Outcomes of a 5-week individualised MDT outpatient (day-patient) treatment programme for functional neurological symptom disorder (FNSD).

AIM: We report results from a 5-week MDT treatment programme, with individualised sessions, for a selected group of patients with FNSD, delivered in a neuropsychiatric outpatient setting. Primary aims were to (1) reduce symptoms, (2) improve functional performance and (3) improve health status. METHODS: Treatment involved individual sessions of neuropsychiatry, cognitive behavioural therapy, physiotherapy, occupational-therapy, education and family meetings. Outcome measures collected at the beginning and end of treatment and at 6 months, were patient and clinician reported. Aims were assessed by the following: symptom reduction (PHQ15, PHQ9, GAD7, SPIN, Rosenberg); health and social functioning (HONOS, WSAS); functional performance (COPM); health status (EQ-5D-5L) and patient-rated perception of improvement (CGI). RESULTS: Analyses of 78 patients completing the programme and attending a 6-month review revealed high-baseline levels of disability compared to EQ-5DL population norms and high rates of disability and psychopathology as indicated by the WSAS and mental health indices (PHQ9, GAD7, SPIN, Rosenberg's self-esteem). At baseline, 92.3% met the IAPT caseness threshold for depression and 71% met the IAPT caseness threshold for anxiety. A Friedman ANOVA over the three time points and Dunn-Bonferroni post hoc tests indicated statistically significant improvements from admission to discharge and admission to 6-month follow-up. Sustained improvements were seen in somatic symptoms (PHQ15), depression (PHQ9), anxiety (GAD7), health and social functioning (HONOS), functionality (COPM), health status (EQ-5D-5L) and patient-rated clinical global improvement (CGI). CONCLUSION: An MDT can effectively deliver an outpatient programme for FNSD which can serve as an alternative to costlier inpatient programmes. Early identification and treatment of co-morbidities is advised.

Subarachnoid haemorrhage guidelines and clinical practice: a cross-sectional study of emergency department consultants' and neurospecialists' views and risk tolerances.

OBJECTIVES: To establish if emergency medicine and neuroscience specialist consultants have different risk tolerances for investigation of suspected spontaneous subarachnoid haemorrhage (SAH), and to establish if their risk-benefit appraisals concur with current guidelines. SETTING: 4 major neuroscience centres in London. PARTICIPANTS: 58 consultants in emergency medicine and neuroscience specialities (neurology, neurosurgery and neuroradiology) participated in an anonymous survey. PRIMARY AND SECONDARY OUTCOME MEASURES: The primary outcome measure was the highest stated acceptable risk of missing SAH in the neurologically intact patient presenting with sudden onset headache. Secondary outcome measures included agreement with guideline recommendations, risk/benefit appraisal and required performance of diagnostic tests, including lumbar puncture. RESULTS: Emergency department clinicians accepted almost 3 times the risk of a missed SAH diagnosis compared with the neuroscience specialists (2.8% vs 1.1%; p=0.02), were more likely to accept a higher risk of missed diagnosis for the benefit of a non-invasive test (p=0.04) and were more likely to disagree with current published guidelines stipulating the need for LP in all CT-negative cases (p=0.001). CONCLUSIONS: Divergence from recognised procedures for SAH investigation is often criticised and attributed to a lack of knowledge of guidelines. This study indicates that divergence from guidelines may be explained by alternative risk-benefit appraisals made by doctors with their patients. Guideline recommendations may gain wider acceptance if they accommodate the requirements of the doctors and patients using them. Further study of clinical risk tolerance may help explain patterns of diagnostic test use and other variations in healthcare delivery.

Educational interventions in neurology: a comprehensive systematic review.

