scholarly journals Capgras syndrome in dementia with Lewy bodies: a possible association of severe cortical Lewy body pathology

2021 ◽  
Author(s):  
Shunsuke Koga ◽  
Dennis W. Dickson ◽  
Zbigniew K. Wszolek
Keyword(s):  
Lewy Body ◽  
Dementia With Lewy Bodies ◽  
Lewy Bodies ◽  
Lewy Body Pathology ◽  
Capgras Syndrome

“I See Them Sitting on My Bed, Doctor”

2016 ◽  
Author(s):  
Susan H. Fox ◽  
Marina Picillo
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Cognitive Impairment ◽  
Lewy Body ◽  
Dementia With Lewy Bodies ◽  
Lewy Bodies ◽  
Body Burden ◽  
The Other ◽  
Lewy Body Pathology ◽  
Over Time

Many movement disorders specialists would consider dementia with Lewy bodies to be on one end of a spectrum of presentations associated with Lewy body pathology. Parkinson’s disease with preserved cognition sits at the other end of the spectrum, and over time it can be associated with a greater cortical Lewy body burden and related cognitive impairment. It is unclear what determines to what degree cognitive impairment is involved in a given patient, and the arbitrary time-based division between Parkinson’s disease and dementia with Lewy bodies is imperfect, although it serves to help recognition of this group of patients in whom management needs to be appropriately tailored.

Characterization of dementia with Lewy bodies (DLB) and mild cognitive impairment using the Lewy body dementia module (LBD‐MOD)

2021 ◽  
Author(s):  
James E. Galvin ◽  
Stephanie Chrisphonte ◽  
Iris Cohen ◽  
Keri K. Greenfield ◽  
Michael J. Kleiman ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
Mild Cognitive Impairment ◽  
Lewy Body ◽  
Dementia With Lewy Bodies ◽  
Lewy Bodies ◽  
Lewy Body Dementia

Clinical Aspects of Non-Alzheimer Disease Dementias

2017 ◽  
Author(s):  
David Knopman
Keyword(s):  
Cognitive Impairment ◽  
Alzheimer Disease ◽  
Cerebrovascular Disease ◽  
Lewy Body ◽  
Dementia With Lewy Bodies ◽  
Lewy Bodies ◽  
Lewy Body Disease ◽  
Clinical Aspects ◽  
Key Words Dementia ◽  
Sleep Behavior

There are a relatively small number of disorders that account for the majority of dementia in the elderly that is not Alzheimer disease (AD): cerebrovascular disease, Lewy body disease (α-synucleinopathies), and the frontotemporal lobar degenerations. Cerebrovascular disease and Lewy body disease account for most non-AD dementia among persons in the eighth decade of life and beyond. These two frequently co-occur with AD but can occur in their pure forms rarely (in the case of dementia associated with cerebrovascular disease) or more commonly (in the case of Lewy body disease). There is no one cognitive or behavioral syndrome associated with cerebrovascular disease; however, attempts to isolate a common theme suggest that cognitive slowing is typical of cerebrovascular contributions to cognitive impairment. Cerebrovascular pathology relevant to cognitive impairment accumulates subclinically more commonly than it causes acute, strokelike declines in cognition. Dementia with Lewy bodies is a multidimensional disorder that includes a nonamnestic dementia, Parkinson disease or at least some parkinsonian features, a disorder of sleep and wakefulness, autonomic disturbances, and depression. The disorders of sleep prominently include rapid eye movement sleep behavior disorder, excessive daytime sleepiness, visual hallucinations, and marked fluctuations in level of alertness. The frontotemporal lobar degenerations are nearly as common as causes of dementia in persons under age 65 as is AD. The group of disorders includes two cognitive syndromes (primary progressive aphasia and behavior variant frontotemporal dementia) and two neuropathologic subtypes (tauopathy and TDP43 proteinopathy) and is associated with three major autosomal dominant genetic mutations (in MAPT, GRN, and C9ORF72). Key words: dementia with Lewy bodies, frontotemporal lobar degenerations, vascular cognitive impairment

scholarly journals A toxic subset of soluble α-synuclein species in dementia with Lewy body

