Altered Gaze Control During Emotional Face Exploration in Patients With Amyotrophic Lateral Sclerosis.

Objectives: Up to 50% of patients with amyotrophic lateral sclerosis (ALS) present with cognitive problems and behavioral dysfunctions including recognition of human faces presenting different emotions. We investigated whether impaired processing of emotional faces is associated with abnormal scan paths during visual exploration.

Methods: Cognitively unimpaired patients with ALS (n = 45) and matched healthy controls (n = 37) underwent neuropsychological assessment and video-based eye tracking.

[Rare forms of dementia-Atypical variants of Alzheimer's dementia].

In Germany, around 1.8 million people currently suffer from dementia and the numbers are increasing. The main cause of dementia is Alzheimer's disease.

The TRACK-MS Test Battery: A Very Brief Tool to Track Multiple Sclerosis-Related Cognitive Impairment.

Tracking cognition in patients with multiple sclerosis (MS) is important for detection of disease progression but it is often not performed in routine settings due to time constraints. This exploratory cohort study aims to develop a very brief repeatable tracking tool with comparable test quality criteria to the current gold standard, the Brief International Cognitive Assessment for MS (BICAMS). The study included 88 participants (22 healthy controls, 66 MS patients) who were examined at baseline and at one-year follow-up.

Cognition in the course of ALS-a meta-analysis.

: The goal of this meta-analysis is to improve insight into the development of cognition over the course of ALS and to assess predictors of cognitive performance.: A literature search was conducted in Pubmed and Web of Science on 29 July 2019 and 16 March 2021. Data were screened in Endnote Version X9 (London, UK).

Predicting disease progression in behavioral variant frontotemporal dementia.

Introduction: The behavioral variant of frontotemporal dementia (bvFTD) is a rare neurodegenerative disease. Reliable predictors of disease progression have not been sufficiently identified. We investigated multivariate magnetic resonance imaging (MRI) biomarker profiles for their predictive value of individual decline.

Clinicoanatomical substrates of selfish behaviour in amyotrophic lateral sclerosis - An observational cohort study.

Objective: ALS primarily affects motor functions, but cognitive functions, including social understanding, may also be impaired. Von Economo neurons (VENs) are part of the neuronal substrate of social understanding and these cells are histopathologically altered in ALS. We investigated whether activity in areas including VENs is associated with an impairment of cognitive tasks that mirror social functioning.

Segmental Alterations of the Corpus Callosum in Progressive Supranuclear Palsy: A Multiparametric Magnetic Resonance Imaging Study.

The regional distribution of the widespread cerebral morphological alterations in progressive supranuclear palsy (PSP) is considered to include segmental parts of the corpus callosum (CC). The study was designed to investigate the regional white matter (WM) of the CC by T1 weighted magnetic resonance imaging (T1w MRI) data combined with diffusion tensor imaging (DTI) data in PSP patients, differentiated in the variants Richardson syndrome and PSP-parkinsonism, and to compare them with Parkinson's Disease (PD) patients and healthy controls, in order to identify macro- and micro-structural alterations . MRI-based WM mapping was used to perform an operator-independent segmentation for the different CC segments in 66 PSP patients vs.

Clinico-genetic findings in 509 frontotemporal dementia patients.

Frontotemporal dementia (FTD) is a clinically and genetically heterogeneous disorder. To which extent genetic aberrations dictate clinical presentation remains elusive. We investigated the spectrum of genetic causes and assessed the genotype-driven differences in biomarker profiles, disease severity and clinical manifestation by recruiting 509 FTD patients from different centers of the German FTLD consortium where individuals were clinically assessed including biomarker analysis.

Neuropsychological deficits have only limited impact on psychological well-being in amyotrophic lateral sclerosis.

Objective: To investigate the association between neuropsychological deficits and psychological well-being in amyotrophic lateral sclerosis (ALS).

Methods: Subjective (Schedule for the Evaluation of the Individual Quality of Life-Direct Weighting, SEIQoL-DW) and global quality of life (QoL; Anamnestic Comparative Self-Assessment, ACSA) as well as depression (ALS-Depression-Inventory, ADI-12) as indicators for psychological well-being were measured in 214 patients with ALS and correlated with neurocognitive performance assessed by the Edinburgh Cognitive and Behavioural ALS Screen (ECAS). Primary caregivers evaluated behaviour.

Caregivers' divergent perspectives on patients' well-being and attitudes towards hastened death in Germany, Poland and Sweden.

During the course of amyotrophic lateral sclerosis (ALS), patients and their families are faced with existential decisions concerning life-prolonging and -shortening measures. Correct anticipation of patient's well-being and preferences is a prerequisite for patient-centered surrogate decision making. : In Germany ( = 84), Poland ( = 77) and Sweden ( = 73) patient-caregiver dyads were interviewed.

Living with global amnesia: self-established compensation strategies of a patient with severe memory impairment - a narrative report.

We report the case of C.H., a 48-year-old patient with global amnesia caused by herpes simplex encephalitis at the age of 20 and subsequent extensive bilateral temporal lobe lesions.

Quantifying progression in primary progressive aphasia with structural neuroimaging.

Introduction: The term primary progressive aphasia (PPA) sums up the non-fluent (nfv), the semantic (sv), and the logopenic (lv) variant. Up to now, there is only limited data available concerning magnetic resonance imaging volumetry to monitor disease progression.