A fear of neurology and neural sciences (neurophobia) may have clinical consequences. There is therefore a need to formulate an evidence-based approach to neurology education. A comprehensive systematic review of educational interventions in neurology was performed. BEI, Cochrane Library, Dialog Datastar, EBSCO Biomedical, EBSCO Psychology & Behavioral Sciences, EMBASE, ERIC, First Search, MDConsult, Medline, Proquest Medical Library and Web of Knowledge databases were searched for all published studies assessing interventions in neurology education among undergraduate students, junior medical doctors and residents up to and including July 2012. Two independent literature searches were performed for relevant studies, which were then classified for level of evidence using the Centre of Evidence-based Medicine criteria and four levels of Kirkpatrick educational outcomes. One systematic review, 16 randomized controlled trials (RCTs), nine non-randomized cohort/follow-up studies, 33 case series or historically controlled studies and three mechanism-based reasoning studies were identified. Educational interventions showed favourable evaluation or assessment outcomes in 15 of 16 (94%) RCTs. Very few studies measured subsequent clinical behaviour (two studies) and patient outcomes (one study). There is very little high quality evidence of demonstrably effective neurology education. However, RCTs are emerging, albeit without meeting comprehensive educational criteria. An improving evidence base in the quality of neurology education will be important to reduce neurophobia.

The self-report Barthel Index: preliminary validation in people with Parkinson's disease.

BACKGROUND AND PURPOSE: To make a preliminary assessment of the reliability and validity of the self-report Barthel Index (SRBI) in people with Parkinson's (PWP) disease. METHODS: Thirty-nine PWP completed the SRBI, the self-report Schwab & England (S & E) scale and the PDQ-39. Standard statistical procedures were incorporated to assess the reliability and validity of the SRBI. RESULTS: A Cronbach's alpha coefficient of 0.69 was calculated, indicating adequate internal consistency. Reliability was further demonstrated through a Guttman split-half reliability coefficient of 0.74. Validity was demonstrated through highly significant correlations between the SRBI and the S & E scale (r = 0.64, P < 0.00), the activities of daily living dimension of the PDQ-39 (r = -0.60, P < 0.00) and mobility dimension of the PDQ-39 (r = -0.49, P < 0.01). CONCLUSIONS: Results suggest that the SRBI has the potential to be a reliable and valid indicator of ADL in PWP and utilized in studies that make comparisons across conditions. Further validation is required in a larger sample and through assessment of additional psychometric properties.

The Gilles de la Tourette syndrome-quality of life scale (GTS-QOL): development and validation.

BACKGROUND: Gilles de la Tourette syndrome (GTS) is a chronic neuropsychiatric disorder which has a significant detrimental impact on the health-related quality of life (HR-QOL) of patients. However, no patient-reported HR-QOL measures have been developed for this population. OBJECTIVE: The development and validation of a new scale for the quantitative assessment of HR-QOL in patients with GTS. METHODS: In stage 1 (item generation), a pool of 40 potential scale items was generated based on interviews with 133 GTS outpatients, literature review, and consultation with experts. In stage 2 (scale development), these items were administered to a sample of 192 GTS outpatients. Standard statistical methods were used to develop a rating scale satisfying criteria for acceptability, reliability, and validity. In stage 3 (scale evaluation), the psychometric properties of the resulting scale were tested in a second sample of 136 subjects recruited through the UK-Tourette Syndrome Association. RESULTS: Response data analysis and item reduction methods led to a final 27-item GTS-specific HR-QOL scale (GTS-QOL) with four subscales (psychological, physical, obsessional, and cognitive). The GTS-QOL demonstrated satisfactory scaling assumptions and acceptability; both internal consistency reliability and test-retest reliability were high (Cronbach alpha > or =0.8 and intraclass correlation coefficient > or =0.8); validity was supported by interscale correlations (range 0.5-0.7), confirmatory factor analysis, and correlation patterns with other rating scales and clinical variables. CONCLUSIONS: The Gilles de la Tourette syndrome (GTS)-specific health-related quality of life (HR-QOL) scale (GTS-QOL) is proposed as a new disease-specific patient-reported scale for the measurement of HR-QOL in patients with GTS, taking into account the complexity of the clinical picture of GTS.

