ORCID Profile
0000-0002-5205-9712
Current Organisations
University of Southampton
,
University Hospital Southampton NHS Foundation Trust
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Publisher: Wiley
Date: 03-2005
DOI: 10.1002/MDS.20325
Abstract: Movement disorders may present acutely, and failure to recognize and exclude important differential diagnoses can result in significant morbidity or mortality. Unfortunately, much of the literature pertaining to this topic is scattered and not easily accessible. This review aims to address this deficit. Movement disorder emergencies are discussed according to their most likely mode of presentation. Diagnostic considerations and early management principles are reviewed, along with appropriate pathophysiology where relevant.
Publisher: Informa UK Limited
Date: 17-06-2009
DOI: 10.1080/13554790802632892
Abstract: Social functioning in FTD is profoundly affected, and forms the basis for the clinical diagnosis of the behavioural variant of the disease (bv-FTD). In particular, there are deficits in emotional processing, but the inter-relationship of such deficits to other aspects of social functioning remains unclear. We studied patients with bv-FTD (n = 14) and AD (n = 14), and compared their performance on a test of emotion recognition with their scores on two carer-based assessments: the Disability Assessment for Dementia (DAD) of activities in daily living (ADL), and the Cambridge Behavioural Inventory (CBI). The bv-FTD group had significantly greater impairments in ADLs, and had higher scores on the CBI, compared to the AD group. Despite a deficit in emotion recognition, particularly involving negative emotions, in the FTD group relative to AD and controls, performance on this task did not correlate with ADL ratings which instead, correlated highly with carer-rated apathy levels on the CBI. The study highlights the multifactorial nature of social dysfunction in FTD which is important in the management of these patients and in designing effective behavioural and therapeutic interventions. The relationship of emotional processing to other aspects of social cognition in FTD is reviewed.
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 12-2006
Publisher: S. Karger AG
Date: 2007
DOI: 10.1159/000100973
Abstract: i Background/Aims: /i The status of imaging findings in the clinical diagnosis of frontotemporal dementia (FTD) remains uncertain while they may be supportive of a diagnosis of frontotemporal dementia, they are not mandatory. Our aim was to assess patterns of lobar atrophy in a large s le of clinically defined, prospectively studied, patients using a magnetic resonance image (MRI) rating scale, to (1) determine whether imaging findings warrant a more prominent position in FTD diagnosis and (2) correlate the extent of lobar atrophy with clinical data. i Methods: /i We adapted a recently devised post mortem rating scale for FTD to rate lobar atrophy on MRI scans. The areas rated included the frontal cortex and both anterior and posterior temporal regions bilaterally. All available brain scans from all patients seen in the Cambridge Dementia Clinic (n = 258) diagnosed as having FTD, together with controls (n = 20), were used to assess the reliability of the method. A subset of these (n = 121) were used for clinico-anatomic analysis. i Results: /i The scale proved quick and reliable (intra-, inter-rater k = 0.80, 0.67). MRI scans were abnormal in the majority of patients (75%), with focal atrophy present in 100% of semantic dementia (SD) patients. By contrast, nearly half (47%) of the patients with clinical behavioural variant FTD had scans within the normal range. Behavioural cases with normal scans generally had fewer cognitive deficits and milder functional impairment than those with abnormal scans, yet displayed a clinically indistinguishable behavioural syndrome. They were not, however, simply at an earlier stage of the disease. i Conclusions: /i MRI findings should form part of the diagnostic criteria for SD the absence of atrophy on MRI in many behavioural cases raises the prospect that the behavioural syndrome of FTD is not specific for patients with a neurodegenerative disease.
Publisher: BMJ
Date: 03-2005
Publisher: American Medical Association (AMA)
Date: 2013
DOI: 10.1001/2013.JAMANEUROL.382
Abstract: BACKGROUND Latrepirdine is an orally administered experimental small molecule that was initially developed as an antihistamine and subsequently was shown to stabilize mitochondrial membranes and function, which might be impaired in Huntington disease. OBJECTIVE To determine the effect of latrepirdine on cognition and global function in patients with mild to moderate Huntington disease. DESIGN Randomized, double-blind, placebo-controlled study. SETTING Sixty-four research centers in Australia, Europe, and North America. PATIENTS Four hundred three patients with mild to moderate Huntington disease and baseline cognitive impairment (Mini-Mental State Examination score, 10-26). INTERVENTION Latrepirdine (20 mg) vs matching placebo administered orally 3 times daily for 26 weeks. MAIN OUTCOME MEASURES The co-primary outcome measures were cognition as measured by the change in Mini-Mental State Examination score from baseline to week 26 and global function at week 26 as measured by the Clinician Interview-Based Impression of Change, plus carer interview, which ranges from 1 (marked improvement) to 7 (marked worsening). Secondary efficacy outcome measures included behavior, daily function, motor function, and safety. RESULTS The mean change in Mini-Mental State Examination score among participants randomized to latrepirdine (1.5-point improvement) did not differ significantly from that among participants randomized to placebo (1.3-point improvement) (P=.39). Similarly, the distribution of the Clinician Interview-Based Impression of Change, plus carer interview did not differ significantly among those randomized to latrepirdine compared with placebo (P=.84). No significant treatment effects were detected on the secondary efficacy outcome measures. The incidence of adverse events was similar between those randomized to latrepirdine (68.5%) and placebo (68.0%). CONCLUSION In patients with mild to moderate Huntington disease and cognitive impairment, treatment with latrepirdine for 6 months was safe and well tolerated but did not improve cognition or global function relative to placebo. TRIAL REGISTRATION clinicaltrials.gov Identifier: NCT00920946.
