Alexander Gerhard
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View article: TMEM106B modulates disease severity in genetic frontotemporal dementia phenoconverters
TMEM106B modulates disease severity in genetic frontotemporal dementia phenoconverters Open
Background A common variant within TMEM106B is associated with risk for Frontotemporal Lobar Degeneration‐Tar DNA binding Protein‐43 (FTLD‐TDP). A recent study has shown that the minor allele G of TMEM106B‐rs1990622 confers protection agai…
View article: Structure‐function decoupling in genetic frontotemporal dementia
Structure‐function decoupling in genetic frontotemporal dementia Open
Background Functional network integrity is important for maintaining cognitive performance during the 10‐20 year presymptomatic period of frontotemporal dementia (FTD), conferring resilience to advancing neuropathology and atrophy. The ext…
View article: Cellular signatures underlying functional resilience in presymptomatic frontotemporal dementia
Cellular signatures underlying functional resilience in presymptomatic frontotemporal dementia Open
Frontotemporal dementia (FTD) shows autosomal dominant transmission in up to a third of families, enabling the study of presymptomatic and prodromal phases. Despite self-reported well-being and normal daily cognitive functioning, brain str…
View article: Cortical microstructure is associated with disease severity and clinical progression in genetic frontotemporal dementia: a GENFI study
Cortical microstructure is associated with disease severity and clinical progression in genetic frontotemporal dementia: a GENFI study Open
The study of genetic frontotemporal dementia (FTD) allows investigating its earliest presymptomatic stages. Using cross-sectional T1-weighted and diffusion-weighted MRI, we test the hypothesis that cortical microstructural alterations, qua…
View article: White matter hyperintensities precede other biomarkers in <i>GRN</i> frontotemporal dementia
White matter hyperintensities precede other biomarkers in <i>GRN</i> frontotemporal dementia Open
INTRODUCTION Increased white matter hyperintensities (WMHs) have been reported in genetic frontotemporal dementia (FTD) in small studies, but the sequence of WMH abnormalities relative to other biomarkers is unclear. METHODS Using a large …
View article: Cross‐country variance in facial emotion recognition in presymptomatic and symptomatic behavioral variant frontotemporal dementia: Insights from the GENFI and ReDLat consortia
Cross‐country variance in facial emotion recognition in presymptomatic and symptomatic behavioral variant frontotemporal dementia: Insights from the GENFI and ReDLat consortia Open
INTRODUCTION We investigated international differences in facial emotion recognition (FER) across stages of frontotemporal dementia (FTD). Previous studies may have missed early decline by combining data and masking variations in FER acros…
View article: Cerebrovascular Reactivity at Rest and Its Association With Cognitive Function in People With Genetic Frontotemporal Dementia
Cerebrovascular Reactivity at Rest and Its Association With Cognitive Function in People With Genetic Frontotemporal Dementia Open
CVR impairment in genetic FTD has a predilection for the middle frontal and posterior cortex, and its preservation may yield a cognitive benefit for at-risk individuals. Although findings do not provide causality and warrant replication, t…
View article: Most prominent challenges in translational neuroscience and strategic solutions to bridge the gaps: Perspectives from an editorial board interrogation
Most prominent challenges in translational neuroscience and strategic solutions to bridge the gaps: Perspectives from an editorial board interrogation Open
Recent progress in translational neuroscience has significantly advanced our understanding of neurological diseases. Research progress closely went in line with innovations in research methods, which have expanded our insights considerably…
View article: Executive Function Deficits in Genetic Frontotemporal Dementia
Executive Function Deficits in Genetic Frontotemporal Dementia Open
Some individuals with C9orf72 mutations show difficulties with executive function from very early on in the disease and this continues to deteriorate with disease severity. By contrast, similar difficulties occur only in the later stages o…
View article: Accuracy of blood-based neurofilament light to different genetic frontotemporal dementia from primary psychiatric disorders
Accuracy of blood-based neurofilament light to different genetic frontotemporal dementia from primary psychiatric disorders Open
