Stochastic Optical Reconstruction Microscopy Imaging of Multiple System Atrophy Inclusions Suggests Stepwise α-Synuclein Aggregation.

Background: The architecture and composition of glial (GCI) and neuronal (NCI) α-synuclein inclusions observed in multiple system atrophy (MSA) remain to be precisely defined to better understand the disease.

Methods: Here, we used stochastic optical reconstruction microscopy (STORM) to characterize the nanoscale organization of glial (GCI) and neuronal (NCI) α-synuclein inclusions in cryopreserved brain sections from MSA patients.

Results: STORM revealed a dense cross-linked internal structure of α-synuclein in all GCI and NCI.

[The use of melatonin in older patients hospitalized in geriatric units in France: survey of hospital pharmacists].

In recent years, melatonin has been increasingly used in hospital settings for the treatment of sleep disorders in older patients, despite many barriers: restriction by ANSM, non-approval to healthcare facility use, and non-reimbursement. In order to describe the use of melatonin in older hospitalized patients, a survey was conducted between February and May 2022, with hospital pharmacists working in geriatric care units in France. Overall, 35 interviews were conducted with hospital pharmacists: 30 dispensed melatonin, with marketed prolonged-release melatonin medications (n = 30), and/or with immediate-release magistral or hospital preparations (n = 11).

Design and application of a customizable relational DataBase to assess clinicopathological correlations and concomitant pathology in neurodegenerative diseases.

The diagnosis of neurodegenerative diseases is made complex by the heterogenous phenotype of the patients and the regular occurrence of concomitant pathology. Studying clinicopathological correlations in autopsy series is a central approach to improve pathological prediction in clinical practice. However, such method requires a wealth of information, and the use of standard spreadsheet software is hardly suitable.

Biomarkers and diagnosis of dementia with Lewy bodies including prodromal: Practical aspects.

Dementia with Lewy Bodies (DLB) is a common form of cognitive neurodegenerative disease. More than half of the patients affected are not or misdiagnosed because of the clinical similarity with Alzheimer's disease (AD), Parkinson's disease but also psychiatric diseases such as depression or psychosis. In this review, we evaluate the interest of different biomarkers in the diagnostic process: cerebrospinal fluid (CSF), brain MRI, FP-CIT SPECT, MIBG SPECT, perfusion SPECT, FDG-PET by focusing more specifically on differential diagnosis between DLB and AD.

Switching to a Dual-Drug Regimen in HIV-Infected Patients Could Be Associated With Macrophage Activation?

The aim of this study was to evaluate the effect on immune activation of switching from a triple-drug to a dual-drug regimen in HIV-1 infected patients on successful combination antiretroviral treatment (cART). Immunadapt is a prospective study evaluating the impact of cART simplification on immune activation. We prospectively collected blood samples in HIV-1 infected patients on stable and successful cART switching from triple to dual regimens as a simplifying strategy.

Late-onset and nonlate-onset schizophrenia: A comparison of clinical characteristics in a multicenter study.

Objectives: Data are scarce regarding the potential clinical differences between non-late onset schizophrenia (NLOS, i.e., disorder occurring before 40 years of age), late-onset schizophrenia (LOS, occurring between ages 40 and 60 years) and very-late-onset schizophrenia-like psychosis (VLOSLP, occurring after 60 years of age).

Semantic loss marks early Alzheimer's disease-related neurodegeneration in older adults without dementia.

Objective: To assess progression of semantic loss in early stages of cognitive decline using semantic and letter fluency performance, and its relation with Alzheimer's disease (AD)-specific neurodegeneration using longitudinal multimodal neuroimaging measures.

Methods: Change in verbal fluency was analyzed among 2261 non-demented individuals with a follow-up diagnosis of no mild cognitive impairment (MCI), amnestic MCI (aMCI), non-amnestic MCI (naMCI), or incident dementia, using linear mixed models across 4 years of follow-up, and relations with magnetic resonance imaging (MRI; n = 1536) and F-fluorodeoxyglucose brain positron emission tomography (F-FDG-PET) imaging (n = 756) using linear regression models across 2 years of follow-up.