Publisher: Bentham Science Publishers Ltd.
Date: 05-2011
DOI: 10.2174/156720511795563818
Abstract: We review the practical importance of lobar atrophy in frontotemporal dementia (FTD), for diagnosis and prognosis. We discuss specific patterns of frontotemporal atrophy that denote clinical and pathological subtypes of FTD (e.g. semantic dementia). We also discuss the unsatisfactory clinical experience of interpreting MRI scans in in idual FTD cases, especially the behavioural presentations (without aphasic or motor impairments). This issue is explored by examining the FTD phenocopy concept. Lobar atrophy emerges as a key observation in defining behavioural FTD patients whose symptoms are likely to progress. In a situation where objective clinical data are few, we highlight the importance of applying caution before diagnosing FTD is the absence of visible brain atrophy.
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 12-2010
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 23-02-2009
Publisher: American Medical Association (AMA)
Date: 11-2006
DOI: 10.1001/ARCHNEUR.63.11.1627
Abstract: To assess the clinical course and prognosis in patients with behavioral-variant frontotemporal dementia (FTD) lacking evidence of brain atrophy on magnetic resonance imaging (MRI). Patients were enrolled into this prospective cohort study over a period of 15 years cognitive status, duration of symptoms, and behavioral indexes were recorded. Brain MRIs were rated using a standardized scale. Regional early-onset dementia clinic. Thirty-one participants diagnosed clinically with behavioral-variant FTD. Intervention Rating of MRIs. Death or institutionalization after a minimum of 3 years' follow-up indicated poor prognosis, while the ability to live independently was regarded as a good prognosis for the purpose of survival (Kaplan-Meier) and discriminant function analysis. Patients with normal or borderline MRI findings (n = 15) showed significantly longer survival to institutionalization or death than those (n = 16) with definite frontotemporal atrophy (mean +/- SE, 9.3 +/- 1.7 years vs 3.0 +/- 0.7 years P<.01). Using groups defined by 3-year outcome (good or bad prognosis), cerebral atrophy predicted poor outcome while age, symptom duration, cognitive performance, behavioral impairment, and overall disability at baseline did not. Patients with FTD with normal MRI results follow a more benign course than cases with atrophy at presentation. The substrate of the behavioral symptoms in such cases may differ from the neurodegenerative pathological features typically associated with FTD.
Publisher: Cambridge University Press (CUP)
Date: 09-2009
DOI: 10.1017/S1355617709990397
Abstract: Neuropsychological data on an extended series of cases of variant Creutzfeldt-Jakob Disease (vCJD) are presented, complementing earlier findings from smaller s le studies of this condition. Distinct neuropsychological features in this extended series included relatively preserved verbal knowledge, immediate verbal memory span, and elementary visual processing. This sparing contrasted with ubiquitous impairment in every vCJD patient on timed tests of verbal fluency and digit-symbol substitution. There were also high rates of impairment on tests of memory, and of visuoperceptual and visuospatial reasoning. Our findings lend support to the view that distinctive neuropsychological features may be one of the diagnostic markers of the condition. ( JINS , 2009, 15 , 807–810.)
Publisher: Oxford University Press (OUP)
Date: 21-01-2009
DOI: 10.1093/BRAIN/AWN314
Abstract: Social interaction is profoundly affected in the behavioural form of frontotemporal dementia (bvFTD) yet there are few means of objectively assessing this. Diagnosis of bvFTD is based on informant report, however a number of in iduals with a clinical profile consistent with the disease have no imaging abnormality and seem to remain stable, with doubt about the presence of underlying neurodegenerative pathology. We aimed to quantify aspects of the behavioural disorder and link it to the underlying level of atrophy in socially relevant brain regions. We tested in iduals with either bvFTD (N = 26) or Alzheimer's disease (N = 9) and 16 controls using The Awareness of Social Inference Test (TASIT) to assess their ability to identify emotion and sarcasm in video vignettes. A subset of bvFTD patients (N = 21) and controls (N = 12) were scanned using MRI within 6 months of assessment. There was marked impairment in the ability of bvFTD patients whose scans showed abnormalities to recognize sarcastic, but not sincere statements. Their capacity to interpret negative emotion was also impaired, and this appeared to be a major factor underlying the deficit in sarcasm recognition. Clinically diagnosed bvFTD patients whose scans were normal, Alzheimer's disease patients and controls had no difficulty in appreciating both types of statement. In a multivariate imaging analysis it was shown that the sarcasm (and emotion recognition) deficit was dependent on a circuit involving the lateral orbitofrontal cortex, insula, amygdala and temporal pole, particularly on the right. Performance on a more global test of cognitive function, the Addenbrooke's Cognitive Examination did not have a unique association with these regions. The TASIT is an objective test of social dysfunction in bvFTD which indexes the frontotemporal volume loss in bvFTD patients and provides an objective measure for separating behavioural patients who are likely to decline from those who may remain stable. These results provide additional evidence for the role of the orbitofrontal cortex and related structures in the processing of socially relevant signals, particularly those where negative emotion recognition is important.
