
Jason WarrenUniversity College London | UCL · Department of Neurodegenerative Disease
Jason Warren
PhD FRACP
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Publications (687)
Background
Responses to individualized music in people living with dementia can be indicated by both verbal and non‐verbal cues. Evidence suggests that elevated pupil dilation responses to familiar vs. unfamiliar music are preserved in people living with typical Alzheimer’s disease (tAD), and to an extent in people with its atypical ‘visual’ varian...
Background
Dysphagia is an important feature of neurodegenerative diseases and potentially life‐threatening in primary progressive aphasia (PPA), but remains poorly characterised in these syndromes. We hypothesised that dysphagia would be more prevalent in nonfluent/agrammatic variant (nfv)PPA than other PPA syndromes, predicted by accompanying mot...
Purpose
Rare forms of dementia bring unique difficulties related to age of onset, impact on family commitments, employment and finances, and also bring distinctive needs for support and care. The aim of the present study was to explore and better understand what the concept of support means for people living with different rare dementia (PLwRD) and...
Background
Patients with behavioural variant frontotemporal dementia (bvFTD) and right temporal variant frontotemporal dementia (rtvFTD) commonly exhibit abnormal hedonic and other behavioural responses to sounds, however hearing dysfunction in this disorder is poorly characterised. Here we addressed this issue using the Queen Square Tests of Audit...
Background
Impaired auditory verbal working memory is a diagnostic hallmark and integral driver of the clinical phenotype in logopenic variant primary progressive aphasia (lvPPA). However, the physiology of the working memory buffer in this syndrome is poorly characterised. Here we addressed the temporal dynamics of auditory verbal working memory i...
Background
Sleep and circadian disruption are associated with increased dementia risk, yet the mechanism remains poorly understood. We examined the relationship between night/shift working in the fourth decade and late‐life brain health. We explored whether significant relationships were mediated by life course factors including cardiovascular risk...
Background
Various explanations have been proposed for how hearing impairment might be associated with increased risk of dementia. Several theories have proposed direct links with Alzheimer’s disease (AD) neuropathology, either due to shared aetiology (i.e. AD proteinopathies cause impaired hearing), or due to an interaction (i.e. functional brain...
Background
Primary Progressive Aphasia (PPA) is a neurodegenerative disorder primarily affecting language abilities, with clinical variants (nonfluent/agrammatic variant [nfvPPA], semantic variant [svPPA], logopenic variant [lvPPA], and mixed‐PPA [mPPA]) categorized based on linguistic features. This study aims to compare PPA cohorts of native spea...
Previous research suggests that emotional prosody perception is impaired in neurodegenerative diseases like Alzheimer’s disease (AD) and primary progressive aphasia (PPA). However, no previous research has investigated emotional prosody perception in these diseases under non-ideal listening conditions. We recruited 18 patients with AD, and 31 with...
Background
Stroke is the second leading cause of disability worldwide. Stroke results in focal neurological deficit and often leads to auditory problems due to its impact on the auditory pathway. Altered connections in the auditory pathway, caused by stroke, can result in hearing difficulties ranging from impaired sound detection to altered auditor...
Human musicality might have co-evolved with social cognition abilities, but common neuroanatomical substrates remain largely unclear. In behavioural variant frontotemporal dementia, social cognitive abilities are profoundly impaired, whereas these are typically spared in Alzheimer’s disease. If musicality indeed shares a neuroanatomical basis with...
Background and objectives:
Current formulations of primary progressive aphasia (PPA) derive largely from English-speaking patients. We hypothesized that language-specific characteristics influence PPA phenotypes in 2 contrasting languages: Italian and English.
Methods:
We undertook a retrospective, cross-sectional, observational comparison of 2...
INTRODUCTION
Interventions to treat speech‐language difficulties in primary progressive aphasia (PPA) often use word accuracy as a highly comparable outcome. However, there are more constructs of importance to people with PPA that have received less attention.
METHODS
Following Core Outcome Set Standards for Development Recommendations (COSSTAD),...
IntroductionSocial cognition is known to be impaired in people with dementia but the differences in social cognitive impairment between MCI and dementia and its subtypes remain unclear. We aimed to systematically review and meta-analyse differences in emotion recognition, theory of mind (ToM) and empathy, between individuals with MCI and those with...
