scholarly journals Genetic Architecture and Molecular, Imaging and Prodromic Markers in Dementia with Lewy Bodies: State of the Art, Opportunities and Challenges

2021 ◽  
Vol 22 (8) ◽  
pp. 3960
Author(s):  
Romina Combi ◽  
Maria Salsone ◽  
Chiara Villa ◽  
Luigi Ferini-Strambi
Keyword(s):  
Dementia With Lewy Bodies ◽  
Genetic Architecture ◽  
State Of The Art ◽  
Imaging Techniques ◽  
Lewy Bodies ◽  
Diagnostic Methods ◽  
Clinical Observations ◽  
Common Causes ◽  
Common Risk Factors ◽  
Clinical Differential Diagnosis

Dementia with Lewy bodies (DLB) is one of the most common causes of dementia and belongs to the group of α-synucleinopathies. Due to its clinical overlap with other neurodegenerative disorders and its high clinical heterogeneity, the clinical differential diagnosis of DLB from other similar disorders is often difficult and it is frequently underdiagnosed. Moreover, its genetic etiology has been studied only recently due to the unavailability of large cohorts with a certain diagnosis and shows genetic heterogeneity with a rare contribution of pathogenic mutations and relatively common risk factors. The rapid increase in the reported cases of DLB highlights the need for an easy, efficient and accurate diagnosis of the disease in its initial stages in order to halt or delay the progression. The currently used diagnostic methods proposed by the International DLB consortium rely on a list of criteria that comprises both clinical observations and the use of biomarkers. Herein, we summarize the up-to-now reported knowledge on the genetic architecture of DLB and discuss the use of prodromal biomarkers as well as recent promising candidates from alternative body fluids and new imaging techniques.

Dementia with Lewy bodies and Parkinson’s disease dementia

2013 ◽  
Author(s):  
Arvid Rongve ◽  
Dag Aarsland
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Dementia With Lewy Bodies ◽  
Clinical Symptoms ◽  
Imaging Techniques ◽  
Lewy Bodies ◽  
Epidemiological Data ◽  
Parkinson’S Disease Dementia ◽  
Esterase Inhibitors ◽  
Staging Systems

Dementia with Lewy bodies and Parkinson’s disease dementia belong to the α-synucleinopathies, a family of diseases pathologically characterized by aggregation of α-synuclein in Lewy bodies in the brain. In this chapter we present the epidemiological data for both conditions including new data on MCI. Clinical diagnostic criteria are reviewed and the different neuropathology staging systems for DLB and PDD and the most important genetic findings are considered. Biomarkers in DLB and PDD with particular focus on imaging techniques like CIT-SPECT and MRI are described. Important clinical symptoms in both conditions are presented in detail and the most important clinical differential diagnoses are discussed. Pharmacological and non- pharmacological treatment of different symptoms in both conditions are discussed with particular emphasis on the choline esterase inhibitors and antipsychotic medications.New data on memantine are presented.

How to diagnose dementia with Lewy bodies: State of the art

2005 ◽  
Vol 20 (S12) ◽  
pp. S11-S20 ◽  
Author(s):  
Felix Geser ◽  
Gregor K. Wenning ◽  
Werner Poewe ◽  
Ian McKeith
Keyword(s):  
Dementia With Lewy Bodies ◽  
State Of The Art ◽  
Lewy Bodies

The Lewy body dementias

2020 ◽  
pp. 495-512
Author(s):  
Arvid Rongve ◽  
Dag Aarsland
Keyword(s):  
Dementia With Lewy Bodies ◽  
Clinical Symptoms ◽  
Imaging Techniques ◽  
Lewy Bodies ◽  
Epidemiological Data ◽  
Antipsychotic Medications ◽  
Esterase Inhibitors ◽  
Staging Systems ◽  
The Brain ◽  
Clinical Diagnostic Criteria

Dementia with Lewy bodies (DLB) and Parkinson’s disease dementia (PDD) belong to the α‎-synucleinopathies, a family of diseases pathologically characterized by aggregation of α‎-synuclein in Lewy bodies in the brain. This chapter presents the epidemiological data for both conditions including new data on MCI. It reviews clinical diagnostic criteria and considers the different neuropathology staging systems for DLB and PDD and the most important genetic findings. It describes biomarkers in DLB and PDD with particular focus on imaging techniques like CIT-SPECT and MRI. It presents in detail important clinical symptoms in both conditions and discusses the most important clinical differential diagnoses. Finally, it examines pharmacological and non- pharmacological treatment of different symptoms in both conditions, with particular emphasis on the choline esterase inhibitors and antipsychotic medications, and presents new data on memantine.

scholarly journals Investigating the genetic architecture of dementia with Lewy bodies: a two-stage genome-wide association study

2018 ◽  
Vol 17 (1) ◽  
pp. 64-74 ◽  
Author(s):  
Rita Guerreiro ◽  
Owen A Ross ◽  
Celia Kun-Rodrigues ◽  
Dena G Hernandez ◽  
Tatiana Orme ◽  
...  
Keyword(s):  
Association Study ◽  
Dementia With Lewy Bodies ◽  
Genetic Architecture ◽  
Genome Wide Association Study ◽  
Lewy Bodies ◽  
Genome Wide Association ◽  
Two Stage ◽  
Genome Wide

What is dementia?