Publisher: S. Karger AG
Date: 26-04-2018
DOI: 10.1159/000486479
Abstract: b i Background: /i /b Dementia caregivers frequently report high stress, with increased burden associated with worse outcomes for both patients and caregivers. Although many studies relate clinical phenotypes to burden, the relationship between imaging pathology and burden, irrespective of diagnosis, is unknown. This study investigated the relationship between caregiver burden and patient regional cerebral blood flow in dementia. b i Methods: /i /b Seventy-sev en patients with cognitive impairment undergoing brain perfusion single-photon emission computed tomography imaging in normal clinical care and their caregivers were recruited. Caregiver burden was ranked from “little” to “severe” using the Zarit Burden Interview and perfusion values extracted from the patient images for predefined regions of interest. The associations between burden score and regional function on imaging were tested. b i Results: /i /b Burden score was significantly higher for caregivers of patients with abnormal perfusion compared to those with normal perfusion in the left and right frontal, right parietal, and right temporal lobes. No difference in burden was found in the left parietal or temporal groups. Correlations showed that a higher caregiver burden was associated with lower patient perfusion scores in the same regions. b i Conclusion: /i /b Caregiver burden is strongly related to the extent of frontal or right-predominant parietal or temporal lobe dysfunction. Regional abnormality on perfusion imaging can be used to facilitate identification of in iduals who are likely to create a high burden on caregivers.
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 12-2017
Publisher: Oxford University Press (OUP)
Date: 04-05-2009
DOI: 10.1093/BRAIN/AWP077
Abstract: In patients with the behavioural variant of frontotemporal dementia, prognosis is often surprisingly good when there is normal structural imaging at presentation. Imaging abnormalities are not, however, mandatory for diagnosis, which in the absence of suitable biomarkers, remains entirely clinical. We aimed to test whether cases with normal structural imaging have hypometabolism suggestive of underlying neurodegeneration, or whether it is likely that such patients are false positive diagnoses of behavioural variant frontotemporal dementia. Patients with this disease (n = 24) and age-matched controls (n = 12) underwent both magnetic resonance imaging (MRI) and quantitative fluorodeoxyglucose-positron emission tomography (FDG-PET) scanning, together with clinical and behavioural assessments. Regions of interest were used to calculate metabolic rate in frontotemporal and control regions. Using a semi-quantitative visual rating scale, patients were ided into MRI-abnormal (n = 15) and MRI-normal groups (n = 9). There was definite frontotemporal hypometabolism in the MRI-abnormal group (particularly in the mesial and orbitofrontal regions) even after accounting for brain volume loss, whereas the MRI-normal group was similar to controls in all regions. In contrast, cognitive and behavioural indices did not separate the two behavioural variant frontotemporal dementia patient groups. The results suggest that the clinical syndrome of the behavioural variant of frontotemporal dementia may not be specific for a neurodegenerative disease, and we hypothesize the existence of a phenocopy. A number of alternative neuropsychiatric and developmental explanations are discussed. We advise caution in diagnosing the illness in patients without imaging abnormalities, and propose that imaging findings are included in criteria for diagnosis.
Publisher: Oxford University Press (OUP)
Date: 02-08-2011
DOI: 10.1093/BRAIN/AWR179
Publisher: Springer Science and Business Media LLC
Date: 02-10-2018
DOI: 10.1038/S41380-018-0224-0
Abstract: Next-generation genetic sequencing (NGS) technologies facilitate the screening of multiple genes linked to neurodegenerative dementia, but there are few reports about their use in clinical practice. Which patients would most profit from testing, and information on the likelihood of discovery of a causal variant in a clinical syndrome, are conspicuously absent from the literature, mostly for a lack of large-scale studies. We applied a validated NGS dementia panel to 3241 patients with dementia and healthy aged controls 13,152 variants were classified by likelihood of pathogenicity. We identified 354 deleterious variants (DV, 12.6% of patients) 39 were novel DVs. Age at clinical onset, clinical syndrome and family history each strongly predict the likelihood of finding a DV, but healthcare setting and gender did not. DVs were frequently found in genes not usually associated with the clinical syndrome. Patients recruited from primary referral centres were compared with those seen at higher-level research centres and a national clinical neurogenetic laboratory rates of discovery were comparable, making selection bias unlikely and the results generalisable to clinical practice. We estimated penetrance of DVs using large-scale online genomic population databases and found 71 with evidence of reduced penetrance. Two DVs in the same patient were found more frequently than expected. These data should provide a basis for more informed counselling and clinical decision making.
Publisher: JMIR Publications Inc.