Testing the validity of the PDQ-39 in patients with MSA.

We assessed the validity of the PDQ-39, a disease-specific health-related quality of life instrument for patients with Parkinson's disease, in patients with multiple system atrophy (MSA). Two hundred and seventy-nine patients completed the PDQ-39, the EQ-5D, the Hospital Anxiety and Depression Scale, and scales of life satisfaction and disease severity. Ceiling and floor effects were noted in some dimensions, and Mobility was skewed towards the severe end of the spectrum. Apart from the dimension of Social Support, all dimensions had high internal consistency. The factor structure of the PDQ-39 in MSA was stable, and convergent and divergent validity with other measures of quality of life and mental health were good. However, many of the specific features of MSA are not reflected in the PDQ-39. Higher order factor analysis did not support the use of a single summary index. We conclude that the PDQ-39 has only limited validity in patients with MSA.

Measuring quality of life in PSP: the PSP-QoL.

OBJECTIVE: To develop a new patient-reported outcome measure for progressive supranuclear palsy (PSP) and to test its psychometric properties. METHODS: First, the authors generated a pool of potential scale items from in-depth patient interviews. Second, the authors administered these items, in the form of a questionnaire, to a sample of people with PSP and traditional psychometric methods were used to develop a rating scale satisfying standard criteria for reliability and validity. Third, the authors examined the psychometric properties of the rating scale in a second sample. RESULTS: In stage 1, a pool of 87 items was generated from 27 patient interviews. In stage 2, a scale with two subscales (physical, 22 items; mental, 23 items), satisfying standard criteria for reliability and validity, was developed from the response data of 225 patients with PSP. In stage 3, the scale was examined in 188 people with PSP. Missing data were low, scores in both subscales were evenly distributed, floor and ceiling effects were small. Reliability was high (Cronbach's alpha 0.93, 0.95; test-retest 0.95, 0.92). Validity was supported by the interscale intercorrelation (0.60), factor analysis, and the magnitude and pattern of correlations with four other rating scales, disease severity, and disease duration. The psychometric properties of the new scale were similar in the United Kingdom and North America, and in clinic- and community-based samples studied. CONCLUSIONS: The Progressive Supranuclear Palsy Quality of Life scale (PSP-QoL) may be a helpful patient-reported scale for clinical trials and studies in PSP.

Measuring health-related quality of life in patients with progressive supranuclear palsy.

The development of psychometrically sound health-related quality of life (Hr-QoL) instruments has made it possible to assess subjectively experienced Hr-QoL quantitatively, and to incorporate Hr-QoL as a measure in medical research and clinical trials. Hr-QoL in patients with progressive supranuclear palsy (PSP) has been measured using generic (not disease-specific) instruments, or Parkinson's disease-specific scales. We give an overview of the development of a disease-specific Hr-QoL instrument for patients with PSP. Based on interviews with 27 patients with PSP a preliminary Hr-QoL instrument was developed and administered to over 300 people with PSP in the UK and US. Following psychometric analysis a rating scale with a physical and a mental subscale emerged. In this patient sample, both subscales satisfied criteria for scaling assumptions, acceptability, reliability and validity (correlations with other measures consistent with a priori hypotheses). The psychometric properties of this questionnaire are undergoing further evaluation.

Psychopathological profile in patients with severe bilateral hippocampal atrophy and temporal lobe epilepsy: evidence in support of the Geschwind syndrome?

