Real-life step and activity measurement: reliability and validity.

OBJECTIVES: Investigation of the reliability and validity of activity monitoring using a range of methods, namely manual counting, self report and two commercially available activity monitors. STUDY DESIGN: Reliability, accuracy and validity study. METHODS: Multiple convenience samples of healthy subjects were recruited to the study. Reliability of manual step count was determined using an intraclass correlation coefficient (ICC) (n = 10). Relationships between data from the Step Watch monitor (SAM) and (a) manual step counts (n = 18); (b) a second (different) activity monitor (ActivPAL); and (c) self reported activity levels (n = 22) were assessed using correlations. A Pearson's correlation and paired t-test was used to assess relations between routinely used monitors. RESULTS: Intra-rater reliability for manual step counts was excellent (ICC 0.99), but inter-rater reliability was poor (ICC 0.26). Indoor accuracy of the SAM was 96.06% and outdoor accuracy was 99.58%. Moderate correlations (rho = 0.423 to 0.595, p < 0.05) were identified between the SAM monitor activity levels and self report diaries. The SAM and the ActivPAL were found to be internally reliable within themselves (ICC 0.96 and 0.95 respectively), significantly correlated (r = 0.93, p < 0.001) but also significantly different (t = 2.179, p < 0.05) when used simultaneously over the same circuit. CONCLUSIONS: Activity monitors provide information that is related to actual activity and provide accurate and reliable data when tested on functional walking circuits. Activity monitors should not be used interchangeably due to the potential for systematic differences between the measurements obtained when applied simultaneously over the same repeated circuit.

Mobility and falls in people with Huntington's disease.

OBJECTIVE: The aim of this study was to estimate the frequency of falls in people with Huntington's disease (HD) and make a preliminary assessment of tools appropriate for assessing the risk of falling. DESIGN: Observational study. SETTING: Hospital clinic. SUBJECTS: 24 people with HD. MAIN MEASURES: Balance was assessed using the Berg Balance Scale (BBS) and Timed "Up & Go" (TUG) test. Walking speed over 10 m was recorded. Long-term monitoring of walking activity was undertaken. Unified Huntington Disease Rating Scale (UHDRS) motor, Functional Assessment Scale (FAS), Independence Scale (IS) and Total Functional Capacity (TFC) scores were obtained as well as data about falls and stumbles. Differences between "recurrent fallers" (>or=2 falls/year) and "non-fallers" (or=2 falls in the previous 12 months. Recurrent fallers walked less (p<0.01) and slower than non-fallers. Their balance (BBS) (p<0.01) was worse and TUG scores were higher (p<0.01). People with HD had increased risk of falls if TUG scores were >or=14 s or BBS scores

Report of the Task Force on pre-graduate education in Europe of the education committee of the European Federation of Neurological Societies Composition of the task force of the education committee on pre-graduate education.

BACKGROUND AND PURPOSE: The demographical evolution and the technological revolution seen in the last decades, in developed countries, have dramatically changed the practice of Neurology. However, the academic curriculum in many medical schools has not been updated accordingly over many of the European Countries. The Education Committee of the European Federation of Neurological Societies (EFNS) implemented in 2000 a Task Force on pre-graduate education trying to give guidelines to adequate pre-graduate education to the present status. METHODS AND DISCUSSION: Based on the results of two questionnaires, the first sent to the delegates of the EFNS and to the delegates of the European Board of Neurology, and the second answered by the Task Force members themselves, this paper describes the Task Force recommendations aimed to improve Neurology Education in the Medical Schools. These recommendations are also discussed with the analyses of the current bibliography available.

Physiotherapy and related activities in multiple sclerosis.

There are now about 50 randomized controlled trials into rehabilitation packages, physiotherapy or related specific and non-specific techniques in multiple sclerosis (MS). Generally these, and related systematic reviews, report benefits. Particular problems arise, however, with the blinding of assessment, determination of what is the active or beneficial input by the therapist, the use of multiple domains of assessment of quality of life and function without, sometimes, a clear statement of a trial hypothesis or primary outcome and the short-term nature of many studies. Therapy inputs can be broadly broken down into verbal interactions with the patient, physical inputs and referral/recommendation processes. Each may be relevant to the outcome. 'Response-shift' may be an important internal mechanism of mind whereby changes in 'quality of life' may not always parallel function emphasizing the case for clearly separating quality of life from functional assessment and attempting to make the latter as objective as possible. Trials of such complex interventions will need to randomize specified components of therapy against appropriate placebos or active treatment arms rather than no therapy, which will be ethically harder to sustain. Classification of physiotherapy inputs by type and 'dosage', a primary hypothesis under test and attention to concealed allocation of treatment, assessor blinding and intention to treat analysis together with improved measurements of function will assist in the consolidation of the evidence base for physiotherapy as an important component of management for MS patients.