The primary progressive aphasias are rare, language-led dementias, with three main variants: semantic, non-fluent/agrammatic, and logopenic. Whilst semantic variant has a clear neuroanatomical profile, the non-fluent/agrammatic and logopenic variants are difficult to discriminate from neuroimaging. Previous phenotype-driven studies have characteris...
Script Club: motivating change through remote group script training for people with primary progressive aphasia
State of the art: Script training has been shown to improve fluency and grammatical well-formedness in non-fluent PPA, including when delivered via telehealth (Henry et al. Brain 2018;141 1799–1814). Research studies on script training h...
Hearing is multifaceted and the relative contributions of peripheral and central hearing loss are rarely considered together in the context of dementia. Here, we assessed peripheral (as measured with pure-tone audiometry) and central (as measured with dichotic listening) hearing in 19 patients with typical amnestic Alzheimer’s disease (tAD), 10 pat...
Background
Inappropriate trusting behaviour may have significant social, financial and other consequences for people living with dementia. However, its clinical associations and predictors have not been clarified. Here we addressed this issue in canonical syndromes of frontotemporal dementia (FTD) and Alzheimer’s disease (AD).
Methods
In 34 patien...
Background: Prosody refers to nonverbal speech features including pitch, intonation, stress, duration and intensity. These features are critical for successful communication, conveying emotions and distinguishing sentence structures. Previous research suggests that emotional prosody perception is impaired in neurodegenerative diseases like Alzheime...
INTRODUCTION
Although frontotemporal dementia (FTD) with right anterior temporal lobe (RATL) predominance has been recognized, a uniform description of the syndrome is still missing. This multicenter study aims to establish a cohesive clinical phenotype.
METHODS
Retrospective clinical data from 18 centers across 12 countries yielded 360 FTD patien...
Background and purpose
Dysphagia is an important feature of neurodegenerative diseases and potentially life‐threatening in primary progressive aphasia (PPA) but remains poorly characterized in these syndromes. We hypothesized that dysphagia would be more prevalent in nonfluent/agrammatic variant (nfv)PPA than other PPA syndromes, predicted by accom...
The primary progressive aphasias are rare, language-led dementias, with three main variants: semantic, non-fluent/agrammatic, and logopenic. Whilst semantic variant has a clear neuroanatomical profile, the non-fluent/agrammatic and logopenic variants are difficult to discriminate from neuroimaging. Previous phenotype-driven studies have characteris...
Introduction
The term primary progressive aphasia (PPA) describes a group of language-led dementias. Disease-modifying treatments that delay, slow or reverse progression of PPA are currently lacking, though a number of interventions to manage the symptoms of PPA have been developed in recent years. Unfortunately, studies exploring the effectiveness...
Background and purpose
Logopenic variant primary progressive aphasia (lvPPA) is a major variant presentation of Alzheimer's disease (AD) that signals the importance of communication dysfunction across AD phenotypes. A clinical staging system is lacking for the evolution of AD‐associated communication difficulties that could guide diagnosis and care...
Background
It is estimated that rare forms of dementia account for about 7% of all dementias and 10–20% of dementias for those under the age of 65. These conditions bring unique difficulties related to age of onset, impact on family commitments, employment and finances, and also bring distinctive needs for support and care in contrast to Alzheimer’...
Background
Hearing loss has been proposed as a modifiable risk factor for dementia. However, the relationship between hearing, neurodegeneration, and cognitive change, and the extent to which pathological processes such as Alzheimer’s disease and cerebrovascular disease influence these relationships, is unclear.
Methods
Data from 287 adults born i...
Background and objectives
Primary progressive aphasia (PPA) signifies a diverse group of neurodegenerative disorders principally affecting language functions. The major syndromic variants of PPA present with distinct profiles of linguistic deficits. However, current concepts and diagnosis of PPA are largely based on English-speaking patients, while...
Objectives
On phenotypic and neuroanatomical grounds, music exposure might potentially affect the clinical expression of behavioural variant frontotemporal dementia (bvFTD). However, this has not been clarified.
Methods
14 consecutive patients with bvFTD fulfilling consensus diagnostic criteria were recruited via a specialist cognitive clinic. Ear...