Date: 11-07-2021
Abstract: he use of digital health technology to promote and deliver post-diagnostic care in neurological conditions is becoming increasingly common. However, the range of digital tools available across different neurological conditions and how they facilitate self-management is unclear. e aimed to identify digital tools that promote self-management in neurological conditions, and to investigate their underlying functionality and salient clinical outcomes. e conducted a search of six databases (i.e. CINAHL, EMBASE, MEDLINE, PsycINFO, Web of Science, and the Cochrane Review) using free text and equivalent database-controlled vocabulary terms. e identified 27 published articles reporting 17 self-management digital tools. Multiple sclerosis had the highest number of digital tools followed by epilepsy, stroke, and headache and migraine with a similar number, and then pain. The majority were aimed at patients with a minority for carers. There were five broad categories of functionality promoting self-management: 1) Knowledge and understanding 2) Behaviour modification 3) Self-management support 4) Facilitating communication and 5) Recording condition characteristics. Salient clinical outcomes included improvements in self-management, self-efficacy, coping, depression, and fatigue. here now exist numerous digital tools to support user self-management, yet relatively few are described in the literature. More research is needed to investigate their use, effectiveness and sustainability, and how this interacts with increasing disability, and their integration within formal neurological care environments. /A
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 2009
DOI: 10.1097/WAD.0B013E318182D293
Abstract: Patients with the behavioral variant of frontotemporal dementia have marked impairment in everyday life, yet little is known about factors underlying this impairment. Moreover, a recently identified subgroup with normal brain imaging has an excellent prognosis (phenocopy cases) and their performance on activities of daily living (ADL) tasks is unknown. Eighteen behavioral variant frontotemporal dementia patients were assessed on 2 ADL measures, the Disability Assessment for Dementia, a caregiver-based interview, and the Assessment of Motor and Process Skills, a performance-based instrument. Behavior change, global cognition, executive function, and magnetic resonance imaging brain atrophy were also evaluated. There was no association between the 2 ADL measures. A model combining the Addenbrooke's Cognitive Examination Revised (global cognition) and Frontal Systems Behavior Scale (frontal dysfunction) explained the variance on ADL performance. A qualitative rating distinguished between pathologic and phenocopy patients better than the performance-based assessment. Degree of frontal dysfunction and overall dementia determined the level of ADL impairment. The phenocopy patients were clearly distinguishable when evaluated using a performance-based, and even better with a qualitative rating assessment.
Publisher: Wiley
Date: 15-08-2009
DOI: 10.1002/MDS.22558
Abstract: Corticobasal syndrome (CBS) has been associated with a heterogeneous spectrum of pathologies with an increasing number of reports of Alzheimer's type pathology. There is, however, no means of predicting pathology of CBS in vivo at present. We compared the clinical features of patients presenting with CBS who have either pathologic changes of classic corticobasal degeneration (CBD) or Alzheimer's disease (AD) at post-mortem to identify predictors of the specific pathological processes in life. Twelve patients with CBS were followed prospectively six had AD and six had classic CBD neuropathology. After review of the presenting clinical features, we identified nine potential predictor variables, compared their frequency in the two groups, and performed a discriminant function analysis. Initial episodic memory complaints and poor performance on the combined orientation-memory subtest of the Addenbrooke's Cognitive Examination (ACE) reliably predicted AD pathology while varying combinations of early frontal-lobe type behavioral symptoms, nonfluent language disturbance, orobuccal apraxia, and utilization behavior predicted CBD pathology ante-mortem. CBS is frequently associated with Alzheimer's disease pathology. Early episodic memory impairment versus early behavioral symptomatology appears to best predict AD or CBD pathology in life.
Publisher: Informa UK Limited
Date: 09-2006
DOI: 10.1080/17470910600989847
Abstract: Patients with frontotemporal dementia (FTD) exhibit marked changes in social and emotional functioning including lack of empathy, disinhibition, altered emotional reactivity, apathy and lack of insight. These changes are believed to be dependent on progressive frontal and temporal lobe degeneration. In this review, we discuss the nature of defective theory of mind and empathy in this group and relate it to regional dysfunction in the orbitofrontal and medial prefrontal cortex, based on evidence from several recent studies. The role of executive ability and co-existing emotional deficits are also considered.
Publisher: Elsevier BV
Date: 2006
DOI: 10.1016/J.NEUROPSYCHOLOGIA.2005.08.009
Abstract: Social cognition is crucial for human interaction, and is markedly impaired in the frontal variant of frontotemporal dementia (fvFTD). The relationship of various aspects of social functioning, however, remains controversial in this group. Patients with fvFTD (n = 18), and matched controls (n = 13), were tested using tasks designed to assess their Theory of Mind (ToM), moral reasoning, emotion recognition and executive function. Caregivers documented changes in empathy compared to premorbid functioning. We found marked impairments in the abilities of fvFTD patients, relative to controls, in ability to mentalise (ToM), which was evident on a cartoon test, but not on a story-based ToM task. Knowledge of social rules was intact, but moral reasoning was defective, and was due, in part, to an inability to rate the seriousness of moral and conventional transgressions appropriately. Executive function was impaired in this group, and compromised aspects of moral reasoning, but ToM performance was independent of this. Emotion recognition was globally impaired in fvFTD, but was particularly so for anger and disgust which may partly explain the difficulty these patients have with identifying social violations. Empathy, as rated by carers, was also shown to be abnormal. It appears that social reasoning is disrupted in a number of ways in fvFTD, and the findings provide a basis for the understanding and further study of abnormal behaviour in this disease. The results are discussed in light of neuroimaging findings in studies of social cognition and the locus of pathology in fvFTD.