Methods: Structural brain imaging and an extensive assessment were applied at baseline and up to 4-year(s) follow-up in 269 participants.

Deficits in verbal fluency in presymptomatic mutation gene carriers-a developmental disorder.

Background: A mutation in constitute a cross-link between amyotrophic lateral sclerosis (ALS) and fronto-temporal dementia (FTD). At clinical manifestation, both patient groups may present with either cognitive impairment of predominantly behaviour or language (in FTD) or motor dysfunctions (in ALS).

Methods: In total, 36 non-symptomatic mutation carriers from ALS or FTD families were examined, including 21 subjects with and 15 with mutations.

Disease progression but not physical state per se determines mental wellbeing in ALS.

Background: Studies provide inconclusive results on the question whether loss of mental wellbeing is actually associated with decline in physical function in amyotrophic lateral sclerosis (ALS). The purpose of this study was to determine predictors of mental wellbeing in ALS.

Methods: In total, n = 330 ALS patients were interviewed on parameters of mental wellbeing to evaluate the patients' capacity of psychosocial adaptation.

Neural substrates of anorexia nervosa patient's deficits to decode emotional information.

Purpose: The aim of the study was to define specific substrates of pathological behaviour patterns by analysing cortical activity using functional magnetic resonance imaging (fMRI) during an emotional processing task.

Methods: In a sample of N = 11 adolescent patients with AN (16.36 years, SD ± 1.

An observational study on quality of life and preferences to sustain life in locked-in state.

Objective: This is an observational study on well-being and end-of-life preferences in patients with amyotrophic lateral sclerosis (ALS) in the locked-in state (LIS) in a Polish sample within the EU Joint Programme-Neurodegenerative Disease Research study NEEDSinALS (NEEDSinALS.com).

Methods: In this cross-sectional study, patients with ALS in LIS (n = 19) were interviewed on well-being (quality of life, depression) as a measure of psychosocial adaptation, coping mechanisms, and preferences towards life-sustaining treatments (ventilation, percutaneous endoscopic gastroscopy) and hastened death.

Moral judgment in patients with behavioral variant of frontotemporal dementia and amyotrophic lateral sclerosis: no impairment of the moral position, but rather its execution.

Objective: To investigate moral judgment competence in patients with the behavioral variant frontotemporal dementia (bvFTD) compared to amyotrophic lateral sclerosis (ALS) and controls.

Methods: N = 12 bvFTD, N = 22 ALS patients and N = 19 neurological unimpaired controls were examined. In the 'Moral Competence Test' (MCT), participants had to evaluate two moral dilemmas by predefined arguments to measure the consistency of their moral judgments.

Cognitive and behavioral impairments in German and Chinese ALS populations - a post-hoc comparison of national study data.

This study aimed to clarify whether differences between German and Chinese studies using the standardized Edinburgh Cognitive and Behavioural ALS Screen (ECAS), might be explained by differences in translated versions of ECAS, by patient demographic or clinical characteristics or by population-specific factors. Comparisons were performed on data from two previous studies in Germany and China. We found except for spelling task (p = 0.

Story of the ALS-FTD continuum retold: rather two distinct entities.

Objective: To determine the evolution and profile of cognitive and behavioural deficits in amyotrophic lateral sclerosis (ALS) and behavioural variant frontotemporal dementia (bvFTD) to disentangle the development of FTD in ALS and vice versa.

Methods: In a prospective design, cognitive and behavioural profiles of 762 patients with motor predominant ALS (flail arm/leg syndrome, primary lateral sclerosis, pseudobulbar palsy, ALS) and behavioural predominant FTD (bvFTD, ALS-FTD) were determined and caregivers of patients with ALS were asked on the evolution of behavioural symptoms. Data were compared with 49 healthy controls.

Serum neurofilament light chain in behavioral variant frontotemporal dementia.

Objective: To determine the association of serum neurofilament light chain (NfL) with functional deterioration and brain atrophy during follow-up of patients with behavioral variant frontotemporal dementia (bvFTD).

Methods: Blood NfL levels from 74 patients with bvFTD, 26 with Alzheimer disease (AD), 17 with mild cognitive impairment (MCI), and 15 healthy controls (Con) at baseline and follow-up were determined and analyzed for the diagnostic potential in relation to functional assessment (Clinical Dementia Rating Scale Sum of Boxes [CDR-SOB], frontotemporal lobar degeneration-related CDR-SOB, Mini-Mental State Examination [MMSE]) and brain volumetry.

Results: At baseline, serum NfL level correlated with CSF NfL (bvFTD = 0.

Screening for Cognitive Function in Complete Immobility Using Brain-Machine Interfaces: A Proof of Principle Study.

In many neurological conditions, there is a combination of decline in physical function and cognitive abilities. For far advanced stages of physical disability where speaking and hand motor abilities are severely impaired, there is a lack of standardized approach to screen for cognitive profile. = 40 healthy subjects were included in the study.

Attitudes of Nurses Toward Disability and Treatment in Space-Occupying Middle Cerebral Artery Stroke.

Background: Attitudes toward the degree of acceptable disability and the importance of aphasia are critical in deciding on decompressive hemicraniectomy (DHC) in space-occupying middle cerebral artery stroke (SOS). The attitudes of nurses deserve strong attention, because of their close interaction with patients during acute stroke treatment.

Methods: This is a multicenter survey among 627 nurses from 132 hospitals in Germany.