Late onset AMACR deficiency with metabolic stroke-like episodes and seizures.

Alpha-methylacyl-CoA racemase (AMACR) deficiency is a rare peroxisomal disorder causing pristanic acid accumulation. Only 16 cases have been described so far. A female in her seventh decade presented with episodes of dysphasia, headache and sensory disturbance inconsistent with migraine, epilepsy or transient ischaemic attack.

Oxfordshire Community Stroke Project Classification: A proposed automated algorithm.

Introduction: The Oxfordshire Community Stroke Project (OCSP) proposed a clinical classification for Stroke patients. This classification has proved helpful to predict the risk of neurological complications. However, the OCSP was initially based on findings on the neurological assesment, which can pose difficulties for classifying patients.

Prevention of mood disorder after stroke: a randomised controlled trial of problem solving therapy versus volunteer support.

Background: Mood disorder after stroke is common but drug and psychosocial treatments have been assessed with disappointing results. Preventing mood disorder from developing in the first place could be a better approach and might reduce the need for pharmacotherapy in this predominantly older patient group. We used a brief problem-solving therapy and evaluated its effect in reducing mood disorder in the 12 months after stroke.

The design and validation of a parent-report questionnaire for assessing the characteristics and quality of early intervention over time.

This article concerns a parent-report repeat questionnaire to evaluate the quality of multiprofessional early intervention following early identification of deafness. It discusses the rationale for the design of the instrument, its theoretical underpinnings, its psychometric properties, and its usability. Results for the validity and reliability of the instrument are based on completion by 82 parents.

Elevated C-reactive protein and long-term mortality after ischaemic stroke: relationship with markers of endothelial cell and platelet activation.

Background And Purpose: Inflammatory biomarkers predict development of atherothrombotic events. In the present study we examined the relationships between C-reactive protein (CRP), complement C3, and long-term mortality after acute ischemic stroke.

Methods: CRP and C3 were analyzed by in-house enzyme-linked immunosorbent assay in 394 subjects with acute ischemic stroke who survived for >30 days, followed-up for a median of 7.

Acceptance, effectiveness and safety of continuous positive airway pressure in acute stroke: a pilot study.

Objectives: To evaluate the acceptance, effectiveness in preventing upper airways obstruction, and haemodynamic effects of continuous positive airway pressure (CPAP) in acute stroke.

Methods: Twelve patients (4 M, and 8 F; mean (SD), 75.2 (5.

Why don't health professionals check patient understanding? A questionnaire-based study.

Checking patient understanding, by asking questions about information presented in a medical consultation, is a core communication skill but its use is not frequently assessed. The newborn hearing screening commenced in England with a pilot of its implementation in January 2002. Because it is a new screening test, it provided an opportunity for studying the effective communication of novel information.

Predictive variables for mortality after acute ischemic stroke.

Background And Purpose: Stroke is a major healthcare issue worldwide with an incidence comparable to coronary events, highlighting the importance of understanding risk factors for stroke and subsequent mortality.

Methods: In the present study, we determined long-term (all-cause) mortality in 545 patients with ischemic stroke compared with a cohort of 330 age-matched healthy control subjects followed up for a median of 7.4 years.

Glucose-potassium-insulin infusions in the management of post-stroke hyperglycaemia: the UK Glucose Insulin in Stroke Trial (GIST-UK).

Background: Hyperglycaemia after acute stroke is a common finding that has been associated with an increased risk of death. We sought to determine whether treatment with glucose-potassium-insulin (GKI) infusions to maintain euglycaemia immediately after the acute event reduces death at 90 days.

Methods: Patients presenting within 24 h of stroke onset and with admission plasma glucose concentration between 6.

A patient with neuro-Behçet's disease is successfully treated with etanercept: further evidence for the value of TNFalpha blockade.

Behçet's disease is a chronic relapsing multisystem vasculitis with 49% of cases involving the CNS. Recently there have been two reports of neuro-Behçet's disease (NB) successfully treated with the tumour necrosis factor alpha (TNFalpha) monoclonal antibody infliximab. We describe a patient with longstanding NB who was poorly responsive to azathioprine, cyclosporin, thalidomide and methotrexate.

Effectiveness of population-based newborn hearing screening in England: ages of interventions and profile of cases.

Objective: The objective of this study was to determine the effectiveness in routine practice of the first phase of a national population-based newborn hearing screening and follow-up program that seeks to identify infants with bilateral permanent hearing loss of > or =40-dB hearing loss.