Publisher: SAGE Publications
Date: 10-08-2016
Abstract: Research is beginning to demonstrate the unique psychosocial effects of young onset dementia. Theorising remains at an early stage and there has been little discussion about measurement and methodological issues. Our aim was to conduct a comprehensive literature search of the young onset dementia psychosocial research, and to identify the domains of experience measured with patients and caregivers. We conducted a search of five electronic databases (Medline, CINAHL, PsycINFO, Embase, the Cochrane Library) using equivalent database controlled vocabulary terms. We supplemented this search by using free text searches within electronic databases, searching reference sections of salient papers, and using online search engines. We defined psychosocial as referring to patient and caregiver psychological, behavioural, and social functioning in the context of living with young onset dementia. We identified 72 published articles, 49 quantitative and 23 qualitative. The quantitative articles form the focus of the present review. We identified 10 domains of patient experience measured and 14 domains of caregiver experience. The patient domains measured most often were behaviour, cognition, functioning, and severity, and reflected a focus on symptoms and clinical features. Quality of Life (QoL) was the patient domain measured least often. The caregiver domains measured most often were mental health and burden, and reflected a focus on psychological well-being and coping. The scope of measurement is broader in caregivers than patients. QoL although under-researched may be a useful domain to measure in future research. Risk factors, measurement and methodological issues are discussed.
Publisher: Springer Science and Business Media LLC
Date: 15-08-2010
DOI: 10.1007/S00406-010-0133-Y
Abstract: The negative symptoms of schizophrenia have been considered to be a psychiatric form of the frontal lobe syndrome. However, no studies have compared these two disorders at the clinical level. In this study, 12 negative symptom schizophrenic patients and 11 patients with behavioural variant frontotemporal dementia (bv-FTD) were rated for negative symptoms and for occurrence of frontal lobe behaviours in everyday life. They were also rated for speech disorder and were given a series of executive tests. Both patient groups showed positive ratings on negative symptoms and frontal lobe behaviours in daily life however, the schizophrenic patients had higher negative symptom scores and the bv-FTD patients had higher carer ratings on frontal behaviours in daily life. Both groups were impaired on the executive tests, but the bv-FTD patients showed significantly greater impairment on verbal fluency and a test requiring inhibition of prepotent responses. A minority of the bv-FTD patients unexpectedly showed speech abnormalities typically associated with schizophrenia. The findings indicate that the negative syndrome in schizophrenia and the frontal lobe syndrome resemble each other clinically in important respects. Some of the differences may be attributable to the additional presence of disinhibition in the frontal lobe syndrome.
Publisher: Wiley
Date: 05-02-2009
DOI: 10.1111/J.1468-1331.2008.02414.X
Abstract: A study of the pattern of Sleep/Wake disturbance in frontotemporal dementia (FTD). Sleep diaries and prolonged actigraphy were used to record the activity, sleep and wake of 13 patients with a clinical diagnosis of FTD. These were compared with diaries and actigraphy from normal age/sex matched controls and also to a population with probable Alzheimer's disease (AD). There was significant sleep/wake disturbance in FTD. This occurred throughout the course of the illness and the nature of the sleep disturbance was different to patients with AD. FTD subjects showed increased nocturnal activity and decreased morning activity compared with controls, suggesting possible phase delay. Sleep diary data confirmed decreased sleep efficiency and decreased total sleep in all FTD patients. We describe significant sleep disturbance in non-institutionalized patients with FTD and suggest that early sleep disturbance may help differentiate between FTD and AD.
Publisher: JMIR Publications Inc.
Date: 28-07-2022
DOI: 10.2196/31929
Abstract: The use of digital health technology to promote and deliver postdiagnostic care in neurological conditions is becoming increasingly common. However, the range of digital tools available across different neurological conditions and how they facilitate self-management are unclear. This review aims to identify digital tools that promote self-management in neurological conditions and to investigate their underlying functionality and salient clinical outcomes. We conducted a search of 6 databases (ie, CINAHL, EMBASE, MEDLINE, PsycINFO, Web of Science, and the Cochrane Review) using free text and equivalent database-controlled vocabulary terms. We identified 27 published articles reporting 17 self-management digital tools. Multiple sclerosis (MS) had the highest number of digital tools followed by epilepsy, stroke, and headache and migraine with a similar number, and then pain. The majority were aimed at patients with a minority for carers. There were 5 broad categories of functionality promoting self-management: (1) knowledge and understanding (2) behavior modification (3) self-management support (4) facilitating communication and (5) recording condition characteristics. Salient clinical outcomes included improvements in self-management, self-efficacy, coping, depression, and fatigue. There now exist numerous digital tools to support user self-management, yet relatively few are described in the literature. More research is needed to investigate their use, effectiveness, and sustainability, as well as how this interacts with increasing disability, and their integration within formal neurological care environments.
