Diagnosis of Dementia with Lewy Bodies: Fluctuations, Biomarkers, and beyond

Dementia with Lewy bodies (DLB), the second most common cause of dementia, remains a difficult condition to accurately diagnose and manage. Variable involvement of motor and cognitive functions, plus psychiatric and behavioral symptoms, contributes to the difficulty in managing DLB. Additionally, DLB can cause severe sleep disruption through REM sleep behavior disorder, autonomic symptoms, disruptions of olfaction/taste and mood, hallucinations, and more. In this chapter, advances and remaining challenges in the diagnosis of DLB are discussed, including a review of the current consensus criteria for DLB. The spectrum of disorders with Lewy bodies (LBs) are described including their wide-range of clinical presentations and overlap with Alzheimer’s disease (AD) and Parkinson’s disease with and without dementia. Particular consideration is given to advancements in quantification of cognitive fluctuations through improved clinical instruments, EEG, and other advanced biomarkers. Detection of DLB has improved, but establishing the “primary” pathology in cases with concomitant LB andd AD remains difficult. Likelihood of a clinical DLB syndrome is thought to be a function of distribution of LBs and severity of AD-type pathology. Further work is needed to better understand LB disease subtypes and the underlying pathophysiological mechanisms to allow for more targeted and comprehensive therapies.

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Diagnostic Value of 123-I-MIBG Cardiac Scintigraphy for the Prediction of Conversion from Idiopathic REM Sleep Behavior Disorder to Dementia with Lewy Bodies, and the Differential Diagnosis of Neurodegenerative Diseases

Alzheimers Disease & Dementia ◽

10.36959/734/373 ◽

2017 ◽

Vol 1 (2) ◽

Author(s):

Seto Makiko ◽

Nakata Ruka ◽

Yuasa Takayuki ◽

Nakao Yoko ◽

Ichinose Katsuhiro ◽

...

Keyword(s):

Differential Diagnosis ◽

Neurodegenerative Diseases ◽

Rem Sleep ◽

Dementia With Lewy Bodies ◽

Lewy Bodies ◽

Rem Sleep Behavior Disorder ◽

Behavior Disorder ◽

Diagnostic Value ◽

Sleep Behavior

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Severe Brain Metabolic Decreases Associated with REM Sleep Behavior Disorder in Dementia with Lewy Bodies

Journal of Alzheimer s Disease ◽

10.3233/jad-151000 ◽

2016 ◽

Vol 52 (3) ◽

pp. 989-997 ◽

Cited By ~ 9

Author(s):

Leonardo Iaccarino ◽

Sara Marelli ◽

Sandro Iannaccone ◽

Giuseppe Magnani ◽

Luigi Ferini-Strambi ◽

...

Keyword(s):

Rem Sleep ◽

Dementia With Lewy Bodies ◽

Lewy Bodies ◽

Rem Sleep Behavior Disorder ◽

Behavior Disorder ◽

Sleep Behavior

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REM Sleep Behavior Disorder (RBD) in Dementia with Lewy Bodies (DLB)

Behavioural Neurology ◽

10.1155/2018/9421098 ◽

2018 ◽

Vol 2018 ◽

pp. 1-10 ◽

Cited By ~ 5

Author(s):

Po-Chi Chan ◽

Hsun-Hua Lee ◽

Chien-Tai Hong ◽

Chaur-Jong Hu ◽

Dean Wu

Keyword(s):

Eye Movement ◽

Rem Sleep ◽

Dementia With Lewy Bodies ◽

Clinical Manifestations ◽

Lewy Bodies ◽

Rem Sleep Behavior Disorder ◽

Behavior Disorder ◽

Rapid Eye Movement ◽

Sleep Behavior ◽

Neurologic Disorders

Rapid eye movement sleep behavior disorder (RBD) is a parasomnia, with abnormal dream-enacting behavior during the rapid eye movement (REM) sleep. RBD is either idiopathic or secondary to other neurologic disorders and medications. Dementia with Lewy bodies (DLB) is the third most common cause of dementia, and the typical clinical presentation is rapidly progressive cognitive impairment. RBD is one of the core features of DLB and may occur either in advance or simultaneously with the onset of DLB. The association between RBD with DLB is widely studied. Evidences suggest that both DLB and RBD are possibly caused by the shared underlying synucleinopathy. This review article discusses history, clinical manifestations, possible pathophysiologies, and treatment of DLB and RBD and provides the latest updates.

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Smaller pineal gland is associated with rapid eye movement sleep behavior disorder in Alzheimer’s disease

10.21203/rs.3.rs-56077/v2 ◽

2020 ◽

Author(s):

Jeongbin Park ◽

Seung Wan Suh ◽

Grace Eun Kim ◽

Subin Lee ◽

Jun Sung Kim ◽

...

