Corticobasal degeneration: a disease with widespread appearance of abnormal tau and neurofibrillary tangles, and its relation to progressive supranuclear palsy

1994 ◽  
Vol 88 (2) ◽  
pp. 113-121 ◽  
Author(s):  
H. Mori ◽  
M. Nishimura ◽  
Y. Namba ◽  
M. Oda
Keyword(s):  
Progressive Supranuclear Palsy ◽  
Neurofibrillary Tangles ◽  
Corticobasal Degeneration

scholarly journals All tangled up: Tau-dependent neurodegeneration

2010 ◽  
Vol 32 (2) ◽  
pp. 10-13
Author(s):  
Ceri Lyn-Adams ◽  
Kevin Moffat ◽  
Calum Sutherland ◽  
Bruno G. Frenguelli
Keyword(s):  
Neurodegenerative Diseases ◽  
Progressive Supranuclear Palsy ◽  
Neurofibrillary Tangles ◽  
Neuronal Loss ◽  
Corticobasal Degeneration ◽  
Chromosome 17 ◽  
Phosphorylated Tau ◽  
Brain Correlates ◽  
Severity Of Dementia ◽  
The Brain

Currently, there are 30 million people worldwide suffering from dementia. This number is predicted to rise to 100 million if effective treatments aren't developed rapidly. Alzheimer's disease (AD) is the most common form of dementia and is also the most prevalent of a group of neurodegenerative diseases known as tauopathies. Tauopathies are characterized by intraneuronal inclusions (pretangles) composed of aggregates of highly phosphorylated tau in the form of paired helical or straight filaments (PHFs), and neuronal loss. As the load of PHFs increases, they will aggregate and eventually form neurofibrillary tangles (NFTs) which fill the whole cell. The number of tau tangles present in the brain correlates well with the severity of dementia. Tau tangles are routinely found in AD, frontotemporal dementia linked to chromosome 17 with parkinsonism (FTDP-17), progressive supranuclear palsy, Pick's disease, corticobasal degeneration, head trauma and Down's syndrome to name but a few.

scholarly journals A molecular pathology, neurobiology, biochemical, genetic and neuroimaging study of progressive apraxia of speech

2021 ◽  
Vol 12 (1) ◽  
Author(s):  
Keith A. Josephs ◽  
Joseph R. Duffy ◽  
Heather M. Clark ◽  
Rene L. Utianski ◽  
Edythe A. Strand ◽  
...  
Keyword(s):  
Progressive Supranuclear Palsy ◽  
Molecular Pathology ◽  
Age At Onset ◽  
Haplotype Frequency ◽  
Corticobasal Degeneration ◽  
Apraxia Of Speech ◽  
Speech Characteristics ◽  
Biochemical Genetic ◽  
Neuroimaging Study ◽  
Longitudinal Imaging

AbstractProgressive apraxia of speech is a neurodegenerative syndrome affecting spoken communication. Molecular pathology, biochemistry, genetics, and longitudinal imaging were investigated in 32 autopsy-confirmed patients with progressive apraxia of speech who were followed over 10 years. Corticobasal degeneration and progressive supranuclear palsy (4R-tauopathies) were the most common underlying pathologies. Perceptually distinct speech characteristics, combined with age-at-onset, predicted specific 4R-tauopathy; phonetic subtype and younger age predicted corticobasal degeneration, and prosodic subtype and older age predicted progressive supranuclear palsy. Phonetic and prosodic subtypes showed differing relationships within the cortico-striato-pallido-nigro-luysial network. Biochemical analysis revealed no distinct differences in aggregated 4R-tau while tau H1 haplotype frequency (69%) was lower compared to 1000+ autopsy-confirmed 4R-tauopathies. Corticobasal degeneration patients had faster rates of decline, greater cortical degeneration, and shorter illness duration than progressive supranuclear palsy. These findings help define the pathobiology of progressive apraxia of speech and may have consequences for development of 4R-tau targeting treatment.

scholarly journals Cerebrospinal Fluid α-Synuclein Species in Cognitive and Movements Disorders

2021 ◽  
Vol 11 (1) ◽  
pp. 119
Author(s):  
Vasilios C. Constantinides ◽  
Nour K. Majbour ◽  
George P. Paraskevas ◽  
Ilham Abdi ◽  
Bared Safieh-Garabedian ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Multiple System Atrophy ◽  
Progressive Supranuclear Palsy ◽  
Movement Disorders ◽  
Corticobasal Degeneration ◽  
Healthy Controls ◽  
Frontotemporal Degeneration ◽  
Blood Contamination ◽  
Dementia Patients ◽  
Specificity And Sensitivity

Total CSF α-synuclein (t-α-syn), phosphorylated α-syn (pS129-α-syn) and α-syn oligomers (o-α-syn) have been studied as candidate biomarkers for synucleinopathies, with suboptimal specificity and sensitivity in the differentiation from healthy controls. Studies of α-syn species in patients with other underlying pathologies are lacking. The aim of this study was to investigate possible alterations in CSF α-syn species in a cohort of patients with diverse underlying pathologies. A total of 135 patients were included, comprising Parkinson’s disease (PD; n = 13), multiple system atrophy (MSA; n = 9), progressive supranuclear palsy (PSP; n = 13), corticobasal degeneration (CBD; n = 9), Alzheimer’s disease (AD; n = 51), frontotemporal degeneration (FTD; n = 26) and vascular dementia patients (VD; n = 14). PD patients exhibited higher pS129-α-syn/α-syn ratios compared to FTD (p = 0.045), after exclusion of samples with CSF blood contamination. When comparing movement disorders (i.e., MSA vs. PD vs. PSP vs. CBD), MSA patients had lower α-syn levels compared to CBD (p = 0.024). Patients with a synucleinopathy (PD and MSA) exhibited lower t-α-syn levels (p = 0.002; cut-off value: ≤865 pg/mL; sensitivity: 95%, specificity: 69%) and higher pS129-/t-α-syn ratios (p = 0.020; cut-off value: ≥0.122; sensitivity: 71%, specificity: 77%) compared to patients with tauopathies (PSP and CBD). There are no significant α-syn species alterations in non-synucleinopathies.

scholarly journals Clinical features differentiating patients with postmortem confirmed progressive supranuclear palsy and corticobasal degeneration

1999 ◽  
Vol 246 (S2) ◽  
pp. II1-II5 ◽  
Author(s):  
I. Litvan ◽  
D. A. Grimes ◽  
A. E. Lang ◽  
J. Jankovic ◽  
A. McKee ◽  
...  
Keyword(s):  
Progressive Supranuclear Palsy ◽  
Clinical Features ◽  
Corticobasal Degeneration
Sign in / Sign up

Export Citation Format

Share Document