Publisher: SAGE Publications
Date: 23-01-2017
Abstract: The qualitative research on young onset dementia is providing insights about the 'lived experience' of patients and caregivers. However, findings from these studies have seldom been integrated into descriptive overviews. Our aim was to search the qualitative research, to integrate the qualitative findings, and offer an account of the lived experience for patients and caregivers. The search of the qualitative research formed part of a broader comprehensive literature search investigating salient measurement issues in the young onset dementia psychosocial research. Five electronic databases were searched (Medline, CINAHL, PsycINFO, Embase, the Cochrane Library) plus supplementary searching of reference sections and use of online search engines. We identified 23 qualitative articles. In the pre-diagnostic period, patients experience changes in cognition and functioning but may be uncertain about changes and their significance. Caregivers may observe changes, think of explanations, and are important in instigating medical intervention. Obtaining a diagnosis may be a demanding process and the possibility of dementia may not be anticipated. In the post-diagnostic period, patients and caregivers use several strategies to cope and adjust. Patients can withdraw from established responsibilities while caregivers assume compensatory roles/duties. Patients perceive changes in their identity while caregivers perceive changes in the caregiver-patient relationship. Both can experience grief, isolation, and stigma. The diagnosis of dementia elicits significant changes in thinking, emotion, and lifestyle that patients and caregivers are unlikely to be ready for. Both receive insufficient support or guidance in particular of a psychological nature on how to cope and adjust.
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 11-06-2007
DOI: 10.1212/01.WNL.0000264897.13722.53
Abstract: Objective: To evaluate activities of daily living (ADLs) in three clinical variants of frontotemporal dementia and the relationship to cognitive dysfunction. Methods: Fifty-nine patients and caregivers participated in this cross-sectional study: behavioral variant frontotemporal dementia (bv-FTD, n = 15), progressive nonfluent aphasia (PNFA, n = 10), semantic dementia (n = 15), and Alzheimer disease (AD, n = 19). Caregivers were interviewed with the Disability Assessment for Dementia (DAD) to provide two outcome measures about ADLs: basic and instrumental ADLs (BADLs, IADL). In addition, patients were rated on the Clinical Dementia Rating Scale (CDR), and performance on cognitive measures (Addenbrooke's Cognitive Examination Revised [ACE-R]) was assessed. Results: On the DAD, the bv-FTD group was most affected (56% of normal), whereas PNFA and semantic dementia patients were least impaired (83% and 85%) AD was intermediate (76%). The opposite pattern was seen on the ACE-R, where PNFA and semantic dementia groups were most affected, and bv-FTD showed least impairment AD was again intermediate. Scores on the DAD did not correlate with cognitive measures, CDR, or disease duration. We further analyzed which aspect of ADLs was most affected, and a unique pattern of deficits emerged for the bv-FTD group (initiation affected planning execution for BADLs). Conclusion: Frontotemporal dementia has a devastating effect on activities of daily living, which is of considerable importance to caregivers and not captured by bedside cognitive tests.
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 03-11-2008
DOI: 10.1212/01.WNL.0000334299.72023.C8
Abstract: Recent studies suggest that behavioral variant frontotemporal dementia (bv-FTD) patients differ in their prognosis with fast-progressing and very slow-progressing cases. We investigated executive and behavioral profiles of progressive and nonprogressive bv-FTD patients to establish diagnostic markers discriminating the two groups. A range of neuropsychological and behavioral tests were used. Mean overlap-based statistical analyses and logistic regression analyses were performed to distinguish progressive from nonprogressive bv-FTD cases. Although progressors and nonprogressors showed similar behavioral profiles, they were distinguishable by their performance on executive tasks. The nonprogressors' performance on all tests was with the normal range, whereas the progressors were consistently impaired on four tests: Digit Span Backward, Hayling Test of inhibitory control, Letter Fluency, and Trails B. Logistic regression showed that 86% of patients could be classified on the basis of Digit Span and Hayling subscores. Contrary to some prior reports, behavioral variant frontotemporal dementia (bv-FTD) patients who progress over time are typically impaired on executive tasks at first presentation, although an important minority of true FTD patients perform normally. Previous inconsistencies are explicable by the mixture of patients with progressing FTD and phenocopy cases.
Publisher: Future Medicine Ltd
Date: 02-2018
Abstract: Aim: Psychosocial research on the lived experiences of young-onset dementia patients and caregivers has identified salient issues about their care, however, views on care from the perspective of young-onset dementia healthcare professionals is less well known. The aim of this study was to investigate and identify important issues in young-onset dementia care provision from a healthcare provider perspective. Methods: The design was an exploratory qualitative interview study. In-depth semistructured interviews were conducted with healthcare professionals with clinical expertise in young-onset dementia drawn from medicine, nursing and allied health. Thematic analysis was applied to interview transcripts to identify themes representing important underlying issues in care across the dementia clinical pathway (i.e., prediagnosis, diagnosis and postdiagnosis). Results: In prediagnosis, it is important for healthcare professionals to recognize symptoms as organic and degenerative and more than psychological, and to refer patients to an appropriate clinical facility for assessment. During diagnosis, it may be challenging to determine dementia, and methods are employed to manage diagnostic uncertainty. Following diagnosis, optimizing routine clinical care is important and can include the provision of practical informational guidance, empathic concern and psychoeducational support. Meeting service-user requirements in the community is an important aspect of care, and may be facilitated by the involvement of clinical nurse specialists. Conclusion: The findings are presented as a paradigm for holistic young-onset dementia care. The paradigm offers a framework for contemplating and evaluating the criteria and quality of young-onset dementia care.