Bilateral symmetrical hippocampal atrophy (BHA) has been implicated as a possible causal element in various neuropsychiatric disorders, in particular depressive disorder and schizophrenia. To test the hypothesis that bilateral symmetrical severe volume loss of the hippocampi is of causal relevance to these psychiatric syndromes rather than an epiphenomenon we assessed the psychopathology in a group of patients with temporal lobe epilepsy (TLE) and very severe bilateral symmetrical hippocampal atrophy and compared it with that of a patient control group. Patients with TLE and hippocampal volumes smaller than three standard deviations below the mean of a control population were identified and compared with a matched patient population with normal hippocampal volumes. Psychopathology was assessed by blinded trained psychiatrists using the Present State Examination and Neurobehavioral Inventory. The prevalence of psychiatric syndromes was high in both patient groups; however, there was no significant difference between the two groups. With use of the more specific Neurobehavioral Inventory a psychopathological pattern reminiscent of the Geschwind syndrome emerged when patients with BHA were characterized by caregivers. While BHA does not result in an increased prevalence of specific psychiatric syndromes, specific symptoms that characterize the Geschwind syndrome like hypergraphia and hyposexuality might be pathogenically related to hippocampal atrophy.

Quality of Life (QOL) of patients with Gilles de la Tourette's syndrome.

OBJECTIVE: This is the first study to investigate the Quality of Life (QOL) of patients with Gilles de la Tourette's Syndrome (GTS). METHOD: One hundred and three out-patients with GTS completed a semi-structured interview and 90 of these completed questionnaires screening for depression, anxiety and obsessive-compulsive behaviour. QOL was measured with the SF-36 and the Quality of Life Assessment Schedule (QOLAS). RESULTS: Patients with GTS showed significantly worse QOL than a general population sample. They had better QOL than patients with intractable epilepsy as measured by the QOLAS, although the SF-36 showed significant differences on the subscales Role Limitation due to physical problems and Social Functioning only. Factors influencing QOL domains were employment status, tic severity, obsessive-compulsive behaviour, anxiety and depression. CONCLUSION: QOL is impaired in patients with GTS. Measurement of QOL could be used alongside conventional measurements to assess benefit of treatment. We recommend the QOLAS and SF-36 be used.

The EQ-5D--a generic quality of life measure-is a useful instrument to measure quality of life in patients with Parkinson's disease.

OBJECTIVE: To test the feasibility and validity of the EQ-5D (a widely used generic (disease non-specific) quality of life (QoL) instrument which allows comparisons between different patient groups and the general population) to assess QoL in patients with Parkinson's disease. METHODS: All 124 patients with Parkinson's disease seen in a community based study on the prevalence of parkinsonism were asked to complete a QoL battery comprising the EQ-5D, the medical outcome study short form (SF-36), the PDQ-39, a disease specific instrument to assess QoL in PD, and the Beck depression inventory. A structured questionnaire interview and a complete neurological examination including the Hoehn and Yahr stage of illness scale, the Schwab and England disability scale, the motor section of the unified Parkinson's disease rating scale (UPDRS), and the mini mental state examination (MMSE) were performed on the same day. RESULTS: The response rate was 78% and the completion rate of the EQ-5D among responders was 96%. The EQ-5D summary index correlated strongly with the PDQ-39 (r=-0.75, p<0.0001) as well as the physical score of the SF-36 (r=0.61, p<0.0001). There was a significant correlation of the EQ-5D summary index with disease severity, as measured by the Hoehn and Yahr stage of illness, the Schwab and England disability scale, the motor section of the UPDRS, and the depression score. The EQ-5D summary index also distinguished between patients with and without depression, falls, postural instability, cognitive impairment hallucinations, and those with deterioration of health over the previous year. CONCLUSION: The EQ-5D is a feasible and valid instrument to measure QoL in Parkinson's disease and reflects the severity and complications of the disease.

Quality of life pre and post epilepsy surgery.

