scholarly journals Evaluation of Incidence and Clinical Features of Antibody-Associated Autoimmune Encephalitis Mimicking Dementia

2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
Arzu Çoban ◽  
Cem İsmail Küçükali ◽  
Başar Bilgiç ◽  
Nazlı Yalçınkaya ◽  
Hazal Haytural ◽  
...  

Background. Anti-neuronal autoimmunity may cause cognitive impairment that meets the criteria for dementia.Objective. Our aim was to detect the incidence and clinical features of autoimmune encephalitis imitating clinical findings of primary dementia disorders and to delineate the validity of anti-neuronal antibody screening in dementia patients.Methods. Fifty consecutive patients fulfilling the clinical criteria for primary dementia, 130 control patients, and 50 healthy controls were included. Their sera were investigated for several ion channel and glutamic acid decarboxylase (GAD) antibodies by a cell-based assay, radioimmunoassay, and ELISA, as required.Results. Sixteen patients satisfying dementia criteria had atypical findings or findings suggestive of autoimmune encephalitis. N-methyl-D-aspartate receptor (NMDAR) antibody was detected in a patient with dementia, Parkinsonism, and REM sleep behavior disorder (RBD) fulfilling the criteria for dementia with Lewy bodies (DLB). One control patient with bipolar disease displayed low anti-GAD antibody levels.Conclusions. Our study showed for the first time the presence of parkinsonism and RBD in an anti-NMDAR encephalitis patient mimicking DLB. Although autoimmune encephalitis patients may occasionally present with cognitive decline, most dementia patients do not exhibit anti-neuronal antibodies, suggesting that routine analysis of these antibodies in dementia is not mandatory, even though they display atypical features.

2016 ◽  
Vol 52 (3) ◽  
pp. 989-997 ◽  
Author(s):  
Leonardo Iaccarino ◽  
Sara Marelli ◽  
Sandro Iannaccone ◽  
Giuseppe Magnani ◽  
Luigi Ferini-Strambi ◽  
...  

2021 ◽  
pp. 1-13
Author(s):  
David Rémillard-Pelchat ◽  
Shady Rahayel ◽  
Malo Gaubert ◽  
Ronald B. Postuma ◽  
Jacques Montplaisir ◽  
...  

Background: Rapid-eye-movement sleep behavior disorder (RBD) is a major risk factor for Parkinson’s disease and dementia with Lewy bodies. More than a third of RBD patients have mild cognitive impairment (MCI), but their specific structural brain alterations remain poorly understood. Objective: This study aimed to investigate the local deformation and volume of gray and white matter tissue underlying MCI in RBD. Methods: Fifty-two idiopathic RBD patients, including 17 with MCI (33%), underwent polysomnography, neuropsychological, neurological, and magnetic resonance imaging assessments. MCI diagnosis was based on a subjective complaint, cognitive impairment on the neuropsychological battery, and preserved daily functioning. Forty-one controls were also included. Deformation-based morphometry (DBM), voxel-based morphometry (VBM), and regional volume analyses of the corpus callosum and basal forebrain cholinergic were performed. Multiple regressions models were also computed using anatomical, cognitive (composite z score), and motor parameters. Results: Globally, patients with MCI displayed a widespread pattern of local deformation and volume atrophy in the cortical (bilateral insula, cingulate cortex, precuneus, frontal and temporal regions, right angular gyrus, and mid-posterior segment of the corpus callosum) and subcortical (brainstem, corona radiata, basal ganglia, thalamus, amygdala, and right hippocampus) regions compared to patients without MCI (DBM) or controls (DBM and VBM). Moreover, brain deformation (DBM) in patients were associated with lower performance in attention and executive functions, visuospatial abilities, and higher motor symptoms severity. Conclusion: The present study identified novel brain structural alterations in RBD patients with MCI which correlated with poorer cognitive performance. These results are consistent with those reported in patients with synucleinopathies-related cognitive impairment.


2018 ◽  
Vol 2018 ◽  
pp. 1-10 ◽  
Author(s):  
Po-Chi Chan ◽  
Hsun-Hua Lee ◽  
Chien-Tai Hong ◽  
Chaur-Jong Hu ◽  
Dean Wu

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.


2020 ◽  
Author(s):  
Jeongbin Park ◽  
Seung Wan Suh ◽  
Grace Eun Kim ◽  
Subin Lee ◽  
Jun Sung Kim ◽  
...  

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.


Neurology ◽  
2020 ◽  
Vol 95 (24) ◽  
pp. e3257-e3268 ◽  
Author(s):  
Daniel Ferreira ◽  
Scott A. Przybelski ◽  
Timothy G. Lesnick ◽  
Afina W. Lemstra ◽  
Elisabet Londos ◽  
...  

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.


Author(s):  
Ronald B. Postuma

A diagnosis of REM sleep behavior disorder (RBD), a disorder characterized by “acting out” of dreams during REM sleep, has critical implications for a patient’s future. Aside from being a treatable parasomnia, usually managed with melatonin or clonazepam, RBD is the most powerful risk factor for Parkinson disease and dementia with Lewy bodies yet discovered. Over 70% of patients with idiopathic RBD will develop a neurodegenerative synucleinopathy. Moreover, the disease course is more severe in patients with RBD than those without. Numerous screens have been developed to aid detection, and clinical history can help distinguish RBD from NREM parasomnia. However, final diagnosis relies on polysomnographic documentation of REM atonia loss. Given the profound implications of idiopathic RBD, patients need careful counseling and the offer of neurological follow-up to detect and treat prodromal disease symptoms. Recognition of RBD is also a means to discover and test protective therapies against neurodegenerative disease.


Neurology ◽  
1998 ◽  
Vol 51 (2) ◽  
pp. 363-370 ◽  
Author(s):  
B. F. Boeve ◽  
M. H. Silber ◽  
T. J. Ferman ◽  
E. Kokmen ◽  
G. E. Smith ◽  
...  

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.


2021 ◽  
Vol 15 ◽  
Author(s):  
Anjiao Peng ◽  
Ruien Wang ◽  
Jiamin Huang ◽  
Haiyan Wu ◽  
Lei Chen

Objective: Rapid eye movement (REM) sleep behavior disorder (RBD) is a disease characterized by dream enacting behavior and is now commonly believed to be a harbinger to alpha-synucleinopathy diseases such as dementia with Lewy bodies, Parkinson's disease, and multiple system atrophy. The aim of this study was to explore the quasi-stable topological structure of the brain in RBD by analyzing resting-state electroencephalography (EEG) microstates.Methods: We enrolled 22 participants with RBD and 46 healthy controls (HCs) with age and gender-matched. After the resting-state EEG recordings were acquired, EEG microstate features were analyzed to assess the functional networks of all participants.Results: Significant differences in the brain topological structure and temporal characteristics of sub-second brain activity were identified between the RBD and HCs. The RBD group had a shorter average duration of microstate A and microstate D when compared with HCs, and microstate B contributed more, while microstate D contributed significantly less to the RBD group. Furthermore, the average duration and proportion of microstate D were negatively correlated with the RBD questionnaire Hong Kong (RBDQ-HK) score.Conclusion: The result of this study indicates that the microstate dynamics is disturbed in RBD, which might jeopardize the flexibility and adaptability of the brain. Microstates are potential biomarkers to explore the early electrophysiological abnormality of alpha-synucleinopathy diseases.


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