Publisher: MIT Press - Journals
Date: 07-2007
DOI: 10.1162/JOCN.2007.19.7.1206
Abstract: Patients with Huntington's disease (HD) can show disproportionate impairments in recognizing facial signals of disgust, but the neural basis of this deficit remains unclear. Functional imaging studies have implicated the anterior insula in the ability to recognize disgust, but have identified other structures as well, including the basal ganglia. In view of variable insula and basal ganglia volume changes in HD, we used voxel-based morphometry to map regional variations in gray matter (GM) volume in participants carrying the mutation for HD, and correlated this with their performance on a test of facial emotion recognition for six basic emotions (disgust, fear, anger, happiness, sadness, surprise). The volume of the anteroventral insula was strongly correlated with performance on the disgust recognition task. The amygdala volume (bilaterally) correlated with the ability to recognize happy facial expressions. There was marked specificity of the regional correlations for the emotion involved. Recognition of other emotion expressions, or more general cognitive or motor performance as measured by a standardized rating scale, did not correlate with regional brain volume in this group. Control participants showed no effect for any measure. The strong linear correlations for disgust and happiness recognition imply direct involvement of the anterior insula in disgust appreciation, and a similar role for the amygdala in recognizing happy facial expressions. The absence of a significant correlation with the basal ganglia suggests a less critical role for these structures in disgust recognition than has previously been suggested. The findings also highlight the role of neurodegenerative diseases combined with statistical imaging techniques in elucidating the brain basis of behavior and cognition.
Publisher: BMJ
Date: 18-02-2009
Abstract: Recent findings suggest that patients with behavioural variant frontotemporal dementia (bv-FTD) differ in their disease progression (progressive vs non-progressive patients). The current study investigates whether the two groups can be discriminated by their clinical features at first presentation. Archival clinical data of the Early Onset Dementia Clinic, Cambridge, UK, were analysed for 71 patients with bv-FTD: 45 progressive and 26 non-progressive cases with more than 3 years of follow-up. The subgroups were largely indistinguishable on the basis of the presenting clinical features but could be distinguished on general cognitive (Addenbrooke's Cognitive Examination-revised) and selected supportive diagnostic features (distractibility, stereotypic speech, impaired activities of daily living (ADLs) and current depression). Progressive and non-progressive patients are difficult to differentiate on the basis of current clinical diagnostic criteria for FTD but a combination of general cognitive, executive dysfunction and impaired ADL measures appear to be the most promising discriminators.
Publisher: Elsevier BV
Date: 09-2011
DOI: 10.1016/J.EJPN.2011.04.005
Abstract: The literature on paediatric acute-onset movement disorders is scattered. In a prospective cohort of 52 children (21 male age range 2mo-15y), the commonest were chorea, dystonia, tremor, myoclonus, and Parkinsonism in descending order of frequency. In this series of mainly previously well children with cryptogenic acute movement disorders, three groups were recognised: (1) Psychogenic disorders (n = 12), typically >10 years of age, more likely to be female and to have tremor and myoclonus (2) Inflammatory or autoimmune disorders (n = 22), including N-methyl-d-aspartate receptor encephalitis, opsoclonus-myoclonus, Sydenham chorea, systemic lupus erythematosus, acute necrotizing encephalopathy (which may be autosomal dominant), and other encephalitides and (3) Non-inflammatory disorders (n = 18), including drug-induced movement disorder, post-pump chorea, metabolic, e.g. glutaric aciduria, and vascular disease, e.g. moyamoya. Other important non-inflammatory movement disorders, typically seen in symptomatic children with underlying aetiologies such as trauma, severe cerebral palsy, epileptic encephalopathy, Down syndrome and Rett syndrome, include dystonic posturing secondary to gastro-oesophageal reflux (Sandifer syndrome) and Paroxysmal Autonomic Instability with Dystonia (PAID) or autonomic 'storming'. Status dystonicus may present in children with known extrapyramidal disorders, such as cerebral palsy or during changes in management e.g. introduction or withdrawal of neuroleptic drugs or failure of intrathecal baclofen infusion the main risk in terms of mortality is renal failure from rhabdomyolysis. Although the evidence base is weak, as many of the inflammatory/autoimmune conditions are treatable with steroids, immunoglobulin, plasmapheresis, or cyclophosphamide, it is important to make an early diagnosis where possible. Outcome in survivors is variable. Using illustrative case histories, this review draws attention to the practical difficulties in diagnosis and management of this important group of patients.
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 27-05-2008
DOI: 10.1212/01.WNL.0000313366.76973.8A
Abstract: Background: There is a need for instruments which can measure progression of disease in frontotemporal dementia (FTD), particularly with respect to the assessment of potential therapeutic agents. Methods: The Cambridge Early Onset Dementia Clinic database was reviewed for all prospectively enrolled cases of FTD with documented scores on the Mini-Mental State Examination (MMSE) or Addenbrooke’s Cognitive Examination (ACE) on at least two occasions. We identified 50 cases fulfilling these criteria: pathologic confirmation was present in 11 of 16 patients who had died, 12 of the remainder had imaging abnormalities on their initial scans, and 22 had structural scans no different from controls. We compared these groups to a cohort with early AD (n = 25) and healthy controls (n = 10). Results: There was clear cognitive decline (measured by the MMSE and ACE) in patients who had died, and those with documented atrophy on initial MRI scan. In contrast, patients with FTD with normal scans showed no change in cognitive scores over a much longer interval, and serial ACE measurements paralleled those of controls. Power calculations showed that the inclusion of these patients with FTD would significantly increase the number of cases needed in any therapeutic trial. Conclusion: Addenbrooke’s Cognitive Examination is a simple monitoring tool which can detect progression of disease in frontotemporal dementia over a 1- to 2-year interval without the need for serial imaging. We estimated that a clinical trial that enrolled subjects with abnormal MR scans would require 135 subjects per group to detect a small effect, and 35 for a medium effect.
