Clinical Features and Disability Milestones in Multiple System Atrophy and Progressive Supranuclear Palsy

The diagnosis of Parkinson's disease is predominantly clinical, based on a combination of the cardinal features of tremor, bradykinesia and rigidity. The differential essentially lies between other conditions resulting in tremor, of which essential tremor is the commonest, and other akinetic-rigid syndromes. These include progressive supranuclear palsy, multiple system atrophy, toxins and other degenerative disorders, including diffuse Lewy body disease and corticobasal degeneration. The key clinical features of these disorders and a practical diagnostic approach are briefly reviewed in this article.

Download Full-text

Faculty Opinions recommendation of Clinical outcomes of progressive supranuclear palsy and multiple system atrophy.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.1108144.565997 ◽

2008 ◽

Author(s):

Gregor Wenning

Keyword(s):

Multiple System Atrophy ◽

Progressive Supranuclear Palsy ◽

Clinical Outcomes

Download Full-text

Cerebrospinal Fluid α-Synuclein Species in Cognitive and Movements Disorders

Brain Sciences ◽

10.3390/brainsci11010119 ◽

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.

Download Full-text