The aim of this work was to assess the health-related quality of life (HRQL) of patients pre and post surgical treatment for epilepsy. A total of 145 patients were interviewed during their pre-surgical assessment on the telemetry unit, Queen Square. The HRQL assessment comprised the quality of life assessment schedule (QOLAS), the EuroQol EQ-5D and the epilepsy surgery inventory (ESI-55). A total of 40 patients were followed up, of which 22 had undergone surgery and achieved 75% or greater reduction in seizures. The QOLAS scores for the patients who achieved 75% or greater seizure reduction post-op were significantly lower (i.e. improved HRQL) compared to baseline. The descriptive data suggest that the EQ-5D may not be capturing all of the QOL issues of relevance to patients with chronic, intractable epilepsy and the EQ-5D may not be valid for this group. Most patients queried the visual analogue scale (VAS) which asks for an overall rating of the respondent's self-perceived health. The most frequent comments, from 42% of patients, was that 'health' did not include their epilepsy. Despite this, the group whose seizures were reduced had significantly higher VAS scores at follow-up. We can conclude that the VAS is sensitive to clinical change. The baseline EQ-5D utility and follow-up scores were compared. There were no significant changes in QOL scores for either group. The patients who achieved 75% or greater reduction in seizures post-op scored significantly higher (i.e. better QOL) on 2/3 composite scores of the ESI-55 at follow-up. The QOLAS, the EQ-5D VAS and the ESI-55 were sensitive to clinically defined outcome. The results for the EQ-5D profile and the EQ-5D utility suggest that the EQ-5D is not a valid and responsive instrument for use in patients with intractable epilepsy.

Adjunctive therapy in epilepsy: a cost-effectiveness comparison of two AEDs.

The objective of this study was to compare the relative cost-effectiveness of two AEDs by a prospective clinical audit. Patients starting on the adjunctive therapies lamotrigine and topiramate were recruited from the out-patient epilepsy clinics at Queen Square. Three interview were scheduled: baseline; three months follow-up and six months from baseline. Of the 81 patients recruited, a total of 73 patients completed all three interviews. An intention to treat analysis was performed on the data. Seizure severity and frequency were assessed using the National Hospital Seizure Severity Scale. Side-effects, adverse events and reasons for stopping medication were also recorded. At the third interview, a total of 47/73 (64%) were still on the prescribed adjunctive drug. Outcome was assessed by two methods: the > 50% seizure reduction cited in the literature and a more stringent assessment of patient 'satisfaction' which we defined operationally on clinical criteria. Using this definition, a total of 10/73 (14%) patients were 'satisfied'. The relative costs of starting patients on each of the two AEDs were calculated, both drug costs and the costs of adverse events (the latter were defined as events requiring urgent medical attention). The costs of the two drugs were compared. A number of methodological issues relating to cost comparison are discussed. Outcome and pharmaco-economic studies need to assess more than reduction in number of seizures. They should take into account variables important for quality of life including side-effects and adverse events.

Adjunctive therapy in epilepsy with new antiepileptic drugs: is it of any value?

A total of 97 patients were recruited into a prospective, follow-up study after they were prescribed an adjunctive antiepileptic drug. The patients were followed up over a 6-month period. The interview included questions on Quality of Life, side-effects, adverse events and seizure frequency and severity. We operationally defined patients 'satisfaction' as (i) still on new drug; (ii) experiencing no side-effects (iii) experiencing no adverse events and (iv) had a greater than 50% reduction in seizures. A total of 13 patients (17%) reported being 'satisfied' according to our operational definition.

Eliciting EuroQol descriptive data and utility scale values from inpatients. A feasibility study.

We conducted a microstudy to elicit health state descriptions and utility values, using the EuroQol instrument, from a sample of acutely ill inpatients on 5 wards at University College London Medical School. Most current work to date has elicited such descriptive and valuation data from random surveys of the general population. One problem with this is that most responders from the general population have not actually experienced the states being valued. Our goal was to ascertain whether there were any differences between the values given by inpatients and those of the general population. However, the small sample size of patients included in our feasibility study means our conclusions must remain tentative. Nevertheless, the results suggest that patients give higher values than the general population. We suggest that more research needs to be done eliciting values from patients.