Neurodegenerative dementias have a profound impact on higher-order cognitive and behavioural functions. Investigating macroscale functional networks through cortical gradients provides valuable insights into the neurodegenerative dementia process and overall brain function. This approach allows for the exploration of unimodal-multimodal differentia...
Background
Perceptual plasticity depends on dynamic, coordinated synaptic tuning across neural circuits and is therefore likely to be vulnerable to neurodegenerative pathologies. However, the nature and limits of perceptual plasticity in dementia are largely unknown. Here we addressed this issue using a dramatic instance of auditory perceptual plas...
Background
Music has gained more attention than other art forms as a potent tool in supporting cognitive, emotional and social functioning in dementia populations. Methods for measuring moment‐to‐moment responses ‐ rather than comparing pre‐ and post‐stimulus effects ‐ may enable tailoring of musical experiences for the individual. The current stud...
Background
Primary progressive aphasia (PPA) is an atypical neurodegenerative dementia with three main clinical variants: semantic (svPPA), non‐fluent/agrammatic (nfvPPA) and logopenic (lvPPA). While svPPA is typically associated with left anterior temporal lobe atrophy, neuroimaging findings in nfvPPA, lvPPA and PPA not otherwise specified (PPA‐no...
Background
Accurately interpreting a speaker’s nonverbal emotional vocal signals (their prosody) is often essential for successful communication. In the noisy conditions of daily life, this presents the brain with a difficult computational challenge that may be vulnerable to neurodegenerative pathologies. However, processing of acoustically degrade...
Background
Sleep disturbance is associated with an increased risk of developing Alzheimer’s disease, yet the relationship between sleep metrics and brain pathology remains poorly understood. Complementing ‘traditional’ metrics such as total sleep time (TST) and sleep efficiency (SE), the midpoint of sleep, interdaily stability and intradaily variab...
Background
Hearing loss has emerged as a marker of cognitive decline and potential treatment target in dementia, however the symptom profiles that characterise hearing impairment in different dementia syndromes are poorly characterised. Beyond sound detection, these diseases impair aspects of auditory perception that are crucial for communication i...
Background
Impaired auditory verbal working memory is a diagnostic hallmark and integral driver of the clinical phenotype in logopenic aphasia. However, the physiology of the working memory buffer in this syndrome is poorly characterised. Here we addressed the temporal dynamics of auditory verbal working memory in patients with logopenic aphasia an...
Background
Patients with behavioural variant frontotemporal dementia commonly exhibit abnormal hedonic and other behavioural responses to sounds, however hearing dysfunction in this disorder is poorly characterised. Here we addressed this issue using the Queen Square Tests of Auditory Cognition (QSTAC) – a neuropsychological battery for the systema...
Background
The cognitive organisation of working memory for nonverbal sounds and the unusual musical ability of absolute pitch is poorly understood. We recently had the opportunity to investigate this in a musician with absolute pitch who developed posterior cortical atrophy (PCA), the canonical ‘visual variant’ of Alzheimer’s disease.
Method
We s...
Background
Sleep abnormalities are increasingly recognized as markers and potential drivers of neurodegenerative proteinopathies. However, the sleep phenotype of less common dementias remains poorly characterised. In particular, clinical experience suggests that patients with focal temporal lobe degeneration in the frontotemporal dementia spectrum...
Background
While impaired hearing has emerged as a major potentiating factor for dementia, the basis for this relationship has not been defined. Most studies have emphasised the role of peripheral hearing loss. However, we hear with our brains: natural listening places immense computational demands on neural circuitry, and the neural networks pre‐e...
Background
Frontotemporal dementia (FTD) is characterized by abnormal white matter (WM) integrity measured with conventional diffusion tensor imaging techniques. No study has yet investigated the microstructural WM changes across different clinical FTD forms using the novel neurite orientation dispersion and density imaging (NODDI). Here, we focuse...
Background
Music has gained more attention than other art forms as a potent tool in supporting cognitive, emotional and social functioning in dementia populations. Methods for measuring moment‐to‐moment responses ‐ rather than comparing pre‐ and post‐stimulus effects ‐ may enable tailoring of musical experiences for the individual. The current stud...