Clinical skills evaluation of trainees in a neurology department.

Three to 12 evaluations of clinical performance using the mini-clinical evaluation exercise (Mini-CEX) (n = 124) or direct observation of procedural skills (DOPS) (n = 21) were performed on 27 trainees working in an NHS neurology department. The communications/ counselling skills subdomain was scored in 64 evaluations. For Mini-CEX the focus was on gathering data (22%), diagnosis (31%), management (34%) and counselling (7%) (focus not recorded in 6%). For DOPS, lumbar puncture was the most common evaluated procedure (57%). Mini-CEX evaluations lasted 23.8 minutes (10.6) (mean, sd) and DOPS 25.9 minutes (12.6). Mini-CEX scores for overall competence and communication skills were mean 5.99 (sd 0.95, range 4-8) and 5.98 (sd 1.21, range 3-9) and for DOPS 5.71 (sd 0.90, range 4-8) both on scales of 1 to 9. Overall trainee competence and communication scores increased with year of training (p < 0.001, p < 0.004 univariate analysis). Assessors undertook up to three or four assessments in a session. Assessors and trainees considered that the observation and feedback had been 'very' or 'quite' useful in providing a relevant element of assessment. These assessments were feasible and useful in a neurology department and provided some evidence for increasing performance with trainee seniority. More assessor time (approximately one hour) than trainee time (24-26 min) was needed for each assessment undertaken.

Selecting specialist registrars by station interview.

Appointments to the specialist registrar (SpR) grade depend almost entirely on performance at interview, yet standard panel interviews do not directly assess the competences required of a medical trainee. In this study, station interviews were used to select neurology SpRs. Eighteen candidates were assessed in three interviews, each involving three stations: a curriculum vitae (CV)-based interview, an interview with a simulated patient, and a discussion of scenarios based upon teaching, audit and research. Two or three assessors at each station ranked candidates independently before discussing the pooled rankings and reading written references. The CV-based interview rankings (resembling a traditional panel interview) correlated less well with the overall rankings (r=0.54) than did research (r=0.83), information giving (r=0.75), audit (r=0.70) or teaching presentation (r=0.59). Station interviews appear fairer (providing more time, more independent examiners, fresh starts at each station), although they require more planning and expense. Competency-based assessments should be more widely used in selecting medical trainees.

Community walking activity in neurological disorders with leg weakness.

BACKGROUND: The aims of this study were to determine walking mobility in the community in individuals with lower limb weakness and to establish the extent to which some clinic based measures predict such activity. METHODS: Five groups (n = 12-18) of independently ambulant patients with lower limb weakness due to neurological conditions and a matched healthy control group were recruited. Measures of isometric knee extension/flexion muscle strength, time to stand up (sit-to-stand, STS), gait speed, and daily step counts (recorded over 7 days) were obtained. The Rivermead Mobility Index (RMI) provided a measure of functional ability. Between group differences and associations were explored. Backward stepwise regression analysis was used to identify variables influencing daily step count in individuals with neurological impairment. RESULTS: Patients were significantly weaker (mean (SD) quadriceps strength 69+/-34% v 102+/-37% predicted), slower to stand up (2.9+/-1.3 v 2.0+/-0.6 s), and had slower self selected gait speed (0.74+/-0.3 v 1.2+/-0.2 m/s) than controls. Mean daily step count was also lower (3090+/-1902 v 6374+/-1819) than in controls. In neurology patients step count was correlated with RMI score (r(s) = 0.49, p<0.01) and STS (r = -0.19, p<0.05). However, self selected gait speed was the only significant predictor in the regression analysis (p<0.01) of daily mean step count. CONCLUSIONS: Measures of muscle strength, timed STS, and RMI do not appear to closely reflect community walking activity in these patient groups. Self selected gait speed was partially predictive. Measurement of community walking activity may add a new dimension to evaluating the impact of interventions in neurological disorders.

Falls and stumbles in myotonic dystrophy.

OBJECTIVE: To investigate falls and risk factors in patients with myotonic dystrophy type 1 (DM1) compared with healthy volunteers. METHODS: 13 sequential patients with DM1 from different kindreds were compared with 12 healthy volunteers. All subjects were evaluated using the Rivermead Mobility Index, Performance Oriented Mobility Assessment, and modified Activities Specific Balance Confidence scale. Measures of lower limb muscle strength, gait speed, and 7-day ambulatory activity monitoring were recorded. Subjects returned a weekly card detailing stumbles and falls. RESULTS: 11 of 13 patients (mean age 46.5 years, seven female) had 127 stumbles and 34 falls over the 13 weeks, compared with 10 of 12 healthy subjects (34.4 years, seven female) who had 26 stumbles and three falls. Patients were less active than healthy subjects but had more falls and stumbles per 5000 right steps taken (mean (SD) events, 0.21 (0.29) v 0.02 (0.02), p = 0.007). Patients who fell (n = 6) had on average a lower Rivermead Mobility score, slower self selected gait speed, and higher depression scores than those who did not. CONCLUSIONS: DM1 patients stumble or fall about 10 times more often than healthy volunteers. Routine inquiry about falls and stumbles is justified. A study of multidisciplinary intervention to reduce the risk of falls seems warranted.

