Corticobasal degeneration, progressive supranuclear palsy, multiple system atrophy, argyrophilic grain disease, and rarer neurodegenerative diseases

2016 ◽  
pp. 413-424
Author(s):  
Elizabeth A. Coon ◽  
Keith A. Josephs
Keyword(s):  
Multiple System Atrophy ◽  
Neurodegenerative Diseases ◽  
Progressive Supranuclear Palsy ◽  
Corticobasal Degeneration ◽  
Argyrophilic Grain Disease ◽  
Argyrophilic Grain

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 Progressive Supranuclear Palsy: A Review of Co-existing Neurodegeneration

2008 ◽  
Vol 35 (5) ◽  
pp. 602-608 ◽  
Author(s):  
J Keith-Rokosh ◽  
L C Ang
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Cognitive Impairment ◽  
Neurodegenerative Diseases ◽  
Progressive Supranuclear Palsy ◽  
Lewy Bodies ◽  
Corticobasal Degeneration ◽  
Pathological Changes ◽  
Subcortical Regions ◽  
Argyrophilic Grains

Objectives:The neuropathological findings of 32 progressive supranuclear palsy (PSP) cases over a period of 17 years were reviewed.Results:Of the 26 cases with adequate clinical data, 20 patients either presented with cognitive dysfunction or developed a cognitive impairment subsequently during the course of the disease. Co-existing changes of argyrophilic grains and corticobasal degeneration (CBD) were found in 28% and 32% of the cases respectively. Alzheimer-related pathology was found in 69% of cases but only 18.75% of cases fulfilled the consortium to establish a registry for Alzheimer's disease (CERAD) criteria for either definite or probable Alzheimer's disease. Lewy bodies were noted in four cases (12.5%), all in the subcortical regions. Only seven cases of PSP showed no pathological evidence of other co-existing neurodegenerative diseases. The severity of the cerebrovascular pathology in this cohort was insufficient to explain any clinical symptomatology.Conclusions:As in previous studies, this study has demonstrated the frequent co-existence of pathological changes usually noted in other neurodegenerative diseases in PSP. Whether these coexisting pathological changes contribute to the cognitive impairment in PSP remains uncertain.

Parkinson’s disease and parkinsonism

2016 ◽  
Author(s):  
Sathiji Nageshwaran ◽  
Heather C Wilson ◽  
Anthony Dickenson ◽  
David Ledingham
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Multiple System Atrophy ◽  
Progressive Supranuclear Palsy ◽  
Withdrawal Syndrome ◽  
Impulse Control ◽  
Serotonin Syndrome ◽  
Corticobasal Degeneration ◽  
Evidence Based ◽  
Pharmacological Management

This chapter discusses the evidence-based pharmacological management of motor and non-motor (autonomic disease, dementia, psychosis, depression, and sleep disorder) Parkinson’s disease (PD) and Parkinson’s plus syndromes (progressive supranuclear palsy (PSP), multiple system atrophy (MSA), and corticobasal degeneration (CBD)). Drugs to use with caution in parkinsonism are highlighted. Clinical features and evidence-based management of impulse control disorders (ICDs), serotonin syndrome, dopamine agonist withdrawal syndrome (DAWS), and neuroleptic malignant syndrome (NMS)/Parkinson’s hyperpyrexia syndrome (PHS) are also reviewed.

Orofacial apraxia in corticobasal degeneration, progressive supranuclear palsy, multiple system atrophy and Parkinson?s disease

2004 ◽  
Vol 251 (11) ◽  
pp. 1317-1323 ◽  
Author(s):  
Canan Ozsancak ◽  
Pascal Auzou ◽  
Kathy Dujardin ◽  
Niall Quinn ◽  
Alain Dest�e
Keyword(s):  
Multiple System Atrophy ◽  
Progressive Supranuclear Palsy ◽  
Corticobasal Degeneration
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