Publications by authors named "Rickards H"

Article Synopsis
  • Researchers developed a Huntington's disease (HD) progression model combined with an economic model to evaluate the societal costs of HD, using data from the Enroll-HD observational study.
  • They identified five distinct disease states through a continuous-time hidden Markov model and enhanced the life expectancy estimations with a new data correction method.
  • The simulations demonstrated that early cognitive decline is significant for HD assessment and highlighted that indirect costs, like state benefits and lost GDP, outweigh direct healthcare costs in the overall societal costs of HD.
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Background: Staff sickness absenteeism and presenteeism (attending work while unwell) incur high costs to the NHS, are associated with adverse patient outcomes and have been exacerbated by the COVID-19 pandemic. The main causes are mental and musculoskeletal ill health with cardiovascular risk factors common.

Objectives: To undertake a feasibility study to inform the design of a definitive randomised controlled trial of the effectiveness and cost effectiveness of a health screening clinic in reducing absenteeism and presenteeism amongst the National Health Service staff.

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Background: Huntington's disease (HD) is a progressive neurodegenerative disease with a devastating impact on patients and their families. Quantifying how treatments affect patient outcomes is critical for informing reimbursement decisions. Many countries mandate a formal value assessment in which the treatment benefit is measured as quality-adjusted life-years, calculated with the use of utility estimates that reflect respondents' preferences for health states.

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Background: Huntington's disease (HD) is a rare inherited neurodegenerative disorder characterized by complex evolving needs that change as the condition progresses. There is limited understanding about the organization of HD clinical services and their resourcing in the United Kingdom (UK).

Objective: To understand the organization and resourcing of specialist HD services for people with HD (PwHD) in the UKMethods:This cross-sectional study collected quantitative data via on online survey, and qualitative data via telephone semi-structured interviews.

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Introduction: Social cognition is problematic in Huntington's disease (HD). Despite the observations of clinicians and families, there is minimal empirical literature about how it presents in daily life and the impact on social functioning. This protocol forms the basis of a scoping review to synthesise both the quantitative knowledge and qualitative experiences of the HD community so that a visual and narrative map can address what is known and what is not known for the benefit of the community and clinicians and academics alike.

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Individuals with Huntington's disease (HD) and their close others report difficulties with social interaction, and previous studies have shown that the areas of quality of life detrimentally impacted by HD include social and emotional domains. However, despite the finding that people with HD often exhibit difficulties on standard tests of social cognition, the relationship between such impairments and patients' everyday life has remained largely unexplored. We used a range of tasks assessing empathy, emotion recognition and Theory of Mind, to investigate whether patients' performance may predict quality of life within the social and emotional domains, while also accounting for broader cognitive function, behavioural changes, motor symptoms, disease stage and functional capacity.

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Background: Staff absenteeism and presenteeism incur high costs to the NHS and are associated with adverse health outcomes. The main causes are musculoskeletal complaints and mental ill-health, which are potentially modifiable, and cardiovascular risk factors are also common. We will test the feasibility of an RCT to evaluate the clinical and cost-effectiveness of an employee health screening clinic on reducing sickness absenteeism and presenteeism.

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White matter (WM) alterations have been observed in Huntington disease (HD) but their role in the disease-pathophysiology remains unknown. We assessed WM changes in premanifest HD by exploiting ultra-strong-gradient magnetic resonance imaging (MRI). This allowed to separately quantify magnetization transfer ratio (MTR) and hindered and restricted diffusion-weighted signal fractions, and assess how they drove WM microstructure differences between patients and controls.

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Psychogenic nonepileptic seizures resemble epileptic seizures but lack the physiological basis of epileptic seizures. We conducted a systematic review to explore whether childhood abuse is a risk factor for subsequent development of PNES. We reviewed only papers with an epilepsy control group, which employed strict criteria for diagnosis of epilepsy and well-validated tools for assessing abuse history.

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Objective: The relationship between idiopathic and inherited (monogenic) forms of isolated and combined dystonia and psychiatric disorders remains unclear. In the present review, the authors aimed to provide increased clarity on this association through a systematic review of all controlled quantitative studies using a structured or semi-structured psychiatric interview to diagnose psychiatric disorders in individuals with these conditions.

Methods: Three databases were searched to identify 20 eligible studies, with a total of 1,275 participants fulfilling inclusion criteria.

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White matter (WM) alterations have been identified as a relevant pathological feature of Huntington's disease (HD). Increasing evidence suggests that WM changes in this disorder are due to alterations in myelin-associated biological processes. Multi-compartmental analysis of the complex gradient-echo MRI signal evolution in WM has been shown to quantify myelin in vivo, therefore pointing to the potential of this technique for the study of WM myelin changes in health and disease.

