Parkinsonism and Related Dementias

2021 ◽  
pp. 680-688
Author(s):  
Rodolfo Savica ◽  
Pierpaolo Turcano ◽  
Bradley F. Boeve

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.

PLoS ONE ◽  
2020 ◽  
Vol 15 (12) ◽  
pp. e0243454
Author(s):  
Heide Baumann-Vogel ◽  
Hyun Hor ◽  
Rositsa Poryazova ◽  
Philipp Valko ◽  
Esther Werth ◽  
...  

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.


Author(s):  
Tamara Kaplan ◽  
Tracey Milligan

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.


Neurology ◽  
2018 ◽  
Vol 91 (3) ◽  
pp. e268-e279 ◽  
Author(s):  
Koji Kasanuki ◽  
Keith A. Josephs ◽  
Tanis J. Ferman ◽  
Melissa E. Murray ◽  
Shunsuke Koga ◽  
...  

ObjectiveTo describe clinical and pathologic characteristics of diffuse Lewy body disease (DLBD) manifesting as corticobasal syndrome (CBS).MethodsIn 523 autopsy-confirmed cases of DLBD, we identified 11 patients diagnosed with CBS. For comparison, we studied 22 DLBD brains with antemortem presentation of dementia with Lewy bodies (DLB). Given previous studies suggesting the importance of pathology in peri-Rolandic cortices in CBS, we used digital pathology to count Lewy bodies and to quantify intracytoplasmic and neuritic α-synuclein and phospho-tau burden in the motor cortex.ResultsDLBD patients with antemortem features of CBS were significantly younger at disease onset and less likely to have REM sleep behavior disorder than DLBD cases who met clinical criteria for DLB during life. Patients with DLBD manifesting as CBS had more Lewy bodies in the motor cortex than DLBD manifesting as clinically probable DLB. Three cases had concomitant progressive supranuclear palsy and 4 cases had concomitant Alzheimer disease as probable correlates of CBS.ConclusionThe neuropathology underlying CBS is heterogeneous, including corticobasal degeneration, Alzheimer disease, and progressive supranuclear palsy. This study suggests that atypical variants of Lewy body disease with severe peri-Rolandic Lewy-related pathology can present clinically as CBS. Patients with DLBD who present as CBS tend to have an earlier age at onset and are less likely to have clinical features of DLB, such as dream enactment behavior during sleep, visual hallucinations, and levodopa-responsive parkinsonism. Future studies with biofluid or molecular imaging biomarkers for α-synuclein will permit better recognition of this uncommon pathologic substrate of CBS.


2019 ◽  
Author(s):  
Elizabeth J. Slow ◽  
Anthony E. Lang

Parkinsonism describes the core clinical criteria of tremor, bradykinesia, rigidity, and postural instability. There is a large differential diagnosis, but the most common cause of parkinsonism is due to Parkinson disease. This review details the epidemiology, etiology/genetics, pathogenesis, diagnosis and differential diagnosis, management, and prognosis of Parkinson disease, dementia with Lewy bodies, progressive supranuclear palsy, corticobasal degeneration, vascular parkinsonism, normal pressure hydrocephalus, and drug-induced parkinsonism.  This review contains 8 figures, 32 tables, and 73 references. Keywords: Parkinson disease, parkinsonism, levodopa, cogwheel ridigity, multiple system atrophy, dementia, substantia nigra, palsy, neurodegenerative disease, hydrocephalus, Lewy body, Lewy neurite


2019 ◽  
Author(s):  
Elizabeth J. Slow ◽  
Anthony E. Lang

Parkinsonism describes the core clinical criteria of tremor, bradykinesia, rigidity, and postural instability. There is a large differential diagnosis, but the most common cause of parkinsonism is due to Parkinson disease. This review details the epidemiology, etiology/genetics, pathogenesis, diagnosis and differential diagnosis, management, and prognosis of Parkinson disease, dementia with Lewy bodies, progressive supranuclear palsy, corticobasal degeneration, vascular parkinsonism, normal pressure hydrocephalus, and drug-induced parkinsonism.  This review contains 8 figures, 32 tables, and 73 references. Keywords: Parkinson disease, parkinsonism, levodopa, cogwheel ridigity, multiple system atrophy, dementia, substantia nigra, palsy, neurodegenerative disease, hydrocephalus, Lewy body, Lewy neurite


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