Young-Onset Dementia with Lewy Bodies

Young-onset (< 65 years) dementia is a challenging clinical problem. A 61-year-old man visited our clinic because of a 2-year history of mild cognitive impairment of the executive disorder type. He was initially suspected of having young-onset Alzheimer’s disease due to the lack of motor signs or hippocampal atrophy by conventional brain MRI. However, he proved to have anosmia, erectile dysfunction, hypersexuality, constipation, REM sleep behavior disorder, and emotional lability; imaging findings included positive brain perfusion SPECT, nigrosome MRI, DAT scan, and MIBG myocardial scintigraphy. All these clinical imaging features led to the correct diagnosis of young-onset dementia with Lewy bodies (YOD-DLB). It is hoped that this case report will help facilitate a future prospective study to diagnose and follow YOD-DLB patients with the aim of determining appropriate management and care.

Download Full-text

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

Download Full-text

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

Download Full-text

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.

Download Full-text

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.

Download Full-text

β-Amyloid and tau biomarkers and clinical phenotype in dementia with Lewy bodies

Neurology ◽

10.1212/wnl.0000000000010943 ◽

2020 ◽

Vol 95 (24) ◽

pp. e3257-e3268 ◽

Cited By ~ 1

Author(s):

Daniel Ferreira ◽

Scott A. Przybelski ◽

Timothy G. Lesnick ◽

Afina W. Lemstra ◽

Elisabet Londos ◽

...

Keyword(s):

Cognitive Performance ◽

Clinical Features ◽

Dementia With Lewy Bodies ◽

Clinical Phenotype ◽

Lewy Bodies ◽

Rem Sleep Behavior Disorder ◽

Tau Pathology ◽

Sleep Behavior ◽

Amyloid A ◽

Β Amyloid

ObjectiveIn a multicenter cohort of probable dementia with Lewy bodies (DLB), we tested the hypothesis that β-amyloid and tau biomarker positivity increases with age, which is modified by APOE genotype and sex, and that there are isolated and synergistic associations with the clinical phenotype.MethodsWe included 417 patients with DLB (age 45–93 years, 31% women). Positivity on β-amyloid (A+) and tau (T+) biomarkers was determined by CSF β-amyloid1-42 and phosphorylated tau in the European cohort and by Pittsburgh compound B and AV-1451 PET in the Mayo Clinic cohort. Patients were stratified into 4 groups: A−T−, A+T−, A−T+, and A+T+.ResultsA−T− was the largest group (39%), followed by A+T− (32%), A+T+ (15%), and A−T+ (13%). The percentage of A−T− decreased with age, and A+ and T+ increased with age in both women and men. A+ increased more in APOE ε4 carriers with age than in noncarriers. A+ was the main predictor of lower cognitive performance when considered together with T+. T+ was associated with a lower frequency of parkinsonism and probable REM sleep behavior disorder. There were no significant interactions between A+ and T+ in relation to the clinical phenotype.ConclusionsAlzheimer disease pathologic changes are common in DLB and are associated with the clinical phenotype. β-Amyloid is associated with cognitive impairment, and tau pathology is associated with lower frequency of clinical features of DLB. These findings have important implications for diagnosis, prognosis, and disease monitoring, as well as for clinical trials targeting disease-specific proteins in DLB.Classification of evidenceThis study provides Class II evidence that in patients with probable DLB, β-amyloid is associated with lower cognitive performance and tau pathology is associated with lower frequency of clinical features of DLB.

Download Full-text