Methods: The study was a part of the independent evaluation of the 23 first phase sites (annual birth population approximately 120,000) of the national newborn hearing screening program in England. For each infant identified with the defined hearing loss, the measures of interest were degree and type of hearing loss, presence of risk factors, age of first audiologic assessment, age of identification of hearing loss, age of enrollment in an early support program, and age of hearing aid fitting.

Maternal anxiety following newborn hearing screening: the moderating role of knowledge.

Objectives: To describe the impact upon maternal anxiety of newborn hearing screening and examine the Possible moderating role of knowledge.

Methods: Questionnaires assessing maternal state anxiety, worry and certainty about the baby's hearing, and knowledge about screening, were sent to four groups of mothers three weeks after screening: Group 1 consisted of mothers whose babies had clear responses on a first or second screening test (n=103); Group 2 consisted of mothers whose babies had clear responses on the third screening test (n=81); Group 3 consisted of mothers whose babies did not have clear responses in one ear at the third screening test and were referred for audiological assessment (n=105); and Group 4 consisted of mothers whose babies did not have clear responses in either ear at the third hearing test and were referred for audiological assessment (n=55).

Results: Although mean anxiety levels were in the normal range, there was a significant trend for anxiety to rise as testing increased (F(1,327)=4.

Neonatal hearing screening: modelling cost and effectiveness of hospital- and community-based screening.

Background: Children with congenital hearing impairment benefit from early detection and management of their hearing loss. These and related considerations led to the recommendation of universal newborn hearing screening. In 2001 the first phase of a national Newborn Hearing Screening Programme (NHSP) was implemented in England.

Readiness of paediatric audiology services for newborn hearing screening: findings and implications from the programme in England.

As well as evaluating the newborn hearing screen itself, the government-funded evaluation of the implementation of a Newborn Hearing Screening Programme (NHSP) in England is assessing the impact of the screen on follow-up services. In the UK context, these are principally paediatric audiology, education, and social services. This article presents results from a mixed method research study involving paediatric audiology services specifically.

Screening for hearing loss in childhood: issues, evidence and current approaches in the UK.

Until recently, screening for childhood hearing loss in the UK was based on two universal (i.e. whole population) screens: the infant distraction test screen at age eight months and the school entry hearing screen at age four to five years.

Interprofessional working in the context of newborn hearing screening: Education and Social Services compare challenges.

This article presents data from a study undertaken as part of the national evaluation of the introduction of the newborn hearing screening programme (NHSP) in England. It considers the impact on Education and Social Services of NHSP from the perspective of how each agency perceives each other's role in circumstances where NHSP is requiring a greater focus on interagency and interprofessional working. The qualitative interview study involved 27 education and 15 social services respondents from phase 1 NHSP sites.

Maternal anxiety and satisfaction following infant hearing screening: a comparison of the health visitor distraction test and newborn hearing screening.

Background: Newborn hearing screening is currently replacing the health visitor distraction test (HVDT) conducted at eight months. Our previous research indicates that recall for further tests following newborn hearing screening can have a negative impact on the emotional well being of mothers, but it is not known if this is greater than that caused by recall following the distraction test.

Objective: To compare the impact on maternal anxiety and satisfaction of recall following newborn hearing screening and the HVDT.

Digital signal processing hearing aids, personal FM systems, and interference: is there a problem?

Objectives: To determine whether digital signal processing (DSP) hearing aids produce conducted radio frequency interference that can affect the use of personal FM systems, to quantify the nature of any such interference, and to discuss practical remedies.

Design: Sixteen DSP hearing aids were used. Measurements were made of the spectral characteristics of any conducted radio frequency interference produced by each aid with FM shoe and 40 cm direct audio input (DAI) lead when the DAI facility was enabled.

To what extent do the characteristics of the object of evaluation influence the choice of epistemological framework? The case of universal newborn hearing screening.

In this article, the authors explore the role that characteristics and circumstances attendant on the object of an evaluation might play in the choice of epistemological framework underpinning research design. They consider examples from the consumer-focused evaluation of the introduction of universal newborn hearing screening in England. In particular, they look at how screen- and program-specific issues exerted influence at the levels of epistemology and method, arguing that these choices are not simply a product of values and questions that underpinned one kind of approach to knowledge production in comparison with another.