2020 ◽  
Vol 2 (1) ◽  
Author(s):  
Diana L Castillo-Carranza
Keyword(s):  
Cerebral Cortex ◽  
Lewy Body ◽  
Dementia With Lewy Bodies ◽  
Dementia With Lewy Body ◽  
Lewy Bodies

This scientific commentary refers to ‘Analysis of α-synuclein species enriched from cerebral cortex of humans with sporadic dementia with Lewy bodies’, by Sanderson et al. (https://doi.org/10.1093/braincomms/fcaa010).

scholarly journals Diagnostic accuracy of dopaminergic imaging in prodromal dementia with Lewy bodies

2018 ◽  
Vol 49 (3) ◽  
pp. 396-402 ◽  
Author(s):  
Alan J. Thomas ◽  
Paul Donaghy ◽  
Gemma Roberts ◽  
Sean J. Colloby ◽  
Nicky A. Barnett ◽  
...  
Keyword(s):  
Diagnostic Accuracy ◽  
Lewy Body ◽  
Dementia With Lewy Bodies ◽  
Lewy Bodies ◽  
Lewy Body Disease ◽  
High Specificity ◽  
Diagnostic Confidence ◽  
Neuropsychological Assessments ◽  
Diagnostic Symptoms ◽  
Prodromal Dementia

AbstractBackgroundDopaminergic imaging has high diagnostic accuracy for dementia with Lewy bodies (DLB) at the dementia stage. We report the first investigation of dopaminergic imaging at the prodromal stage.MethodsWe recruited 75 patients over 60 with mild cognitive impairment (MCI), 33 with probable MCI with Lewy body disease (MCI-LB), 15 with possible MCI-LB and 27 with MCI with Alzheimer's disease. All underwent detailed clinical, neurological and neuropsychological assessments and FP-CIT [123I-N-fluoropropyl-2β-carbomethoxy-3β-(4-iodophenyl)] dopaminergic imaging. FP-CIT scans were blindly rated by a consensus panel and classified as normal or abnormal.ResultsThe sensitivity of visually rated FP-CIT imaging to detect combined possible or probable MCI-LB was 54.2% [95% confidence interval (CI) 39.2–68.6], with a specificity of 89.0% (95% CI 70.8–97.6) and a likelihood ratio for MCI-LB of 4.9, indicating that FP-CIT may be a clinically important test in MCI where any characteristic symptoms of Lewy body (LB) disease are present. The sensitivity in probable MCI-LB was 61.0% (95% CI 42.5–77.4) and in possible MCI-LB was 40.0% (95% CI 16.4–67.7).ConclusionsDopaminergic imaging had high specificity at the pre-dementia stage and gave a clinically important increase in diagnostic confidence and so should be considered in all patients with MCI who have any of the diagnostic symptoms of DLB. As expected, the sensitivity was lower in MCI-LB than in established DLB, although over 50% still had an abnormal scan. Accurate diagnosis of LB disease is important to enable early optimal treatment for LB symptoms.

scholarly journals 18F-fluorodeoxyglucose positron emission tomography in dementia with Lewy bodies

2020 ◽  
Vol 2 (1) ◽  
Author(s):  
Jonathan Graff-Radford ◽  
Timothy G Lesnick ◽  
Rodolfo Savica ◽  
Qin Chen ◽  
Tanis J Ferman ◽  
...  
Keyword(s):  
Lewy Body ◽  
Dementia With Lewy Bodies ◽  
Rating Scale ◽  
Clinical Course ◽  
Lewy Bodies ◽  
Lewy Body Disease ◽  
Diffuse Lewy Body Disease ◽  
18F Fluorodeoxyglucose ◽  
Alzheimer’S Pathology ◽  
Fluorodeoxyglucose Pet