Background Genetic frontotemporal dementia (FTD) along with Alzheimer's disease (AD), is one of the most prevalent early-onset dementias. The differential diagnosis of FTD from primary psychiatric disorder (PPD) has been challenging due to…
View article: Structural and functional connectivity in tau mutation carriers: from presymptomatic to symptomatic frontotemporal dementia
Structural and functional connectivity in tau mutation carriers: from presymptomatic to symptomatic frontotemporal dementia Open
INTRODUCTION Microtubule‐associated protein tau ( MAPT ) mutations cause frontotemporal dementia (FTD), characterised by behavioural, language, and motor impairments due to brain connectivity disruptions. We investigated structural and fun…
View article: Hemispheric asymmetry of [ <sup>11</sup> C]( <i>R)</i> PK11195 binding to translocator protein 18 kDa (TSPO) in normal brain
Hemispheric asymmetry of [ <sup>11</sup> C]( <i>R)</i> PK11195 binding to translocator protein 18 kDa (TSPO) in normal brain Open
Many positron emission tomography (PET) imaging studies in health and disease of the translocator protein 18 kDa (TSPO) using different radioligands have been published, however, only few separately reported left and right regions of inter…
View article: Anatomical progression of genetic frontotemporal lobar degeneration across the lifespan
Anatomical progression of genetic frontotemporal lobar degeneration across the lifespan Open
The recent development of brain charts for the human lifespan offers an ideal modelling framework for pathologies such as genetic frontotemporal lobar degeneration (FTLD) which likely involve both neurodevelopmental and neurodegenerative p…
View article: Disease-modifying effects of <i>TMEM106B</i> in genetic frontotemporal dementia: a longitudinal GENFI study
Disease-modifying effects of <i>TMEM106B</i> in genetic frontotemporal dementia: a longitudinal GENFI study Open
Common variants within TMEM106B are associated with risk for frontotemporal lobar degeneration with TDP-43 pathology (FTLD-TDP). The G allele of the top single nucleotide polymorphism, rs1990622, confers protection against FTLD-TDP, includ…
View article: Cognitive and neuropsychiatric profiles distinguish atypical parkinsonian syndromes
Cognitive and neuropsychiatric profiles distinguish atypical parkinsonian syndromes Open
Atypical parkinsonian syndromes are distinguished from Parkinson's disease (PD) by additional neurological signs and characteristic underlying neuropathology. However, they can be diagnostically challenging, rapidly progressive and are oft…
View article: Sex differences in clinical phenotypes of behavioral variant frontotemporal dementia
Sex differences in clinical phenotypes of behavioral variant frontotemporal dementia Open
INTRODUCTION Higher male prevalence in sporadic behavioral variant frontotemporal dementia (bvFTD) has been reported. We hypothesized differences in phenotypes between genetic and sporadic bvFTD females resulting in underdiagnosis of spora…
View article: Cellular signatures of functional resilience in presymptomatic frontotemporal dementia
Cellular signatures of functional resilience in presymptomatic frontotemporal dementia Open
Frontotemporal dementia (FTD) shows autosomal dominant transmission in up to a third of families, enabling the study of presymptomatic and prodromal phases. Despite self-reported well-being and normal daily cognitive functioning, brain str…
View article: Proteomic analysis reveals distinct cerebrospinal fluid signatures across genetic frontotemporal dementia subtypes
Proteomic analysis reveals distinct cerebrospinal fluid signatures across genetic frontotemporal dementia subtypes Open
We used an untargeted mass spectrometric approach, tandem mass tag proteomics, for the identification of proteomic signatures in genetic frontotemporal dementia (FTD). A total of 238 cerebrospinal fluid (CSF) samples from the Genetic FTD I…
View article: Thalamus involvement in genetic frontotemporal dementia assessed using structural and diffusion MRI: a GENFI study
Thalamus involvement in genetic frontotemporal dementia assessed using structural and diffusion MRI: a GENFI study Open
Thalamic subregions are commonly, but variably, affected by different forms of frontotemporal dementia. We aimed to better characterize thalamic subregional involvement in genetic frontotemporal dementia with a recently published thalamus …
View article: Association of Initial Side of Brain Atrophy With Clinical Features and Disease Progression in Patients With <i>GRN</i> Frontotemporal Dementia
Association of Initial Side of Brain Atrophy With Clinical Features and Disease Progression in Patients With <i>GRN</i> Frontotemporal Dementia Open