Background
Despite considerable interest in music as an index of brain function and a therapeutic modality in dementia, the neural correlates of music processing in different dementias remain poorly defined. In the frontotemporal dementia spectrum, behavioral variant frontotemporal dementia (bvFTD) and semantic variant primary progressive aphasia (...
Background
While it is well established that musical expertise can shape brain structure and function, the potential of premorbid musical training to modify the clinical expression of neurodegenerative brain pathologies has been largely unexplored. In healthy subjects, long‐term musical exposure is associated with both enhanced emotion recognition...
Background
Primary progressive aphasia (PPA) is an atypical neurodegenerative dementia with three main clinical variants: semantic (svPPA), non‐fluent/agrammatic (nfvPPA) and logopenic (lvPPA). While svPPA is typically associated with left anterior temporal lobe atrophy, neuroimaging findings in nfvPPA, lvPPA and PPA not otherwise specified (PPA‐no...
The alignment between visual pathway signaling and pupil dynamics offers a promising non-invasive method to further illuminate the mechanisms of human color perception. However, only limited research has been done in this area and the effects of healthy aging on pupil responses to the different color components have not been studied yet. Here we ai...
Background
Binary reversals (exemplified by ‘yes’/‘no’ confusions) have been described in patients with primary progressive aphasia (PPA) but their diagnostic value and phenotypic correlates have not been defined.
Methods
We conducted a retrospective cohort study analysing demographic, clinical, neuropsychological, linguistic and behavioural data...
Here, we review recent progress in the diagnosis and management of primary progressive aphasia—the language-led dementias. We pose six key unanswered questions that challenge current assumptions and highlight the unresolved difficulties that surround these diseases. How many syndromes of primary progressive aphasia are there—and is syndromic diagno...
Background: Primary progressive aphasia describes a group of three rare language-led dementias: semantic, logopenic, and non-fluent. The small number of conversation analysis studies to date suggest that repair and turn-construction practices in primary progressive aphasia are similar to those seen in post-stroke aphasia. This study investigates th...
Objectives
This study was part of a process evaluation for a single‐blind, randomized controlled pilot study comparing Better Conversations with Primary Progressive Aphasia (BCPPA), an approach to communication partner training, with no speech and language therapy treatment. It was necessary to explore fidelity of delivery (delivery of intervention...
The foundation of art processes in the social brain can guide the scientific study of how human beings perceive and interact with their environment. Here, we applied the theoretical frameworks of the social and artistic brain connectomes to an eye-tracking paradigm with the aim to elucidate how different viewing conditions and social cues influence...
INTRODUCTION
Here we set out to create a symptom‐led staging system for the canonical semantic and non‐fluent/agrammatic variants of primary progressive aphasia (PPA), which present unique diagnostic and management challenges not well captured by functional scales developed for Alzheimer's disease and other dementias.
METHODS
An international PPA...
Background
Peripheral hearing impairment has been proposed as a risk factor for dementia. However, the relationship between hearing ability, neurodegeneration and cognitive decline, and the extent to which pathological processes associated with increased risk of specific causes of dementia, such as β‐amyloid and small vessel disease, influence thes...
Background
Aberrant reward processing is a cardinal feature of frontotemporal dementias (FTD), driving abnormal socio‐emotional behaviours that define these diseases clinically. However, the hedonic architecture of FTD remains poorly characterised. Here we addressed this issue for a multimodal spectrum of hedonic behaviours in a large patient cohor...
Background:
Frontotemporal dementia (FTD) is an early onset dementia that is diagnosed in ~20% of the progressive dementia cases. Heterogeneity in FTD clinical presentation too often delays clinical diagnosis and calls for molecular biomarkers to assist diagnosis, including cell free microRNAs (miRNA). However, nonlinearity in the relationship of...
Successful communication in daily life depends on accurate decoding of speech signals that are acoustically degraded by challenging listening conditions. This process presents the brain with a demanding computational task that is vulnerable to neurodegenerative pathologies. However, despite recent intense interest in the link between hearing impair...
The primary progressive aphasias (PPA) present complex and diverse challenges of diagnosis, management and prognosis. A clinically-informed, syndromic staging system for PPA would take a substantial step toward meeting these challenges. This study addressed this need using detailed, multi-domain mixed-methods symptom surveys of people with lived ex...