Abstract Among individuals with dementia with Lewy bodies, pathologic correlates of clinical course include the presence and extent of coexisting Alzheimer’s pathology and the presence of transitional or diffuse Lewy body disease. The objectives of this study are to determine (i) whether 18F-fluorodeoxyglucose PET signature patterns of dementia with Lewy bodies are associated with the extent of coexisting Alzheimer’s pathology and the presence of transitional or diffuse Lewy body disease and (ii) whether these 18F-fluorodeoxyglucose pattern(s) are associated with clinical course in dementia with Lewy bodies. Two groups of participants were included: a pathology-confirmed subset with Lewy body disease (n = 34) and a clinically diagnosed group of dementia with Lewy bodies (n = 87). A subset of the clinically diagnosed group was followed longitudinally (n = 51). We evaluated whether 18F-fluorodeoxyglucose PET features of dementia with Lewy bodies (higher cingulate island sign ratio and greater occipital hypometabolism) varied by Lewy body disease subtype (transitional versus diffuse) and Braak neurofibrillary tangle stage. We investigated whether the PET features were associated with the clinical trajectories by performing regression models predicting Clinical Dementia Rating Scale Sum of Boxes. Among autopsied participants, there was no difference in cingulate island sign or occipital hypometabolism by Lewy body disease type, but those with a lower Braak tangle stage had a higher cingulate island sign ratio compared to those with a higher Braak tangle stage. Among the clinically diagnosed dementia with Lewy bodies participants, a higher cingulate island ratio was associated with better cognitive scores at baseline and longitudinally. A higher 18F-fluorodeoxyglucose PET cingulate island sign ratio was associated with lower Braak tangle stage at autopsy, predicted a better clinical trajectory in dementia with Lewy body patients and may allow for improved prognostication of the clinical course in this disease.

In dementia with Lewy bodies, Braak stage determines phenotype, not Lewy body distribution

Neurology ◽  
2007 ◽  
Vol 69 (4) ◽  
pp. 356-359 ◽  
Author(s):  
D. Weisman ◽  
M. Cho ◽  
C. Taylor ◽  
A. Adame ◽  
L. J. Thal ◽  
...  
Keyword(s):  
Lewy Body ◽  
Dementia With Lewy Bodies ◽  
Lewy Bodies ◽  
Braak Stage ◽  
Body Distribution

scholarly journals Insular cortex sub-region-dependent distribution pattern of α-synuclein immunoreactivity in Parkinson’s disease and dementia with Lewy bodies

2017 ◽  
Author(s):  
Yasmine Y. Fathy ◽  
Frank Jan de Jong ◽  
Anne-Marie van Dam ◽  
Annemieke J.M. Rozemuller ◽  
Wilma D.J. van de Berg
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Distribution Pattern ◽  
Lewy Body ◽  
Dementia With Lewy Bodies ◽  
Lewy Bodies ◽  
Insular Cortex ◽  
Disease Stage ◽  
Incidental Lewy Body Disease ◽  
Lewy Body Diseases

AbstractThe insular cortex is a heterogeneous and widely connected brain region. It plays a role in autonomic, cognitive, emotional and somatosensory functions. Its complex and unique cytoarchitecture includes a periallocortical agranular, pro-isocortical dysgranular, and isocortical granular sub-regions. In Parkinson’s disease (PD), the insula shows α-synuclein inclusions in advanced stages of the disease and its atrophy correlates with cognitive deficits. However, little is known regarding its regional neuropathological characteristics and vulnerability in Lewy body diseases. The aim of this study is to assess the distribution pattern of α-synuclein pathology in the insular sub-regions and the selective vulnerability of its different cell types in PD and dementia with Lewy bodies (DLB). Human post-mortem insular tissues from 10 donors with incidental Lewy body disease (iLBD), PD, DLB, and age-matched controls were immunostained for α-synuclein and glial fibrillary acid protein (GFAP). Results showed that a decreasing gradient of α-synuclein pathology was present from agranular to granular sub-regions in iLBD, PD and PD with dementia (PDD) donors. The agranular insula was heavily inflicted, revealing various α-synuclein immunoreactive morphological structures, predominantly Lewy neurites (LNs), and astroglial synucleinopathy. While dysgranular and granular sub-regions showed a decreasing gradient of inclusions and more Lewy bodies (LBs) in deeper layers. In DLB, this gradient was less pronounced and severe pathology was observed in the granular insula compared to PDD and regardless of disease stage. Protoplasmic astrocytes showed α-synuclein inclusions and severe degenerative changes increasing with disease severity. While few von Economo neurons (VENs) in the fronto-insular region revealed inclusions, particularly in PDD patients. Our study reports novel findings on the differential involvement of the insular sub-regions in PD and particular involvement of the agranular sub-region, VENs and astrocytes. Thus, the differential cellular architecture of the insular sub-regions portrays the topographic variation and vulnerability to α-synuclein pathology in Lewy body diseases.

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.

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