FTD-GRN affects the brain hemispheres asymmetrically and causes 2 anatomical asymmetry patterns depending on the side of the disease onset. We demonstrated that these 2 anatomical asymmetry patterns present different symptoms, severity at …
View article: Association of Changes in Cerebral and Hypothalamic Structure With Sleep Dysfunction in Patients With Genetic Frontotemporal Dementia
Association of Changes in Cerebral and Hypothalamic Structure With Sleep Dysfunction in Patients With Genetic Frontotemporal Dementia Open
These findings suggest that sleep dysfunction in patients with genetic FTD may be attributable to atrophy in sleep-relevant hypothalamic subunits, with the most severe and consistent deficits observed in MAPT carriers. While biologically p…
View article: Frontoparietal network integrity supports cognitive function in pre‐symptomatic frontotemporal dementia: Multimodal analysis of brain function, structure, and perfusion
Frontoparietal network integrity supports cognitive function in pre‐symptomatic frontotemporal dementia: Multimodal analysis of brain function, structure, and perfusion Open
INTRODUCTION Genetic mutation carriers of frontotemporal dementia can remain cognitively well despite neurodegeneration. A better understanding of brain structural, perfusion, and functional patterns in the pre‐symptomatic stage could info…
View article: Identifying latent disease factors differently expressed in patient subgroups using group factor analysis
Identifying latent disease factors differently expressed in patient subgroups using group factor analysis Open
In this study, we propose a novel approach to uncover subgroup-specific and subgroup-common latent factors addressing the challenges posed by the heterogeneity of neurological and mental disorders, which hinder disease understanding, treat…
View article: Frequency and Longitudinal Course of Behavioral and Neuropsychiatric Symptoms in Participants With Genetic Frontotemporal Dementia
Frequency and Longitudinal Course of Behavioral and Neuropsychiatric Symptoms in Participants With Genetic Frontotemporal Dementia Open
We identified multiple clusters of behavioral and neuropsychiatric symptoms in participants with genetic FTD that relate to distinct cerebral atrophy patterns. Their severity depends on time, affected gene, sex, and education. These clinic…
View article: Long Non-Coding RNA Profile in Genetic Symptomatic and Presymptomatic Frontotemporal Dementia: A GENFI Study
Long Non-Coding RNA Profile in Genetic Symptomatic and Presymptomatic Frontotemporal Dementia: A GENFI Study Open
Background: Long non-coding RNAs (lncRNAs) play crucial roles in gene regulation and are implicated in neurodegenerative diseases, including frontotemporal dementia (FTD). However, their expression patterns and potential as biomarkers in g…
View article: Executive function deficits in genetic frontotemporal dementia: results from the GENFI study
Executive function deficits in genetic frontotemporal dementia: results from the GENFI study Open
Background Executive dysfunction is a core feature of frontotemporal dementia (FTD). Whilst there has been extensive research into such impairments in sporadic FTD, there has been little research in the familial forms. Methods 752 individu…
View article: Longitudinal cerebral perfusion in presymptomatic genetic frontotemporal dementia: GENFI results
Longitudinal cerebral perfusion in presymptomatic genetic frontotemporal dementia: GENFI results Open
INTRODUCTION Effective longitudinal biomarkers that track disease progression are needed to characterize the presymptomatic phase of genetic frontotemporal dementia (FTD). We investigate the utility of cerebral perfusion as one such biomar…
View article: Extending the phenotypic spectrum assessed by the CDR plus NACC FTLD in genetic frontotemporal dementia
Extending the phenotypic spectrum assessed by the CDR plus NACC FTLD in genetic frontotemporal dementia Open
INTRODUCTION We aimed to expand the range of the frontotemporal dementia (FTD) phenotypes assessed by the Clinical Dementia Rating Dementia Staging Instrument plus National Alzheimer's Coordinating Center Behavior and Language Domains (CDR…
View article: Cerebrovascular reactivity impairment in genetic frontotemporal dementia
Cerebrovascular reactivity impairment in genetic frontotemporal dementia Open
INTRODUCTION Cerebrovascular reactivity (CVR) is an indicator of cerebrovascular health and its signature in hereditary frontotemporal dementia (FTD) remains unknown. We investigated CVR in genetic FTD and its relationship to cognition. ME…