopathy-associated dementias.
Molecular features of human pathological Tau distinguish tauopathy-associated dementias. Open
In Alzheimer’s disease (AD), pathological Tau protein shows a progressive accumulation of post-translational modifications (PTMs), reflecting disease severity, progression, and prion-like activity. While many neurodegenerative diseases wit…
View article: Molecular features of human pathological Tau distinguish tauopathy-associated dementias.
Molecular features of human pathological Tau distinguish tauopathy-associated dementias. Open
In Alzheimer’s disease (AD), pathological Tau protein shows a progressive accumulation of post-translational modifications (PTMs), reflecting disease severity, progression, and prion-like activity. While many neurodegenerative diseases wit…
View article: Rapidly Progressive Multiple System Atrophy—Cerebellar Type in a 52‐Year‐Old Woman: Diagnostic Challenges in a Resource‐Limited Setting
Rapidly Progressive Multiple System Atrophy—Cerebellar Type in a 52‐Year‐Old Woman: Diagnostic Challenges in a Resource‐Limited Setting Open
Multiple system atrophy (MSA) is a progressive adult‐onset neurodegenerative disorder that manifests as a variable combination of cerebellar, parkinsonian, and autonomic dysfunctions. We report a case of a 52‐year‐old woman who presented w…
View article: Functional network collapse in neurodegenerative disease
Functional network collapse in neurodegenerative disease Open
Cognitive and behavioral deficits in Alzheimer’s disease (AD) and frontotemporal dementia (FTD) arise alongside gray matter atrophy and altered functional connectivity, yet the structure-function relationship across the dementia spectrum r…
View article: Interpeduncular Angle: A Possible Marker to Differentiate Atypical Parkinsonism from Idiopathic Parkinson’s Disease
Interpeduncular Angle: A Possible Marker to Differentiate Atypical Parkinsonism from Idiopathic Parkinson’s Disease Open
Atypical parkinsonian disorders (APD), such as progressive supranuclear palsy (PSP), multiple system atrophy (MSA), corticobasal syndrome (CBS), and Lewy body dementia (LBD) are frequently misdiagnosed, commonly as Parkinson’s disease (PD)…
View article: Utility of Progression Rate Since Symptom Onset in Predicting Subsequent Survival in Progressive Supranuclear Palsy
Utility of Progression Rate Since Symptom Onset in Predicting Subsequent Survival in Progressive Supranuclear Palsy Open
View article: Movement Disorders and Oculomotor Abnormalities in Whipple’s Disease: An Updated Systematic Review
Movement Disorders and Oculomotor Abnormalities in Whipple’s Disease: An Updated Systematic Review Open
Introduction: Whipple’s disease (WhD) is a rare multisystemic infection caused by Tropheryma whipplei, with central nervous system (CNS) involvement seen in up to 50% of cases. Neurological symptoms may precede systemic features or occur i…
View article: Extracellular matrix remodeling links vascular–stromal dysfunction to neuronal tau pathology in Progressive Supranuclear Palsy
Extracellular matrix remodeling links vascular–stromal dysfunction to neuronal tau pathology in Progressive Supranuclear Palsy Open
Progressive supranuclear palsy-Richardson’s syndrome (PSP-RS) is a primary tauopathy lacking disease-modifying treatments, and the pathogenic mechanisms remain poorly understood. Here, combining patient-derived midbrain organoids with sing…
View article: Progressive supranuclear palsy–like parkinsonism ensuing from anti–N-methyl-Daspartate receptor encephalitis
Progressive supranuclear palsy–like parkinsonism ensuing from anti–N-methyl-Daspartate receptor encephalitis Open
View article: Refining the diagnostic accuracy of Parkinsonian disorders using metaphenomic annotation of the clinicopathological literature
Refining the diagnostic accuracy of Parkinsonian disorders using metaphenomic annotation of the clinicopathological literature Open
The diagnostic precision of Parkinsonian disorders is not accurate enough. Even in expert clinics, up to one in five diagnoses are incorrect. Gold standard diagnosis is post-mortem confirmation of the underlying proteinopathy; however, man…
View article: NDPACX: a newly defined X-linked Parkinsonian syndrome associated with <i>SLC9A6</i> hemizygote mutation
NDPACX: a newly defined X-linked Parkinsonian syndrome associated with <i>SLC9A6</i> hemizygote mutation Open
X-linked female-restricted neurodegenerative disorder with Parkinsonian syndrome and cognitive impairment (NDPACX) is a recently recognized clinical entity caused by heterozygous mutations in SLC9A6 on the X chromosome, which encodes the e…
View article: Automated <scp>MRI</scp> ‐Based Classification of Parkinsonism: A Deep Learning Approach to Distinguish <scp>PD</scp> From <scp>PSP</scp>
Automated <span>MRI</span> ‐Based Classification of Parkinsonism: A Deep Learning Approach to Distinguish <span>PD</span> From <span>PSP</span> Open
Objective Differentiating Parkinson's disease (PD) from progressive supranuclear palsy (PSP) is crucial for appropriate treatment, as each disease has distinct therapeutic requirements. The Magnetic Resonance Parkinsonism Index (MRPI) has …
View article: 2 Evaluation of Alzheimer’s Disease CSF biomarkers for distinguishing CSF Dynamic Disorders from other cognitive impairment etiologies