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Background: Huntington's disease (HD) is an inherited neurodegenerative disorder caused by an expanded CAG repeat in the HTT gene. It is diagnosed following a standardized examination of motor control and often presents with cognitive decline and psychiatric symptoms. Recent studies have detected genetic loci modifying the age at onset of motor symptoms in HD, but genetic factors influencing cognitive and psychiatric presentations are unknown.

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Background: Huntington's disease is an autosomal-dominant neurodegenerative disease caused by CAG trinucleotide repeat expansion in , resulting in a mutant huntingtin protein. IONIS-HTT (hereafter, HTT) is an antisense oligonucleotide designed to inhibit messenger RNA and thereby reduce concentrations of mutant huntingtin.

Methods: We conducted a randomized, double-blind, multiple-ascending-dose, phase 1-2a trial involving adults with early Huntington's disease.

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Article Synopsis
  • Huntington's disease (HD) onset is mainly linked to the length of CAG repeat expansion in the huntingtin gene, with other factors contributing to variability.
  • This study investigates the effect of arterial hypertension (AHT) on the age of onset of motor symptoms in HD patients using data from 630 European cases.
  • Findings suggest a significant association where patients with pre-HD AHT developed motor symptoms 5-8 years later than those without AHT, highlighting the potential neuroprotective role of antihypertensive treatment and the need for further research.
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Huntington's disease (HD) can impair social cognition. This study investigated whether patients with HD exhibit neural differences to healthy controls when they are considering mental and physical states relating to the static expressions of human eyes. Thirty-two patients with HD and 28 age-matched controls were scanned with fMRI during two versions of the Reading the Mind in the Eyes Task: The standard version requiring mental state judgments, and a comparison version requiring judgments about age.

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Unlabelled: Transcranial direct current stimulation (tDCS) combined with a cognitive task can enhance targeted aspects of cognitive functioning in clinical populations. The movement disorder Huntington's disease (HD) is associated with progressive cognitive impairment. Deficits in working memory (WM) can be apparent early in the disease and impact functional capacity.

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Changes in mental state and behaviour have been acknowledged in Huntington's disease since the original monograph in 1872 provided evidence of disinhibition and impaired social cognition. Behavioural problems can manifest before obvious motor symptoms and are frequently the most disabling part of the illness. Although pharmacological treatments are used routinely for psychiatric difficulties in Huntington's disease, the scientific evidence base for their use is somewhat sparse.

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Tourette syndrome (TS) is a neurodevelopmental disorder characterized by tics, repetitive movements and vocalizations which are prompted by a sensory-cognitive premonitory urge. Complex tics include environmentally dependent social behaviors such as echoing of other people's speech and actions. Recent studies have suggested that adults with TS can show differences to controls in Theory of Mind (ToM): reasoning about mental states (e.

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Introduction: This study aimed to evaluate the feasibility and benefit of a structured exercise intervention in people with Huntington's Disease (HD).

Methods: This study was conducted at 6 sites, and participants were randomized into either exercise or control (usual care) groups, and were assessed at baseline, 13 and 26 weeks. The intervention was a 12 week, three times per week progressive exercise program, including aerobic (stationary cycling) and upper and lower body strengthening exercise with tapered 1:1 support for 20 of 36 sessions.

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Article Synopsis
  • Tourette syndrome (TS) and chronic tic disorder (CTD) affect 1-2% of youth, but the best treatment methods remain uncertain, prompting a systematic review of available interventions.
  • A total of 40 trials were evaluated, showing that α2-adrenergic receptor agonists (like clonidine and guanfacine) and behavioral therapies (such as habit reversal training) offer moderate success in reducing tic severity.
  • The study concludes that α2-adrenergic receptor agonists should be the first choice for medication due to their balance of benefits and risks, while antipsychotic medications may be useful but should be reserved for cases where other treatments fail.
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This study explores the structural relationship between self-report and interview measures of affect in Huntington's disease. The findings suggest continued use of both to recognize the multidimensionality within a single common consideration of distress.

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Article Synopsis
  • Tourette syndrome (TS) affects around 1% of school-age children and is marked by chronic motor and vocal tics, which can lead to significant distress and impact social functioning.
  • This study aims to systematically review the effectiveness and risks of various treatments for tics in children and adolescents with TS, including both medication and behavioral therapies, while also gathering insights from young individuals with TS and their parents about their treatment experiences.
  • The methodology involved reviewing randomized controlled trials and qualitative studies, assessing the quality of evidence and potential biases, and engaging parents through a national survey to gather broader perspectives on treatment satisfaction and challenges.
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