Keyword(s):

Pineal Gland ◽

Eye Movement ◽

Rem Sleep ◽

Dementia With Lewy Bodies ◽

Lewy Bodies ◽

Rem Sleep Behavior Disorder ◽

Behavior Disorder ◽

Rapid Eye Movement ◽

Sleep Behavior ◽

Gland Volume

Abstract Background: To investigate the association between pineal gland volume and symptoms of rapid eye movement (REM) sleep behavior disorder (RBD) in Alzheimer’s disease (AD) patients without any feature of dementia with Lewy bodies.Methods: We enrolled 296 community-dwelling probable AD patients who did not meet the diagnostic criteria for possible or probable dementia with Lewy bodies. Among them, 93 were amyloid beta (Aβ) positive on 18F-florbetaben amyloid brain positron emission tomography. We measured RBD symptoms using the REM Sleep Behavior Disorder Screening Questionnaire (RBDSQ) and defined probable RBD (pRBD) as the RBDSQ of 5 or higher. We manually segmented pineal gland on 3T structural T1-weighted brain magnetic resonance imaging.Results: The participants with pRBD had smaller pineal parenchyma volume (VPP) than those without pRBD (p < 0.001). The smaller the VPP, the more severe the RBD symptoms (p < 0.001). VPP was inversely associated with risk of prevalent pRBD (odds ratio = 0.909, 95% confidence interval [CI] = 0.878–0.942, p < 0.001). Area under the receiver operator characteristic curve for pRBD of VPP was 0.80 (95% CI = 0.750–0.844, p < 0.0001). These results were not changed when we analyzed the 93 participants with Aβ-positive AD separately.Conclusions: In AD patients, reduced pineal gland volume may be associated with RBD.

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Assessment of Amyloid Deposition in Patients With Probable REM Sleep Behavior Disorder as a Prodromal Symptom of Dementia With Lewy Bodies Using PiB-PET

Frontiers in Neurology ◽

10.3389/fneur.2019.00671 ◽

2019 ◽

Vol 10 ◽

Cited By ~ 1

Author(s):

Ryota Kobayashi ◽

Hiroshi Hayashi ◽

Shinobu Kawakatsu ◽

Nobuyuki Okamura ◽

Masanori Yoshioka ◽

...

Keyword(s):

Rem Sleep ◽

Dementia With Lewy Bodies ◽

Lewy Bodies ◽

Rem Sleep Behavior Disorder ◽

Behavior Disorder ◽

Amyloid Deposition ◽

Prodromal Symptom ◽

Sleep Behavior

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REM sleep behavior disorder and degenerative dementia

Neurology ◽

10.1212/wnl.51.2.363 ◽

1998 ◽

Vol 51 (2) ◽

pp. 363-370 ◽

Cited By ~ 256

Author(s):

B. F. Boeve ◽

M. H. Silber ◽

T. J. Ferman ◽

E. Kokmen ◽

G. E. Smith ◽

...

Keyword(s):

Rem Sleep ◽

Clinical Laboratory ◽

Neuropsychological Testing ◽

Age At Onset ◽

Lewy Bodies ◽

Rem Sleep Behavior Disorder ◽

Behavior Disorder ◽

Sleep Behavior ◽

Diagnosis Of Dementia ◽

Degenerative Dementia

Background:REM sleep behavior disorder (RBD) has been reported with various neurodegenerative disorders, most frequently in disorders with Lewy body pathology. RBD often precedes the onset of PD, and a recent prospective study showed that 38% of patients with RBD eventually developed PD.Methods:We identified 37 patients with degenerative dementia and a history of bursts of vigorous movement of the arms and legs with vocalization during sleep and associated with dream recall. Patients with and without two or more signs of parkinsonism were compared. Clinical, laboratory, and neuropsychometric features were analyzed, and criteria for the clinical diagnosis of dementia with Lewy bodies (DLB) were applied to all patients.Results:Thirty-four of the 37 patients were male with mean age at onset of 61.5 years for RBD and 68.1 years for cognitive decline. RBD commenced before or concurrently with dementia in all patients but two. Parkinsonism (two or more signs) occurred in 54% of the sample (20/37), with a mean age at onset of 69.1 years. Polysomnography (PSG) confirmed RBD in all patients studied. Neuropsychological testing demonstrated impaired perceptual-organizational skills, verbal fluency, and marked constructional dyspraxia in more than one-half the patients. There were no statistically significant differences in the frequency of clinical features or in neuropsychological performance between patients with and without parkinsonism. Thirty-four patients (92%) met criteria for clinically possible or probably DLB. Three patients were autopsied; all had limbic with or without neocortical Lewy bodies.Conclusions:We report a group of predominantly male patients with a characteristic association of RBD and degenerative dementia. The clinical and neuropsychometric features of the groups of patients with and without parkinsonism are similar. We hypothesize that the underlying pathology in these patients is DLB.

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