2021 ◽  
pp. 3-10
Author(s):  
Andrew E. Budson ◽  
Maureen K. O’Connor
Keyword(s):  
Primary Care ◽  
Dementia With Lewy Bodies ◽  
Lewy Bodies ◽  
Primary Progressive Aphasia ◽  
Normal Pressure ◽  
Primary Care Providers ◽  
Care Providers ◽  
Common Cause ◽  
Common Causes ◽  
Daily Function

Dementia is the term used to describe progressive impairment of thinking and memory that interferes with daily function. Dementia is not a specific disorder; rather, it is a condition with many causes. Alzheimer’s disease is the most common cause of dementia. Other common causes of dementia include vascular dementia, dementia with Lewy bodies, primary progressive aphasia which comes in the logopenic, semantic, and nonfluent/agrammatic variants, behavioral variant frontotemporal dementia, and normal pressure hydrocephalus. Primary care providers are able to diagnose most straightforward cases of dementia, whereas specialists such as neuropsychologists, neurologists, psychiatrists, and geriatricians may be needed when the diagnosis is not straightforward.

What is dementia?

2021 ◽  
pp. 3-10
Author(s):  
Andrew E. Budson ◽  
Maureen K. O’Connor
Keyword(s):  
Primary Care ◽  
Dementia With Lewy Bodies ◽  
Lewy Bodies ◽  
Primary Progressive Aphasia ◽  
Normal Pressure ◽  
Primary Care Providers ◽  
Care Providers ◽  
Common Cause ◽  
Common Causes ◽  
Daily Function

Dementia is the term used to describe progressive impairment of thinking and memory that interferes with daily function. Dementia is not a specific disorder; rather, it is a condition with many causes. Alzheimer’s disease is the most common cause of dementia. Other common causes of dementia include vascular dementia, dementia with Lewy bodies, primary progressive aphasia which comes in the logopenic, semantic, and nonfluent/agrammatic variants, behavioral variant frontotemporal dementia, and normal pressure hydrocephalus. Primary care providers are able to diagnose most straightforward cases of dementia, whereas specialists such as neuropsychologists, neurologists, psychiatrists, and geriatricians may be needed when the diagnosis is not straightforward.

scholarly journals Analysis of Protein Conformational Strains—A Key for New Diagnostic Methods of Human Diseases

2020 ◽  
Vol 21 (8) ◽  
pp. 2801 ◽  
Author(s):  
Andrei Surguchov
Keyword(s):  
Differential Diagnosis ◽  
Protein Misfolding ◽  
Dementia With Lewy Bodies ◽  
Lewy Bodies ◽  
Diagnostic Methods ◽  
Human Diseases ◽  
Spatial Structures ◽  
Unfolded Protein ◽  
Methods Of Treatment ◽  
Early Diagnostic

α-Synuclein is a naturally unfolded protein which easily aggregates and forms toxic inclusions and deposits. It is associated with several neurodegenerative diseases, including Parkinson’s disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA). These diseases, called synucleinopathies, have overlapping symptoms but require different methods of treatment. There are no reliable approaches for early diagnoses of these diseases, and as a result, the treatment begins late, and the disorders are often misdiagnosed. Recent studies revealed that α-synuclein forms distinctive spatial structures or strains at the early steps of these diseases, which may be used for early diagnosis. One of these early diagnostic methods called PMCA (protein misfolding cyclic amplification) allows identification of the distinct α-synuclein strains specific for different human diseases. The method is successfully used for differential diagnosis of patients with PD and MSA.

Dementia with Lewy Bodies

1999 ◽  
Vol 33 (6) ◽  
pp. 800-808 ◽  
Author(s):  
Ian McKeith ◽  
John O'Brien
Keyword(s):  
Cognitive Impairment ◽  
Dementia With Lewy Bodies ◽  
Single Photon ◽  
Photon Emission ◽  
Lewy Bodies ◽  
High Specificity ◽  
The Elderly ◽  
Diagnostic Methods ◽  
Visual Hallucinations ◽  
Clinical Criteria

Objective: The aim of this paper is to summarise recent clinical and research findings with regard to dementia with Lewy bodies (DLB). Method: A literature review (Medline) was carried out, as well as a review of reports of recent DLB symposia of international meetings and of other relevant papers and data known to the authors. Results: Dementia with Lewy bodies, as the disorder should be known, is the second commonest form of degenerative dementia, accounting for up to 20% cases in the elderly. It is characterised by fluctuating cognitive impairment, spontaneous parkinsonism and recurrent visual hallucinations. Consensus clinical and neuro-pathological criteria have been published. The clinical criteria have been shown to have high specificity, but may still lack sensitivity. Recognition of DLB is clinically important in view of the high incidence (60%) of adverse and life-threatening reaction to antipsychotics, the difference in prognosis and, possibly, the differential treatment response to cholinergic therapy. Neuroimaging changes have not been well described in DLB but some show promise as potential markers to differentiate DLB from AD. These include relative preservation of temporal lobe structures on magnetic resonance imaging and loss of pre- and postsynaptic dopaminergic markers on single photon emission tomography. Conclusions: Dementia with Lewy bodies is a common cause of cognitive impairment in late life which appears to be clinically and neuropathologically distinct from AD. All clinicians should be aware of the typical triad of clinical features (fluctuating cognitive impairment, visual hallucinations and parkinsonism) which characterise the disorder and either avoid antipsychotics or prescribe them with extreme caution in such patients. Further research is likely to result in advances in diagnostic methods and therapeutics in the near future.

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