The role of FP-CIT in the differential diagnosis of Alzheimerʼs and Lewy body dementia

2001 ◽  
Vol 22 (3) ◽  
pp. 342
Author(s):  
D. C. Costa ◽  
Z. Walker ◽  
D. Visvikis ◽  
R. W.H. Walker ◽  
S. Gacinovic ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Lewy Body ◽  
Lewy Body Dementia

Dementia disorders

2017 ◽  
Author(s):  
Roland Zahn ◽  
Alistair Burns
Keyword(s):  
Differential Diagnosis ◽  
Autoimmune Diseases ◽  
Vascular Dementia ◽  
Lewy Body ◽  
Simple Algorithm ◽  
Lewy Body Dementia ◽  
Cortical Atrophy ◽  
Posterior Cortical Atrophy ◽  
Dsm 5

This chapter provides a brief overview of the different forms of dementia syndromes and provides a simple algorithm for initial differential diagnosis. Rapidly progressive dementias have to be excluded which require specific investigations to detect Creutzfeldt–Jakob as well as inflammatory and autoimmune diseases. A lead symptom-based approach in patients with slowly progressive cognitive and behavioural impairments without neurological symptoms is applied: progressive and primary impairments in recent memory are characteristic of typical Alzheimer’s dementia, primary behavioural changes point to the behavioural variant of frontotemporal dementia, primary impairments of language or speech are distinctive for progressive aphasias, fluctuating impairments of attention are a hallmark of Lewy body dementia, whereas primary visuospatial impairments suggest a posterior cortical atrophy. The chapter further discusses updated vascular dementia guidelines and DSM-5 revisions of defining dementia. Current diagnostic criteria for the different dementias are referenced and the role of neuroimaging is illustrated.

scholarly journals The emerging role of α-synuclein truncation in aggregation and disease

2020 ◽  
Vol 295 (30) ◽  
pp. 10224-10244 ◽  
Author(s):  
Zachary A. Sorrentino ◽  
Benoit I. Giasson
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Multiple System Atrophy ◽  
Lewy Body ◽  
Critical Role ◽  
Lewy Body Dementia ◽  
Proteolytic Processing ◽  
Neuronal Protein ◽  
Preventative Strategy

α-Synuclein (αsyn) is an abundant brain neuronal protein that can misfold and polymerize to form toxic fibrils coalescing into pathologic inclusions in neurodegenerative diseases, including Parkinson's disease, Lewy body dementia, and multiple system atrophy. These fibrils may induce further αsyn misfolding and propagation of pathologic fibrils in a prion-like process. It is unclear why αsyn initially misfolds, but a growing body of literature suggests a critical role of partial proteolytic processing resulting in various truncations of the highly charged and flexible carboxyl-terminal region. This review aims to 1) summarize recent evidence that disease-specific proteolytic truncations of αsyn occur in Parkinson's disease, Lewy body dementia, and multiple system atrophy and animal disease models; 2) provide mechanistic insights on how truncation of the amino and carboxyl regions of αsyn may modulate the propensity of αsyn to pathologically misfold; 3) compare experiments evaluating the prion-like properties of truncated forms of αsyn in various models with implications for disease progression; 4) assess uniquely toxic properties imparted to αsyn upon truncation; and 5) discuss pathways through which truncated αsyn forms and therapies targeted to interrupt them. Cumulatively, it is evident that truncation of αsyn, particularly carboxyl truncation that can be augmented by dysfunctional proteostasis, dramatically potentiates the propensity of αsyn to pathologically misfold into uniquely toxic fibrils with modulated prion-like seeding activity. Therapeutic strategies and experimental paradigms should operate under the assumption that truncation of αsyn is likely occurring in both initial and progressive disease stages, and preventing truncation may be an effective preventative strategy against pathologic inclusion formation.

scholarly journals Complex I reductions in the nucleus basalis of Meynert in Lewy body dementia: the role of Lewy bodies

2020 ◽  
Vol 8 (1) ◽  
Author(s):  
Christopher Hatton ◽  
Amy Reeve ◽  
Nichola Zoe Lax ◽  
Alasdair Blain ◽  
Yi Shiau Ng ◽  
...  
Keyword(s):  
Lewy Body ◽  
Complex I ◽  
Lewy Bodies ◽  
Lewy Body Dementia ◽  
Nucleus Basalis ◽  
Nucleus Basalis Of Meynert

scholarly journals Phenotypic Heterogeneity among GBA p.R202X Carriers in Lewy Body Spectrum Disorders

Biomedicines ◽  
2022 ◽  
Vol 10 (1) ◽  
pp. 160
Author(s):  
Valerio Napolioni ◽  
Carolyn A. Fredericks ◽  
Yongha Kim ◽  
Divya Channappa ◽  
Raiyan R. Khan ◽  
...  
Keyword(s):  
Lewy Body ◽  
Disease Onset ◽  
Disease Patient ◽  
Lewy Body Dementia ◽  
Phenotypic Heterogeneity ◽  
Pathogenic Role ◽  
Gaucher's Disease ◽  
Gaucher’S Disease ◽  
Gba Gene

