Widespread cerebral haemodynamics disturbances occur early in amyotrophic lateral sclerosis.

We wished to longitudinally assess early changes in cerebral perfusion (CP) and its relationship to cognitive impairment (CI) in ALS. Fourteen ALS patients at time of diagnosis and 11 spousal controls, both without CI, were longitudinally assessed to determine a relationship between CP and incidence of CI in early stage disease. Neuropsychological testing and CP measurements were performed in both ALS and control groups at the initial assessment (T0) and two time-periods post initial assessment, T1 and T2, taken on average 6.1 and 17.0 months after initial assessment (T0), respectively. CT perfusion was used to measure cerebral blood flow, blood volume, and mean transit time (MTT) for all cortical lobes, and subcortical grey and white matter. Two of 14 ALS patients progressed to CI. No differences in CP measurements existed at T0 or T1 between the ALS and control groups. At T2, widespread cortical differences in MTT were present between the two groups. The ALS group had significantly increased MTT in all cortical regions, as well as the thalamus, compared with the control group. Our findings suggest early widespread changes in CP occur outside the motor area in the absence of CI in ALS.

Neuropsychological functioning in PLS: a comparison with ALS.

OBJECTIVE: In order to characterize the nature and extent of neuropsychological dysfunction in primary lateral sclerosis (PLS), we studied prospectively cognitive, emotional, and behavioral functioning in PLS, and compared performances to functioning in amyotrophic lateral sclerosis (ALS). METHODS: Eighteen patients with PLS and 13 patients with ALS completed a neuropsychological test battery assessing both cognitive skills and emotional/behavioral functioning. RESULTS: Both PLS and ALS groups scored broadly within normal limits (mean T-scores greater than 40) on all cognitive measures and no significant between-group differences were found with the exception of one variable. However, when examined on a case by case basis, the data revealed considerable heterogeneity amongst patients in both groups. Overall, 39% of PLS patients and 31% of ALS patients were considered cognitively impaired. Ahigher than expected frequency of abnormal scores was noted for several tests of executive function in both groups, and a majority of PLS patients also exhibited abnormal behavioural symptoms. There was no relationship in PLS or ALS groups between cognitive functioning and disease duration, current site of disease, site of onset, functional status, and respiratory variables. Comparison between the PLS and ALS groups indicated virtually no differences in cognitive test scores and overall emotional/behavioural symptoms. CONCLUSIONS: We observed deficits in cognition and behaviour in a significant proportion of PLS patients which were comparable to those observed in ALS cases. Although deficits were not in the range of frontotemporal dementia, both ALS and PLS cases demonstrated deficits most prominently on tests of executive functioning.

Cerebral haemodynamic changes accompanying cognitive impairment in primary lateral sclerosis.

Our objective was to elucidate the relationship between cognitive decline and cerebral haemodynamics in patients with PLS. We examined 18 patients with PLS and contrasted both neuropsychological and cerebral perfusion findings with seven age- and education-matched non-PLS controls. PLS patients were stratified into two groups based on the number of abnormal neuropsychological test scores: 1) cognitively intact PLS patients (PLS; those having zero or one abnormal scores (n =14)), and 2) cognitively-impaired PLS patients (PLSci; those having two or more abnormal test scores (n =4)). There was considerable heterogeneity in level of cognitive functioning with four patients meeting the criteria for cognitive impairment. The findings were highly consistent with a frontotemporal lobar dysfunction. Using CT perfusion to assess cerebral haemodynamics, the PLSci group had increased cerebral blood volume (CBV) and mean transit time (MTT) with reduced cerebral blood flow (CBF). More specifically, MTT was significantly increased (p<0.05) in the PLSci group compared with controls in all regions and affected both grey and white matter, with the exception of the temporal lobe and subcortical parietal white matter. These observations suggest that a subset of PLS patients is subject to cognitive decline and that this process is associated with changes in cerebral haemodynamics.

Differentiation between primary lateral sclerosis and amyotrophic lateral sclerosis: examination of symptoms and signs at disease onset and during follow-up.

BACKGROUND: Motor neuron diseases can affect the upper motor neuron and/or the lower motor neuron. Both amyotrophic lateral sclerosis (ALS) and primary lateral sclerosis (PLS) are motor neuron diseases, and there is much debate as to whether these are 2 separate disorders or simply 2 points on a continuum. OBJECTIVE: To determine which clinical features at onset and during follow-up could help differentiate between PLS and ALS. DESIGN: Retrospective study comparing patients with a diagnosis of PLS or ALS for differences in symptoms or signs at disease onset and during follow-up. SETTING: Tertiary referral center. Patients Six hundred sixty-one patients with ALS and 43 patients with PLS were included in the study. RESULTS: At presentation, stiffness was the only symptom that was significantly different between patients with PLS and patients with ALS (observed in 47% and 4% of patients, respectively; P<.001). During follow-up, limb wasting was rare in patients with PLS (2%, compared with 100% in patients with ALS; P<.001). Disease duration was significantly longer in patients with PLS compared with patients with ALS (mean +/- SD, 11.2 +/- 6.1 vs 3.8 +/- 4.2 years, respectively; P<.001). During the 16 years of follow-up, the mortality rate was significantly lower in patients with PLS compared with patients with ALS (only 33% vs 89%, respectively; P<.001). CONCLUSION: Our findings suggest that a patient presenting with spasticity who does not develop wasting within 3 years most likely has PLS.

Prognosis of amyotrophic lateral sclerosis with respiratory onset.