Primary progressive aphasia is most commonly a sporadic disorder but in some cases it can be genetic. This study aimed to understand the clinical, cognitive and imaging phenotype of the genetic forms of primary progressive aphasia in comparison to the canonical nonfluent, semantic and logopenic subtypes seen in sporadic disease. Participants with g...
Abnormal reward processing is a hallmark of neurodegenerative diseases, most strikingly in frontotemporal dementia. However, the phenotypic repertoire and neuroanatomical substrates of abnormal reward behaviour in these diseases remain incompletely characterised and poorly understood. Here we addressed these issues in a large, intensively phenotype...
In daily life, spoken messages are often interrupted by extraneous sounds. However, we generally perceive such messages as continuous: our brains automatically and efficiently ‘repair’ interrupted speech signals by phonemic restoration, a fundamental physiological process whereby speech sounds that are obscured by noise are ‘filled‐in’ perceptually...
Background
Clinical endpoints for upcoming therapeutic trials in frontotemporal dementia (FTD) are increasingly urgent. Cognitive composite scores are often used as endpoints but are lacking in genetic FTD. We aimed to create cognitive composite scores for genetic frontotemporal dementia (FTD) as well as recommendations for recruitment and duration...
In daily life, spoken messages are often “degraded” by competing sounds and vocal idiosyncrasies. Comprehension of degraded speech demands intense computations across distributed neural networks; it is likely to present a particular challenge for patients with neurodegenerative pathologies. However, this issue has not been studied systematically. H...
Accurately interpreting a speaker’s nonverbal emotional vocal signals (their prosody) is often essential for successful communication. In the noisy conditions of daily life, this presents the brain with a difficult computational challenge that may be vulnerable to neurodegenerative pathologies. However, processing of acoustically degraded prosody h...
Background:
Primary progressive aphasia (PPA) describes a group of language-led dementias. PPAs are complex, diverse and difficult to diagnose, and therefore conventional models of aphasia and dementia treatment do not meet their needs. The research evidence on intervention for PPA is developing, but to date there are only a few case studies explo...
Objectives
We explored whether adapting neuropsychological tests for online administration during the COVID-19 pandemic was feasible for dementia research.
Design
We used a longitudinal design for healthy controls, who completed face-to-face assessments 3–4 years before remote assessments. For patients, we used a cross-sectional design, contrastin...
UCL
The recently recognised association between hearing impairment and dementia has important clinical and public health implications but remains poorly understood. In daily life, the most important sound we hear is speech: making sense of spoken messages that are ‘degraded’ by noise is fundamental to suc- cessful communication and depends on neura...
Objectives:
Frontotemporal dementia (FTD) can present with changes in music appreciation. Research has suggested a relationship of altered music appreciation phenotypes with typical socio-emotional changes. We aimed to determine the prevalence and severity of music appreciation phenotypes in FTD and study the relationship with emotion recognition...
Primary progressive aphasia remains a diagnostic challenge despite (or even because of) the increasing availability of ancillary tests and biomarkers. We present a 67-year-old man with apparently sporadic logopenic aphasia and positive Alzheimer biomarkers who was subsequently found also to have a pathogenic mutation in the progranulin gene. This w...
Background
Frontotemporal dementia (FTD) is a spectrum of diseases characterised by language, behavioural and motor symptoms. Among the different subcortical regions implicated in the FTD symptomatology, the hypothalamus regulates various bodily functions, including eating behaviours which are commonly present across the FTD spectrum. The pattern o...
Phonemic restoration – perceiving speech sounds that are actually missing – is a fundamental perceptual process that ‘repairs’ interrupted spoken messages during noisy everyday listening. As a dynamic, integrative process, phonemic restoration is potentially affected by neurodegenerative pathologies but this has not been clarified. Here we studied...
Objectives
We explored whether adapting traditional neuropsychological tests for online administration against the backdrop of COVID-19 was feasible for people with diverse forms of dementia and healthy older controls. We compared face-to-face and remote settings to ascertain whether remote administration affected performance.
Design
We used a lon...
Introduction:
Oculomotor function has not been systematically studied in frontotemporal dementia (FTD) and yet may offer a simple target to monitor disease a