2 Evaluation of Alzheimer’s Disease CSF biomarkers for distinguishing CSF Dynamic Disorders from other cognitive impairment etiologies Open
Background Cognitive impairment (CI) has diverse neurological etiologies, including Alzheimer’s Disease (AD), non-degenerative (ND) conditions, and cerebrospinal fluid (CSF) dynamic disorders, such as normal pressure hydrocephalus (NPH). T…
View article: Corticobasal Degeneration: A Review
Corticobasal Degeneration: A Review Open
Corticobasal degeneration (CBD) is a rare neurodegenerative tauopathy characterized by progressive motor and cognitive dysfunction. This comprehensive review examines the clinical manifestations, neuropathology, diagnostic challenges, and …
View article: Neuropathological Characterisation of McLeod Syndrome With a Proposed New Grading System
Neuropathological Characterisation of McLeod Syndrome With a Proposed New Grading System Open
Aims X‐linked McLeod neuroacanthocytosis syndrome (MLS) is a rare neurodegenerative disorder characterised by the presence of red blood cell acanthocytosis and a chorea syndrome. Analogous to Huntington's disease (HD), MLS displays cogniti…
View article: Brain Atrophy Does Not Predict Clinical Progression in Progressive Supranuclear Palsy
Brain Atrophy Does Not Predict Clinical Progression in Progressive Supranuclear Palsy Open
Background Clinical progression rate is the typical primary endpoint measure in progressive supranuclear palsy (PSP) clinical trials. Objectives This longitudinal multicohort study investigated whether baseline clinical severity and region…
View article: Successful Treatment of Corticobasal Syndrome: Case Study
Successful Treatment of Corticobasal Syndrome: Case Study Open
Corticobasal syndrome (CBS) is an uncommon neurodegenerative syndrome, with a prevalence of only a few thousand patients in the U.S. Symptoms include alien hand syndrome, rigidity, myoclonus, weakness (often asymmetric), dysarthria, aphasi…
View article: Multimodal MRI biomarkers optimize differentiation between progressive supranuclear palsy and Parkinson's disease
Multimodal MRI biomarkers optimize differentiation between progressive supranuclear palsy and Parkinson's disease Open
The multimodal MRI biomarkers offer a more comprehensive understanding of PSP and PD patients from both morphological and pathological perspectives. By combining these MRI biomarkers, it may have the potential to promote reliable MRI-based…
View article: Inflammation PET and plasma neurofilament light predict survival in people with progressive supranuclear palsy
Inflammation PET and plasma neurofilament light predict survival in people with progressive supranuclear palsy Open
Progressive Supranuclear Palsy (PSP) is a primary tauopathy characterised by atrophy and neuroinflammation of the brainstem, basal ganglia and, to a lesser degree of the cortex. This study investigates the association of regional atrophy (…
View article: A Review of FDG-PET in Progressive Supranuclear Palsy and Corticobasal Syndrome
A Review of FDG-PET in Progressive Supranuclear Palsy and Corticobasal Syndrome Open
Although diagnostic criteria and research are constantly advancing, distinguishing between progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) remains a significant challenge. This difficulty stems from their similar clini…
View article: Differences in neuropsychological performance across clinical variants of progressive supranuclear palsy
Differences in neuropsychological performance across clinical variants of progressive supranuclear palsy Open
Objective: We investigated differences in cognition between variants of progressive supranuclear palsy (PSP) including PSP-Richardson (PSP-RS) and subcortical and cortical variants using updated diagnostic criteria and comprehensive neurop…
View article: Detecting amyloid and tau pathology in Parkinson’s disease, 4R-tauopathies and control subjects with plasma pTau217
Detecting amyloid and tau pathology in Parkinson’s disease, 4R-tauopathies and control subjects with plasma pTau217 Open
Introduction Plasma phospho-tau 217 (pTau217) is a biomarker for Alzheimer’s disease (AD) pathology, reflecting amyloid (Aβ) and tau burden, but its role in Parkinson disease (PD) and 4-repeat(4R)-tauopathies remains incompletely understoo…
View article: Increased tau-induced inflammatory responses are associated with a greater degree of atherosclerosis in progressive supranuclear palsy
Increased tau-induced inflammatory responses are associated with a greater degree of atherosclerosis in progressive supranuclear palsy Open
Introduction It has been reported that progressive supranuclear palsy (PSP) has a higher prevalence of cerebrovascular disease (CVD); however, the relationship between PSP and atherosclerotic disease remains poorly understood. This study a…
View article: Classification of Tauopathies from Human Brain Homogenates through Salt-Modulated Tau Amplification
Classification of Tauopathies from Human Brain Homogenates through Salt-Modulated Tau Amplification Open
Tauopathies are a heterogeneous group of neurodegenerative disorders characterised by the aggregation of the microtubule-associated protein tau in the brain. Recent advances in cryo-electron microscopy have revealed that tau aggregates ado…