Results: Semantic fluency declined-fastest in those at higher risk for AD (apolipoprotein E [APOE] e4 carriers, Clinical Dementia Rating score of .

The combined effect of amyloid-β and tau biomarkers on brain atrophy in dementia with Lewy bodies.

Background: Alzheimer's disease (AD)-related pathology is frequently found in patients with dementia with Lewy bodies (DLB). However, it is unknown how amyloid-β and tau-related pathologies influence neurodegeneration in DLB. Understanding the mechanisms underlying brain atrophy in DLB can improve our knowledge about disease progression, differential diagnosis, drug development and testing of anti-amyloid and anti-tau therapies in DLB.

A comprehensive model of predictors of quality of life in older adults with schizophrenia: results from the CSA study.

Background: Numerous factors are known to influence quality of life of adults with schizophrenia. However, little is known regarding the potential predictors of quality of life in the increasing population of older adults with schizophrenia. The main objective of the present study was to propose a comprehensive model of quality of life in this specific population.

[Lewy body dementia: therapeutic propositions according to evidence based medicine and practice].

Lewy body dementia (LBD) may present with two clinical forms: dementia with Lewy bodies (DLB) and Parkinson's disease with dementia. LBD is characterized by a profound deficiency of acetylcholine and a deficiency of dopamine. The cholinergic deficit is implicated in major attention disorders and fluctuations.

Association of Retinal Nerve Fiber Layer Thickness With Brain Alterations in the Visual and Limbic Networks in Elderly Adults Without Dementia.

Importance: The eye is a sensory organ that is easily accessible for imaging techniques, allowing the measurement of the retinal nerve fiber layer (RNFL) thickness. The eye is part of the central nervous system, and its neurons may be susceptible to degeneration; therefore, changes in the RNFL thickness may reflect microstructural and volume alterations in the brain.

Objective: To explore the association between the peripapillary RNFL thickness and brain alterations in the visual and limbic networks in elderly people without dementia.

Diagnostic value of cerebro-spinal fluid biomarkers in dementia with lewy bodies.

Dementia with Lewy Bodies (DLB) is the second most common form of dementia after Alzheimer's disease (AD), accounting for 15% to 20% of neuropathologically defined cases. Two-thirds of the patients affected are not or misdiagnosed because of the clinical similarity of these two pathologies. In this review, we evaluate the discriminatory power of cerebrospinal fluid (CSF) biomarkers by focusing more specifically on differential diagnosis between DLB and AD.

Relations between C9orf72 expansion size in blood, age at onset, age at collection and transmission across generations in patients and presymptomatic carriers.

A (GGGGCC) repeat expansion in C9orf72 gene is the major cause of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). The relations between the repeats size and the age at disease onset (AO) or the clinical phenotype (FTD vs. ALS) were investigated in 125 FTD, ALS, and presymptomatic carriers.

Diagnostic value of cerebrospinal fluid alpha-synuclein in dementia with Lewy body.

Dementia with Lewy body (DLB) is the second most common form of dementia after Alzheimer's disease (AD), accounting for 15% to 20% of neuropathologically defined cases. Two-thirds of the patients affected are not or misdiagnosed. In this review, we evaluate the discriminatory power of cerebrospinal fluid (CSF) alpha-synuclein by focusing more specifically on differential diagnosis between DLB and AD.

Diagnostic value of Alzheimer's biomarkers in cerebrospinal fluid in dementia with Lewy body.

Dementia with Lewy body (DLB) is the second most common form of dementia after Alzheimer's disease (AD), accounting for 15% to 20% of neuropathologically defined cases. Two-thirds of the patients affected are not or misdiagnosed. In this review, we evaluate the discriminatory power of cerebrospinal fluid (CSF) biomarkers by focusing more specifically on differential diagnosis between DLB and AD.

Evidence for a neural signature of musical preference during silence.

One of the most basic and person-specific affective responses to music is liking. The present investigation sought to determine whether liking was preserved during spontaneous auditory imagery. To this purpose, we inserted two-second silent intervals into liked and disliked songs, a method known to automatically recreate a mental image of these songs.

