scholarly journals Impaired Color Discrimination—A Specific Marker of Hallucinations in Lewy Body Disorders

2019 ◽  
Vol 32 (5) ◽  
pp. 257-264 ◽  
Author(s):  
Elie Matar ◽  
Joseph R. Phillips ◽  
Kaylena A. Ehgoetz Martens ◽  
Glenda M. Halliday ◽  
Simon J. G. Lewis
Keyword(s):  
Color Vision ◽  
Lewy Body ◽  
Strong Association ◽  
Lewy Bodies ◽  
Behavior Disorder ◽  
Lewy Body Dementia ◽  
Color Discrimination ◽  
Specific Marker ◽  
Visual Hallucinations ◽  
Sleep Behavior

There is emerging evidence indicating that color discrimination impairments can predict the development of Lewy body dementia in patients with rapid eye movement sleep behavior disorder, Parkinson disease, and in patients with mild cognitive impairment. Despite this clear relationship, color vision deficits are not seen uniformly in patients with dementia with Lewy bodies (DLB), suggesting a more nuanced association with the underlying neuropathology. Visual hallucinations represent a discriminating feature of DLB, and recent evidence implicates visual pathway dysfunction as a significant contributor to this phenomenon. In this study, we examined the relationship between color vision impairment and visual hallucinations, along with other clinical and neuropsychological features in 24 well-characterized patients with DLB alongside 25 healthy controls. Color discrimination impairment was seen in 16 (67%) of 24 DLB participants with a higher error score relative to controls ( P = .001). We demonstrate for the first time a strong association between color discrimination errors on the Farnsworth-Munsell 100 hue test and both the presence and severity of hallucinatory symptoms in DLB based on clinician-derived ( P = .008) and questionnaire-derived ( P = .03) measures. Correlation with clinical and neuropsychological variables revealed that color discrimination is significantly related to visuospatial difficulties measured by the clock-drawing task ( P = .02) but not to global measures of cognition, motor severity, age, or disease duration in our cohort. Factor analysis confirmed a unique relationship between color discrimination, visual hallucinations, and visuospatial function. Our results suggest that color discrimination does not simply relate to dementia but rather indexes higher order perceptual deficits that may predict visual hallucinations in Lewy body disorders and share a common pathophysiological substrate.

scholarly journals 060 Impaired color discrimination is associated with hallucinations in dementia with lewy bodies

2019 ◽  
Vol 90 (e7) ◽  
pp. A19.3-A20
Author(s):  
Elie Matar ◽  
Joseph R Phillips ◽  
Kaylena A Ehgoetz Martens ◽  
Glenda M Halliday ◽  
Simon JG Lewis
Keyword(s):  
Color Vision ◽  
Lewy Body ◽  
Dementia With Lewy Bodies ◽  
Strong Association ◽  
Lewy Bodies ◽  
Color Discrimination ◽  
Visual Hallucinations ◽  
Vision Deficits ◽  
First Time ◽  
The Relationship

IntroductionEmerging evidence indicates that color discrimination impairments can predict the development of dementia across a range of prodromal Lewy body conditions. However, color vision deficits are not seen uniformly in patients with Dementia with Lewy Bodies (DLB), suggesting a more nuanced association. Visual hallucinations(VH) represent a discriminating feature of DLB, and recent evidence implicates visual pathway dysfunction as a significant contributor to this phenomenon. We therefore hypothesized that color impairment will more closely associate with VH in DLB rather than general cognition.MethodsIn this study, we examined the relationship between color vision impairment and VH, along with other clinical and neuropsychological features in 24 patients with DLB alongside 25 age-matched controls. Color discrimination was assessed using the Farnsworth-Munsell-100 Hue (FM-100) test.ResultsColor discrimination impairment was seen in 16/24 DLB participants (67%) with a higher error score relative to controls(p=0.001). We demonstrate for the first time a strong association between color discrimination errors and both the presence and severity of VH in DLB based on clinician-derived(p=0.008) and questionnaire-derived(p=0.03) measures. Correlation with clinical and neuropsychological variables revealed that color discrimination is significantly related to visuospatial impairment(p=0.02) but not to global measures of cognition, motor severity, age or disease duration. Factor analysis confirmed a unique relationship between color discrimination, visual hallucinations and visuospatial function.ConclusionOur results suggest that color impairments may be a specific biomarker of visual hallucinations and associated visuoperceptual deficits in evolving Lewy body disorders rather than dementia per se and thus providing insight into a shared pathophysiological substrate.