Respiratory muscle involvement is a recognised, but often late, complication of amyotrophic lateral sclerosis (ALS). The clinical features and prognosis of 21 patients with respiratory onset ALS are reported here. On a retrospective chart review, it was found that 2.7% of patients with ALS presenting to a tertiary care specialty clinic have respiratory symptoms as their first clinical symptom of ALS. Only 14% of these individuals presented acutely and required emergency intubation. The mean survival time of the total group from symptom onset to death or permanent ventilation was 27.0 (14.9) months, which was not significantly different from the survival time in patients with bulbar onset ALS. Non-invasive positive pressure ventilation (NIPPV) significantly improved survival compared with those who did not use NIPPV. This study suggests that ALS with respiratory onset does not necessarily follow a rapidly progressive course.

The effect of involvement in participatory research on parent researchers in a Sure Start programme.

Involving service users, patients and members of local communities in health and social care research is becoming increasingly common. However, surprisingly little research has been carried out to examine the experiences of such lay researchers. This paper presents the findings of a study to investigate the experiences of a group of parent researchers involved in a community survey within a UK Sure Start programme. The survey was undertaken to provide insight into the early impact of the programme and inform Sure Start programme expansion. Researchers were recruited from the local community and were given an accredited training programme, before working on the development of the research itself. They took a lead role in the development, data collection, analysis and report writing phases of the survey and have been actively involved with the dissemination of findings. In order to gain insight into the experiences of the lay researchers involved in this work, three separate methods were used to collect data: questionnaires before and after the study, a focus group at the end of the data collection phase and by analysis of personal diaries kept by the parent researchers. Findings reported include lay researchers responses to the accredited training programme, the development of new skills and understanding, access and the conduct of interviews and the impact of the work both for Sure Start and the researchers themselves. Some of the strengths and difficulties of participatory research are discussed and comment made on the extent to which lay involvement impacted on the conduct of the survey.

Tian ma, an ancient Chinese herb, offers new options for the treatment of epilepsy and other conditions.

Our purpose is to bring attention to the antiepileptic properties of the Chinese herb tian ma and its constituents, as well as to suggest the potential for the development of new antiepileptic drugs (AEDs) related to this herb. All available literature regarding the chemistry, pharmacology, animal data, and clinical use of tian ma and its constituents are reviewed, showing that tian ma, its constituents, and its symbiotic fungus Armillaria mellea have antiepileptic properties in in vitro and in vivo models. One clinical study reportedly demonstrated the AED effects of a component of tian ma, vanillin. Thus, tian ma, its constituent vanillin, and its symbiotic fungus armillaria hold promise as cost-effective and less toxic alternatives to standard AEDs. In addition, similar chemical compounds may be developed as AEDs.

Use of complex adaptive systems metaphor to achieve professional and organizational change.

AIM: This paper uses the experiences of a programme designed to bring about change in performance of public health nurses (health visitors and school nurses) in an inner city primary care trust, to explore the issues of professional and organizational change in health care organizations. BACKGROUND: The United Kingdom government has given increasing emphasis to programmes of modernization within the National Health Service. A central facet of this policy shift has been an expectation of behaviour and practice change by health care professionals. METHODS: Change was brought about through use of a Complex Adaptive Systems approach. This enabled change to be seen as an inclusive, evolving and unpredictable process rather one which is linear and mechanistic. The paper examines in detail how the use of concepts and metaphors associated with Complex Adaptive Systems influenced the development of the programme, its implementation and outcomes. FINDINGS: The programme resulted in extensive change in professional behaviour, service delivery and transformational change in the organizational structures and processes of the employing organization. This gave greater opportunities for experimentation and innovation, leading to new developments in service delivery, but also meant higher levels of uncertainty, responsibility, decision-making and risk management for practitioners. CONCLUSION: Using a Complex Adaptive Systems approach was helpful for developing alternative views of change and for understanding why and how some aspects of change were more successful than others. Its use encouraged the confrontation of some long-standing assumptions about change and service delivery patterns in the National Health Service, and the process exposed challenging tensions within the Service. The consequent destabilising of organizational and professional norms resulted in considerable emotional impacts for practitioners, an area which was found to be underplayed within the Complex Adaptive Systems literature. A Complex Adaptive Systems approach can support change, in particular a recognition and understanding of the emergence of unexpected structures, patterns and processes. The approach can support nurses to change their behaviour and innovate, but requires high levels of accountability, individual and professional creativity.

Public health nursing: barriers and opportunities.

This paper reports the findings of a descriptive study that examined the understanding and practice of public health nursing in one English NHS Region. Using questionnaires and focus groups, data were collected from nurses working in Health Authorities, senior Trust management, operational management and nursing practice across hospital and community services. The data were analysed using qualitative methods. The paper reports one aspect of the study, namely the views of the lead nurses in the Trusts and the Health Authorities regarding public health and nursing. The findings suggest that there are varying interpretations of public health nursing, lack of collaboration between and within organizations and disciplines and limitations in the knowledge and skills possessed by practitioners. It is argued that these issues all present barriers to the development of public health nursing and need to be considered as a matter of urgency if organizations and clinicians are to make the most of the current opportunities.

The development and application of a public health skills assessment tool for use in primary care organisations.

The public health skills assessment instrument described in this paper was developed to provide a key group of UK primary care practitioners (health visitors) with a personal development planning tool and researchers with a robust assessment tool for use in evaluations of innovative local public health programmes. Pilot testing with over 120 practitioners has provided evidence of its reliability and validity as a research instrument as well as yielding useful insights for public health educators, practitioners and policy-makers. Factor analysis confirms 10 core competency domains for health visitors which are remarkably consistent with those recently identified for UK public health specialists, interpreted here as grounds for optimism in the greater co-ordination between strategic and front-line approaches to public health work in primary care. However, results also confirm earlier findings indicating low levels of skill amongst health visitors in tackling health inequalities through interventions such as community development, raising questions about their capability to work effectively in new roles proposed by the Department of Health.