Movement Disorders 2: Parkinson’s Plus and Degenerative Diseases (DRAFT)

2018 ◽  
Author(s):  
Tamara Kaplan ◽  
Tracey Milligan
Keyword(s):  
Huntington's Disease ◽  
Progressive Supranuclear Palsy ◽  
Movement Disorders ◽  
Dementia With Lewy Bodies ◽  
Wilson’S Disease ◽  
Lewy Bodies ◽  
Corticobasal Degeneration ◽  
Degenerative Diseases ◽  
Multiple Systems ◽  
Multiple Systems Atrophy

The video in this chapter explores movement disorders, and focuses on Parkinson’s Plus and degenerative diseases. It outlines the features and pathology of dementia with lewy bodies (DLB), progressive supranuclear palsy (PSP), multiple systems atrophy (MSA) and corticobasal degeneration (CBD), as well as genetic movement disorders, Wilson’s disease, and Huntington’s disease.

Parkinsonism and Related Dementias

2021 ◽  
pp. 680-688
Author(s):  
Rodolfo Savica ◽  
Pierpaolo Turcano ◽  
Bradley F. Boeve
Keyword(s):  
Differential Diagnosis ◽  
Parkinson Disease ◽  
Progressive Supranuclear Palsy ◽  
Frontotemporal Dementia ◽  
Lewy Body ◽  
Dementia With Lewy Bodies ◽  
Lewy Bodies ◽  
Corticobasal Degeneration ◽  
Corticobasal Syndrome ◽  
Progressive Course

The differential diagnosis for dementia is wide. A slowly progressive course for parkinsonism suggests a degenerative cause and helps to narrow the differential diagnosis considerably. In patients with dementia in combination with parkinsonism (often collectively termed the parkinsonism-related dementias), the 4 most common neurodegenerative entities are 1) Lewy body dementias (which include dementia with Lewy bodies and Parkinson disease with dementia); 2) corticobasal syndrome or corticobasal degeneration; 3) Richardson syndrome or progressive supranuclear palsy; and 4) frontotemporal dementia with parkinsonism.

Amyloid and glucose imaging in dementia with Lewy bodies and multiple systems atrophy

2011 ◽  
Vol 17 (3) ◽  
pp. 160-165 ◽  
Author(s):  
Daniel O. Claassen ◽  
Val J. Lowe ◽  
Patrick J. Peller ◽  
Ronald C. Petersen ◽  
Keith A. Josephs
Keyword(s):  
Dementia With Lewy Bodies ◽  
Lewy Bodies ◽  
Multiple Systems ◽  
Multiple Systems Atrophy

scholarly journals REM sleep behavior in Parkinson disease: Frequent, particularly with higher age

PLoS ONE ◽  
2020 ◽  
Vol 15 (12) ◽  
pp. e0243454
Author(s):  
Heide Baumann-Vogel ◽  
Hyun Hor ◽  
Rositsa Poryazova ◽  
Philipp Valko ◽  
Esther Werth ◽  
...  
Keyword(s):  
Multiple System Atrophy ◽  
Parkinson Disease ◽  
Progressive Supranuclear Palsy ◽  
Rem Sleep ◽  
Dementia With Lewy Bodies ◽  
Lewy Bodies ◽  
Rem Sleep Behavior Disorder ◽  
Behavior Disorder ◽  
Corticobasal Degeneration ◽  
Sleep Behavior

This retrospective single-center polysomnography-based study was designed to assess the frequency of REM sleep behavior disorder (RBD) in consecutive patients with Parkinsonism, including Parkinson disease, dementia with Lewy bodies, multiple system atrophy, progressive supranuclear palsy, and corticobasal degeneration. We observed RBD in 77% of 540 Parkinson patients, with rising frequency at higher age and regardless of sex, in >89% of 89 patients with dementia with Lewy bodies or multiple system atrophy, and in <15% of 42 patients with progressive supranuclear palsy or corticobasal degeneration. Thus, the prevalence of RBD in sporadic Parkinson disease might be higher than previously assumed, particularly in elderly patients.

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.

Neuropathology

2020 ◽  
pp. 77-98
Author(s):  
Johannes Attems ◽  
Kurt A. Jellinger
Keyword(s):  
Frontotemporal Lobar Degeneration ◽  
Dementia With Lewy Bodies ◽  
Prion Diseases ◽  
Hippocampal Sclerosis ◽  
Neurofibrillary Tangle ◽  
Lewy Bodies ◽  
Corticobasal Degeneration ◽  
Amyloid Angiopathy ◽  
Argyrophilic Grain ◽  
Cerebral Amyloid

This chapter describes the main neuropathological features of the most common age-associated neurodegenerative diseases, including Alzheimer’s disease, Parkinson’s disease, and dementia with Lewy bodies, as well as other less frequent ones such as multiple system atrophy, Pick’s disease, corticobasal degeneration, progressive supranuclear palsy, argyrophilic grain disease, neurofibrillary tangle-dominant dementia, frontotemporal lobar degeneration with TDP-43 pathology, and Huntington’s disease. Likewise, cerebral amyloid angiopathy, hippocampal sclerosis, vascular dementia, and prion diseases are described. A main aim of this chapter is to assist the reader in interpreting neuropathological reports; hence criteria for the neuropathological classifications of the major diseases are provided. One section covers general considerations on neurodegeneration, and basic pathophysiological mechanisms of tau, amyloid-β‎, α‎-synuclein, TDP-43, and prions are briefly described in the sections on the respective diseases. Finally, one section is dedicated to cerebral multimorbidity, and a view on currently emerging neuropathological methods is given.

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.

scholarly journals Dementia With Lewy Bodies: the Principles of Diagnostics, Treatment, and Management

Medicina ◽  
2012 ◽  
Vol 48 (1) ◽  
pp. 1 ◽  
Author(s):  
Jūratė Macijauskienė ◽  
Vita Lesauskaitė
Keyword(s):  
Dementia With Lewy Bodies ◽  
Management Strategies ◽  
Laboratory Data ◽  
Lewy Bodies ◽  
Corticobasal Degeneration ◽  
Later Life ◽  
Alpha Synuclein ◽  
Diagnostic Features ◽  
Protein Deposits ◽  
Diagnosis Of Dementia

Dementia with Lewy bodies was first recognized as a separate entity about 30 years ago. The prevalence varies from 0% to 5% in the general population, and this disease accounts for 0% to 30.5% of all dementia cases. Dementia with Lewy bodies is considered the second most common cause of degenerative dementia after Alzheimer’s disease. The disease is characterized by alpha-synuclein immunoreactive protein deposits in both neurons and glial cells. The protein deposits are especially prominent in dopaminergic neurons, where they can be detected using conventional histological stains, such as hematoxylin and eosin, and are commonly referred to as Lewy bodies. The diagnosis of dementia with Lewy bodies is based on the presence of dementia as well as 2 of the following 3 core diagnostic features: 1) fluctuating cognition, 2) visual hallucinations, and 3) movement disorder. Diagnostic tests include laboratory data, structural and functional imaging, and electroencephalography. Differential diagnosis of dementia with Lewy bodies focuses on other later life dementia syndromes, other parkinsonian diseases (Parkinson’s disease, progressive supranuclear palsy, corticobasal degeneration), and primary psychiatric illnesses. There is type 1b evidence to support treatment with cholinesterase inhibitors. Glutamatergic and dopaminergic therapies are used as well. Standard neuroleptics are contraindicated, and atypical agents should be used cautiously. Nonpharmacologic measures – therapeutic environment, psychological and social support, physical activity, behavioral management strategies, caregivers’ education and support, and different services – could be suggested.

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