Predicting dementia using socio-demographic characteristics and the Free and Cued Selective Reminding Test in the general population.

Background: Our study aimed to determine whether the consideration of socio-demographic features improves the prediction of Alzheimer's dementia (AD) at 5 years when using the Free and Cued Selective Reminding Test (FCSRT) in the general older population.

Methods: Our analyses focused on 2558 subjects from the prospective Three-City Study, a cohort of community-dwelling individuals aged 65 years and over, with FCSRT scores. Four "residual scores" and "risk scores" were built that included the FCSRT scores and socio-demographic variables.

Trends in Prevalence of Dementia in French Farmers from Two Epidemiological Cohorts.

Objectives: To determine the prevalence of dementia and cognitive impairment in older people across generations.

Design: Two prospective cohort studies (Personnes Agées QUID (PAQUID), Aging Multidisciplinary Investigation (AMI)).

Setting: Baseline data from two subsamples of older farmers in southwestern France.

Semantic and nonfluent aphasic variants, secondarily associated with amyotrophic lateral sclerosis, are predominant frontotemporal lobar degeneration phenotypes in TBK1 carriers.

Introduction: TBK1 mutations represent a rare novel genetic cause of amyotrophic lateral sclerosis (ALS) without or with dementia. The full spectrum of TBK1 phenotypes has not been completely defined so far.

Methods: We describe the clinical and neuroimaging characteristics of loss-of-function mutation carriers initially presenting with frontotemporal lobar degeneration (FTLD) phenotypes.

Don't forget the lyrics! Spatiotemporal dynamics of neural mechanisms spontaneously evoked by gaps of silence in familiar and newly learned songs.

The vast majority of people experience musical imagery, the sensation of reliving a song in absence of any external stimulation. Internal perception of a song can be deliberate and effortful, but also may occur involuntarily and spontaneously. Moreover, musical imagery is also involuntarily used for automatically completing missing parts of music or lyrics from a familiar song.

Psychotropic Drug Prescription in Patients with Dementia: Nursing Home Residents Versus Patients Living at Home.

Background: Psychotropic drugs are frequently prescribed in nursing homes (NH). Nonetheless, we hoped that institutionalization decreases the number of psychotropic drug classes prescribed, because NH residents may have more psychosocial interventions than patients living at home.

Objective: The aim was to compare the type and number of psychotropic drugs prescribed in elderly NH residents with dementia with those in community-living patients.

Extensive white matter involvement in patients with frontotemporal lobar degeneration: think progranulin.

Importance: Mutations in the progranulin (GRN) gene are responsible for 20% of familial cases of frontotemporal dementias. All cause haploinsufficiency of progranulin, a protein involved in inflammation, tissue repair, and cancer. Carriers of the GRN mutation are characterized by a variable degree of asymmetric brain atrophy, predominantly in the frontal, temporal, and parietal lobes.

Semantic dementia: aspects of the early diagnosis.

Semantic dementia is a lobar atrophy syndrome, related to a degeneration of anterior temporal regions, and characterized by a very predominant impairment of semantic memory. Whereas the diagnosis is relatively easy to establish in the typical form and if the patient is seen early, the emergence of possible additional cognitive or psycho-behavioural disorders can lead to a misdiagnosis in favour of a frontotemporal dementia syndrome or even probable Alzheimer's disease.

[Vascular dementia].

Vascular dementias are the second leading cause of dementia after Alzheimer disease (AD). In recent years, the concept of "cognitive disorders of a vascular origin" has replaced that of vascular dementias. The new term combines vascular dementia in the strict sense of the term with milder cognitive disorders not including dementia but of vascular origins.

A case report of exhibitionist behaviour revealing unsuspected Huntington's disease: a pitfall in forensic medicine.

We present the case of a patient who was recently convicted for exhibitionism whose clinical assessment suggested undiagnosed Huntington's disease. We summarise the clinical characteristics, the genetics involved and the diagnostic difficulties which can be encountered. Finally, we discuss the implications of this diagnosis in forensic medicine.