Tufted astrocyte‐like glia in two autopsy cases of multiple system atrophy: Is it a concomitant neurodegenerative disorder with multiple system atrophy and progressive supranuclear palsy?

2021 ◽  
Author(s):  
Taku Homma ◽  
Yoko Mochizuki ◽  
Shinsuke Tobisawa ◽  
Takashi Komori ◽  
Kazushi Takahashi
Keyword(s):  
Multiple System Atrophy ◽  
Progressive Supranuclear Palsy ◽  
Neurodegenerative Disorder ◽  
Tufted Astrocyte

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 Multiple-System Atrophy in Long-Term Professional Painter: A Case Report

2012 ◽  
Vol 2012 ◽  
pp. 1-5
Author(s):  
Yusa Nagai ◽  
Riko Kitazawa ◽  
Miku Nakagawa ◽  
Munenori Komoda ◽  
Takeshi Kondo ◽  
...  
Keyword(s):  
Multiple System Atrophy ◽  
Medulla Oblongata ◽  
Neurodegenerative Disorder ◽  
Histopathological Examination ◽  
Neuronal Loss ◽  
Cytoplasmic Inclusion ◽  
Multiple Organ ◽  
Old Japanese ◽  
History Of

Introduction. Multiple system atrophy (MSA) is a rare and severe adult-onset, sporadic, and progressive neurodegenerative disorder. Here, we describe an autopsy case of MSA in a long-term professional painter. Although typical glial cytoplasmic inclusion (GCI) was not observed in a routine histological examination, strong α-synuclein immunostaining in the nucleus confirmed the diagnosis of MSA.Case Presentation. A 48-year-old Japanese man with a long occupational history of professional painter was sent to the emergency room, where he died of multiple organ failure. The patient had suffered tremors and inarticulateness at age 28, developed diabetes at 42 and was diagnosed with spinocerebellar degeneration at 46. A histopathological examination showed severe neuronal loss, gliosis, and tissue rarefaction in the paleostriatum, striate body of the substantia nigra, the pons, and the olivary nucleus of the upper medulla oblongata, intermediolateral of the spinal gray matter (sacral region). α-synuclein-positive GCI in oligodendroglia was occurred in the cerebral cortex, the midbrain, the medulla oblongata, and the spinal cord. These findings confirmed the presence of multiple-system atrophy (OPCA+SDS).Conclusion. Although the pathogenesis of MSA is still unclear, prolonged, and extensive exposure to organic solvents, together with a hyperglycemic morbidity attributed to diabetes, may have contributed to the onset and clinical course of the present case.

scholarly journals Differentiating Progressive Supranuclear Palsy and Parkinson's Disease With Head-Mounted Displays

2021 ◽  
Vol 12 ◽  
Author(s):  
Arvid Herwig ◽  
Almedin Agic ◽  
Hans-Jürgen Huppertz ◽  
Randolf Klingebiel ◽  
Frédéric Zuhorn ◽  
...  
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Eye Movements ◽  
Progressive Supranuclear Palsy ◽  
Neurodegenerative Disorder ◽  
High Sensitivity ◽  
Brain Regions ◽  
Inpatient Setting ◽  
Head Mounted Displays ◽  
Diagnostic Index

Background: Progressive supranuclear palsy (PSP) is a neurodegenerative disorder that, especially in the early stages of the disease, is clinically difficult to distinguish from Parkinson's disease (PD).Objective: This study aimed at assessing the use of eye-tracking in head-mounted displays (HMDs) for differentiating PSP and PD.Methods: Saccadic eye movements of 13 patients with PSP, 15 patients with PD, and a group of 16 healthy controls (HCs) were measured. To improve applicability in an inpatient setting and standardize the diagnosis, all the tests were conducted in a HMD. In addition, patients underwent atlas-based volumetric analysis of various brain regions based on high-resolution MRI.Results: Patients with PSP displayed unique abnormalities in vertical saccade velocity and saccade gain, while horizontal saccades were less affected. A novel diagnostic index was derived, multiplying the ratios of vertical to horizontal gain and velocity, allowing segregation of PSP from PD with high sensitivity (10/13, 77%) and specificity (14/15, 93%). As expected, patients with PSP as compared with patients with PD showed regional atrophy in midbrain volume, the midbrain plane, and the midbrain tegmentum plane. In addition, we found for the first time that oculomotor measures (vertical gain, velocity, and the diagnostic index) were correlated significantly to midbrain volume in the PSP group.Conclusions: Assessing eye movements in a HMD provides an easy to apply and highly standardized tool to differentiate PSP of patients from PD and HCs, which will aid in the diagnosis of PSP.

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