We describe the clinical and neuropathologic features of patients with Lewy body spectrum disorder (LBSD) carrying a nonsense variant, c.604C>T; p.R202X, in the glucocerebrosidase 1 (GBA) gene. While this GBA variant is causative for Gaucher’s disease, the pathogenic role of this mutation in LBSD is unclear. Detailed neuropathologic evaluation was performed for one index case and a structured literature review of other GBA p.R202X carriers was conducted. Through the systematic literature search, we identified three additional reported subjects carrying the same GBA mutation, including one Parkinson’s disease (PD) patient with early disease onset, one case with neuropathologically-verified LBSD, and one unaffected relative of a Gaucher’s disease patient. Among the affected subjects carrying the GBA p.R202X, all males were diagnosed with Lewy body dementia, while the two females presented as PD. The clinical penetrance of GBA p.R202X in LBSD patients and families argues strongly for a pathogenic role for this variant, although presenting with a striking phenotypic heterogeneity of clinical and pathological features.

scholarly journals Improving diagnostic communication in dementia

2017 ◽  
Vol 30 (8) ◽  
pp. 1149-1152 ◽  
Author(s):  
Sheung-Tak Cheng ◽  
Linda C. W. Lam
Keyword(s):  
Differential Diagnosis ◽  
Lewy Body ◽  
Help Seeking ◽  
Language Barriers ◽  
Lewy Body Dementia ◽  
The World ◽  
The Usa ◽  
Proper Diagnosis

In many countries around the world, owing to the lack of specialists and equipment, delay up to a few years in help-seeking and getting diagnostic examinations for dementia is not uncommon (Sayegh and Knight, 2013), and this situation is considerably more serious in “atypical dementias” due to the challenge they present for differential diagnosis. For instance, a survey in the USA showed that misdiagnosis was common in patients with Lewy body dementia who, on average, saw at least three physicians over a year's time or more before getting the proper diagnosis (Lewy Body Dementia Association, 2010). Furthermore, in multiethnic communities, cultural and language barriers between practitioners and patients may lead to substantial delay as well (Sayegh and Knight, 2013).

Added Value of QEEG for the Differential Diagnosis of Common Forms of Dementia

2020 ◽  
pp. 155005942097112
Author(s):  
Livia Livinț Popa ◽  
Hanna-Maria Dragoș ◽  
Ștefan Strilciuc ◽  
Cristina Pantelemon ◽  
Ioana Mureșanu ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Lewy Body Dementia ◽  
Quantitative Eeg ◽  
Added Value ◽  
Signal Acquisition ◽  
Imaging Features ◽  
Diagnosis Of Dementia ◽  
Quantitative Analyses ◽  
Signal Acquisition And Processing

Introduction Quantitative electroencephalography (QEEG) has been documented as a helpful tool in the differential diagnosis of Alzheimer’s disease (AD) with common forms of dementia. The main objective of the study was to assess the role of QEEG in AD differential diagnosis with other forms of dementia: Lewy body dementia (LBD), Parkinson’s disease dementia (PDD), frontotemporal dementia (FTD), and vascular dementia (VaD). Methods We searched PubMed, Embase, and PsycNET, for articles in English published in peer-reviewed journals from January 1, 1980 to April 23, 2019 using adapted search strategies containing keywords quantitative EEG and Alzheimer. The risk of bias was assessed by applying the QUADAS tool. The systematic review was conducted in line with the PRISMA methodology. Results We identified 10 articles showcasing QEEG features used in diagnosing dementia, EEG slowing phenomena in AD and PDD, coherence changes in AD and VaD, the role of LORETA in dementia, and the controversial QEEG pattern in FTD. Results vary significantly in terms of sociodemographic features of the studied population, neuropsychological assessment, signal acquisition and processing, and methods of analysis. Discussion This article provides a comparative synthesis of existing evidence on the role of QEEG in diagnosing dementia, highlighting some specific features for different types of dementia (eg, the slow-wave activity has been remarked in both AD and PDD, but more pronounced in PDD patients, a diminution in anterior and posterior alpha coherence was noticed in AD, and a lower alpha coherence in the left temporal-parietal-occipital regions was observed in VaD). Conclusion QEEG may be a useful investigation for settling the diagnosis of common forms of dementia. Further research of quantitative analyses is warranted, particularly on the association between QEEG, neuropsychological, and imaging features. In conjunction, these methods may provide superior diagnostic accuracy in the diagnosis of dementia.

Role of Inflammation in Lewy Body Dementia

2021 ◽  
pp. 190-212
Author(s):  
Ajenthan Surendranathan ◽  
John T. O’Brien
Keyword(s):  
Lewy Body ◽  
Lewy Body Dementia
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