scholarly journals Cortical Lewy Body Dementia

1990 ◽  
Vol 3 (3) ◽  
pp. 189-196 ◽  
Author(s):  
W. R. G. Gibb
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Nervous System ◽  
Lewy Body ◽  
Age Of Onset ◽  
Lewy Bodies ◽  
Lewy Body Dementia ◽  
Survival Duration ◽  
Visual Hallucinations ◽  
Alzheimer Pathology

In cortical Lewy body dementia the distribution of Lewy bodies in the nervous system follows that of Parkinson's disease, except for their greater profusion in the cerebral cortex. The cortical tangles and plaques of Alzheimer pathology are often present, the likely explanation being that Alzheimer pathology provokes dementia in many patients. Pure cortical Lewy body dementia without Alzheimer pathology is uncommon. The age of onset reflects that of Parkinson's disease, and clinical features, though not diagnostic, include aphasias, apraxias, agnosias, paranoid delusions and visual hallucinations. Parkinsonism may present before or after the dementia, and survival duration is approximately half that seen in Parkinson's disease without dementia.

Dementia with Lewy bodies may present as dementia and REM sleep behavior disorder without parkinsonism or hallucinations

2002 ◽  
Vol 8 (7) ◽  
pp. 907-914 ◽  
Author(s):  
TANIS J. FERMAN ◽  
BRADLEY F. BOEVE ◽  
GLENN E. SMITH ◽  
MICHAEL H. SILBER ◽  
JOHN A. LUCAS ◽  
...  
Keyword(s):  
Rem Sleep ◽  
Verbal Memory ◽  
Dementia With Lewy Bodies ◽  
Lewy Bodies ◽  
Rem Sleep Behavior Disorder ◽  
Behavior Disorder ◽  
Subsequent Development ◽  
Visual Hallucinations ◽  
Sleep Behavior ◽  
Presenting Symptoms

Rapid eye movement (REM) sleep behavior disorder (RBD) is a sleep disturbance that commonly occurs in Dementia with Lewy bodies (DLB). Retrospective examination of DLB course has shown that RBD and cognitive decline may precede the onset of parkinsonism and visual hallucinations. Therefore, some patients with DLB may initially present with dementia and RBD, but would not meet current formal criteria for probable DLB at that time. The purpose of this study is to determine whether patients with dementia and RBD, who do not have parkinsonism or visual hallucinations, have cognitive profiles that can be distinguished from autopsy-confirmed definite AD, but not from clinically probable DLB. If so, this would support the hypothesis that the presence of RBD and dementia, as the only presenting symptoms, reflects the early manifestation of DLB. Results show that early dementia in probable DLB and dementia with RBD are neuropsychologically indistinguishable. Both groups differ from definite AD of a similar early stage with significantly worse visual perceptual organization, sequencing and letter fluency but significantly better confrontation naming and verbal memory. In addition, follow-up data from a subset of patients with dementia and RBD reveal the subsequent development of parkinsonism or hallucinations 1 to 6 years later. Results indicate that the presentation of dementia and RBD is suggestive of underlying Lewy body disease and not Alzheimer's disease. This provides further evidence in support of including RBD as one of the core diagnostic features of DLB. (JINS, 2002, 8, 907–914.)

The pathology of REM sleep behavior disorder with comorbid Lewy body dementia

Neurology ◽  
2000 ◽  
Vol 55 (11) ◽  
pp. 1730-1732 ◽  
Author(s):  
R. S. Turner ◽  
C. J. D'Amato ◽  
R. D. Chervin ◽  
M. Blaivas
Keyword(s):  
Rem Sleep ◽  
Lewy Body ◽  
Rem Sleep Behavior Disorder ◽  
Behavior Disorder ◽  
Lewy Body Dementia ◽  
Sleep Behavior

849 REM Behavioral Disorder as a Predictor of Lewy body Dementia- A Case Report

SLEEP ◽  
2021 ◽  
Vol 44 (Supplement_2) ◽  
pp. A330-A330
Author(s):  
Rupa Koothirezhi ◽  
Pratibha Anne ◽  
Minh Tam Ho ◽  
Brittany Monceaux ◽  
Cesar Liendo ◽  
...  
Keyword(s):  
Clinical Features ◽  
Lewy Body ◽  
Neurodegenerative Disorder ◽  
Lewy Bodies ◽  
Rem Sleep Behavior Disorder ◽  
Lewy Body Dementia ◽  
Alpha Synuclein ◽  
Behavioral Disorder ◽  
Sleep Behavior ◽  
Obstructive Sleep