Publisher: Ovid Technologies (Wolters Kluwer Health)
Date: 10-2007
Publisher: Elsevier BV
Date: 08-2018
DOI: 10.1016/J.MSARD.2018.05.015
Abstract: The UK Multiple Sclerosis Register (UKMSR) is a large cohort study designed to capture 'real world' information about living with multiple sclerosis (MS) in the UK from erse sources. The primary source of data is directly from people with Multiple Sclerosis (pwMS) captured by longitudinal questionnaires via an internet portal. This population's diagnosis of MS is self-reported and therefore unverified. The second data source is clinical data which is captured from MS Specialist Treatment centres across the UK. This includes a clinically confirmed diagnosis of MS (by Macdonald criteria) for consented patients. A proportion of the internet population have also been consented at their hospital making comparisons possible. This dataset is called the 'linked dataset'. The purpose of this paper is to examine the characteristics of the three datasets: the self-reported portal data, clinical data and linked data, in order to assess the validity of the self-reported portal data. The internet (n = 11,021) and clinical (n = 3,003) populations were studied for key shared characteristics. We found them to be closely matched for mean age at diagnosis (clinical = 37.39, portal = 39.28) and gender ratio (female %, portal = 73.1, clinical = 75.2). The Two S le Kolmogorov-Smirnov test was for the continuous variables to examine is they were drawn from the same distribution. The null hypothesis was rejected only for age at diagnosis (D = 0.078, p < 0.01). The populations therefore, were drawn from different distributions, as there are more patients with relapsing disease in the clinical cohort. In all other analyses performed, the populations were shown to be drawn from the same distribution. Our analysis has shown that the UKMSR portal population is highly analogous to the entirely clinical (validated) population. This supports the validity of the self-reported diagnosis and therefore that the portal population can be utilised as a viable and valid cohort of people with Multiple Sclerosis for study.
Publisher: Informa UK Limited
Date: 19-11-2007
DOI: 10.1080/13554790701594870
Abstract: The diagnosis of the behavioural variant of frontotemporal dementia (bvFTD) can be challenging. At present there is a paucity of prospective work addressing the specificity of current diagnostic criteria for bvFTD with respect to long-term outcome (i.e., false positives versus true positives). Here we report two in iduals who met current clinical criteria for bvFTD and who underwent detailed long-term clinical and neuropsychological follow-up. In addition, both had serial volumetric MRI and functional metabolic (FDG-PET) imaging separated by 5 years. One case had a slow clinical decline as well as both progressive atrophy and hypometabolism in a frontotemporal distribution, consistent with a neurodegenerative FTD syndrome. However, the second developed neither atrophy nor hypometabolism and remained clinically stable, a decade from symptom onset. We propose that these cases illustrate that while there may be a slow evolution in bvFTD, it is possible that some cases who meet current criteria may not have a neurodegenerative syndrome. If correct, this hypothesis has important implications for the current diagnostic criteria. A potential hierarchy for diagnostic certainty in bvFTD is suggested.
Publisher: Elsevier BV
Date: 2007
DOI: 10.1016/J.NEUROPSYCHOLOGIA.2006.05.031
Abstract: Structural brain imaging and neuropsychological data implicate the orbital aspects of prefrontal cortex in the developing neuropathology of fvFTD. Damage to this region is associated with deficient performance on laboratory tasks assessing theory of mind (ToM) and affective decision-making (DM), but the relationship between these two capacities in patients with prefrontal cortex dysfunction is unclear. We studied a group of patients with early/mild fvFTD (n=20) and a group of matched normal controls (n=10) on the Iowa gambling task (IGT) of affective decision-making, and the "reading the mind in the eyes" (MIE) and "faux pas" (FP) tests of ToM. The fvFTD group was impaired in both ToM tasks and the IGT. While performance measures from the two ToM tasks were significantly correlated, they were not associated with IGT performance. This suggests that whilst similar prefrontal circuitry is implicated in ToM and DM tasks, these cognitive domains may be independent. In clinical settings, the IGT may be useful as a complementary tool to the frontal test battery for patients with early/mild fvFTD. Deficits in decision-making and ToM observed in this study have distinct but additive effects upon the development of social behaviour in patients with prefrontal dysfunction.
Publisher: BMJ
Date: 05-2005
Publisher: Elsevier
Date: 2008
Location: United Kingdom of Great Britain and Northern Ireland
Location: United Kingdom of Great Britain and Northern Ireland
Location: United Kingdom of Great Britain and Northern Ireland
No related grants have been discovered for Christopher Kipps.