Abstract Introduction Dementia with Lewy bodies (DLB) is one of the most common types of degenerative dementia after Alzheimer’s dementia. The core clinical features for diagnosis includes cognitive fluctuations, visual hallucinations, rapid eye movement (REM) sleep behavior disorder (RBD), and parkinsonism. Other symptoms include daytime drowsiness, longer daytime naps, prolonged staring spells, and episodes of disorganized speech. REM behavioral disorder (RBD) is commonly associated with DLB, occurring in 85 percent of individuals, often early in the course of the disease. It can precede the clinical diagnosis of DLB by up to 20 years. Report of case(s) A seventy-six-year-old female with a history of well controlled obstructive sleep apnea was diagnosed with REM behavioral disorder in 2012. She had presented with episodes of screaming, attempt to ambulate during sleep, resulting in injury. Her polysomnogram revealed evidence of REM without atonia and a screaming episode during REM. Her RBD symptoms were controlled with clonazepam and melatonin with less frequency of the RBD episodes. The patient gradually started noticing memory issues and by January 2020 she was diagnosed with dementia and was initiated on Aricept. Within 7 months of diagnosis of dementia, she started reporting vivid hallucinations that were not threatening or violent compared to her violent content of RBD. Physical exam revealed impaired cognitive function and mild intermittent resting tremor of the right hand. The neurological exam was normal including normal tone, strength, and gait. She also reported repeated falls and fractures. The diagnosis of Lewy body dementia was made based on the presence of 2 core clinical features. Conclusion The current management of these conditions is mainly symptomatic. In the evolution of neurodegenerative disorder, RBD precedes other conditions like LBD, parkinsonism, etc. Research suggests that alpha-synuclein neurodegeneration is the common pathology behind these conditions. The understanding that RBD presents at the beginning of the evolution, provides us with a unique opportunity for preemptive treatment to prevent further degeneration in turn preventing the debilitation consequence like dementia, parkinsonism, neuroleptic sensitivity, and dysautonomia. Further research is needed for developing these early interventional strategies. Support (if any) NOne

scholarly journals Cross-platform transcriptional profiling identifies common and distinct molecular pathologies in Lewy body diseases

2021 ◽  
Author(s):  
Rahel Feleke ◽  
Regina H. Reynolds ◽  
Amy M. Smith ◽  
Bension Tilley ◽  
Sarah A. Gagliano Taliun ◽  
...  
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Neurodegenerative Diseases ◽  
Lewy Body ◽  
Molecular Mechanisms ◽  
Lewy Bodies ◽  
Subsequent Development ◽  
Lewy Body Dementia ◽  
Anterior Cingulate ◽  
Lewy Body Diseases

AbstractParkinson’s disease (PD), Parkinson’s disease with dementia (PDD) and dementia with Lewy bodies (DLB) are three clinically, genetically and neuropathologically overlapping neurodegenerative diseases collectively known as the Lewy body diseases (LBDs). A variety of molecular mechanisms have been implicated in PD pathogenesis, but the mechanisms underlying PDD and DLB remain largely unknown, a knowledge gap that presents an impediment to the discovery of disease-modifying therapies. Transcriptomic profiling can contribute to addressing this gap, but remains limited in the LBDs. Here, we applied paired bulk-tissue and single-nucleus RNA-sequencing to anterior cingulate cortex samples derived from 28 individuals, including healthy controls, PD, PDD and DLB cases (n = 7 per group), to transcriptomically profile the LBDs. Using this approach, we (i) found transcriptional alterations in multiple cell types across the LBDs; (ii) discovered evidence for widespread dysregulation of RNA splicing, particularly in PDD and DLB; (iii) identified potential splicing factors, with links to other dementia-related neurodegenerative diseases, coordinating this dysregulation; and (iv) identified transcriptomic commonalities and distinctions between the LBDs that inform understanding of the relationships between these three clinical disorders. Together, these findings have important implications for the design of RNA-targeted therapies for these diseases and highlight a potential molecular “window” of therapeutic opportunity between the initial onset of PD and subsequent development of Lewy body dementia.

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
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