Electromyography characterization of stretch responses in hemiparetic stroke patients and their relationship with the Modified Ashworth scale.

OBJECTIVES: To determine the validity of the Modified Ashworth Scale as a measure of spasticity by determining its relationship to surface electromyography activity and contracture. DESIGN: A controlled study of hemiparetic stroke patients with spasticity. SETTING: A physiotherapy department in a secondary care hospital. SUBJECTS: Thirty-one stroke patients and 20 healthy volunteers. MAIN MEASURES: The resistance to passive movement around the knee and ankle of the affected and unaffected legs was rated using the Modified Ashworth Scale. Passive range of movement was measured with a goniometer. Surface electromyography recordings of four lower limb muscles were taken during passive stretches of the knee and ankle. RESULTS: Hemiparetic patients produced surface electromyography responses to stretch that were of greater amplitude (unaffected limbs: mean = 25.82 mV (43.85), affected limbs: mean = 24.77 mV (35.46)) than those of healthy volunteers (mean = 15.85 (29.96)). The affected muscles of hemiparetic patients were more likely to produce surface electromyography responses to stretch of a sustained duration (45% of cases) compared with unaffected limbs (24% of cases) and those of healthy volunteers (16% of cases). The Modified Ashworth Scale showed a positive correlation with the magnitude (p < 0.05) and duration (p < 0.001) of the surface electromyography response. High scores on the Modified Ashworth Scale were associated with contracture (p < 0.001). Contracted muscles produced significantly greater surface electromyography reflex responses compared with noncontracted muscles (p < 0.05). CONCLUSION: The Modified Ashworth Scale reflects spasticity in terms of surface electromyography stretch responses produced by passive movement, but the relationship of spasticity to contracture remains unclear.

Population based study of late onset cerebellar ataxia in south east Wales.

OBJECTIVE: To determine the prevalence and causation of late onset cerebellar ataxia (LOCA) in south east Wales, United Kingdom. METHODS: A population based study of LOCA was conducted in a defined geographical region with a total population of 742,400. Multiple sources of ascertainment were used to identify all cases prevalent on 1 January 2001. The inclusion criteria were: a predominantly progressive cerebellar ataxia with onset of symptoms at age > or = 18 years; and disease duration of > or = 1 year. Cases with known acquired ataxias, ataxic syndromes with associated prominent autonomic dysfunction and/or atypical parkinsonism suggestive of multiple system atrophy and disorders with ataxia as a minor feature were excluded. RESULTS: We identified 76 index cases of LOCA, of whom 63 were sporadic, idiopathic LOCA (ILOCA) and 13 were familial LOCA, of whom six had either spinocerebellar ataxia type 6, Friedreich's ataxia or dominant episodic ataxia. The mean annual incidence rate for the period 1999-2001 was 0.3/100,000 population/year. The crude prevalence rates were 8.4 per 100,000 (95% CI 7.2 to 11.6) for ILOCA and 1.8 per 100,000 (95% CI 0.8 to 2.7) for inherited LOCA. Of the 54/63 (85.7%) patients with ILOCA who were assessed, mean (SD) age at onset of symptoms was 53.8 (14.1) years (range 19 to 78) with a male:female ratio of 2.1:1. The mean disease duration was 8.7 (6.3) years (range 1 to 31). The most frequent presenting complaint was disturbance in gait (90.7%). One-third had a relatively pure cerebellar syndrome (33.3%) and two-thirds (66.7%) had additional extracerebellar neurological features. The majority (92%) were ambulant but only 9.3% were independently self-caring. CONCLUSION: This population based study provides insight into LOCA within a defined region and will inform decisions about the rational use of healthcare resources for patients with LOCA.

Quantified measurement of activity provides insight into motor function and recovery in neurological disease.

BACKGROUND: A direct quantitative measurement of locomotor activity in an individual's own environment over an extended period may help in evaluating the impact of impairments in neurological disorders. OBJECTIVE: To investigate the reliability and validity of activity monitoring in neurological patients and healthy subjects. METHODS: Initial reliability studies were completed on 10 healthy subjects and 10 mobility restricted neurological patients. Validity was investigated using 7 days of ambulatory monitoring with the Step Watch( step activity monitor, laboratory based measures of gait and the Rivermead Mobility Index (RMI) in 10 patients with multiple sclerosis, 10 with Parkinson's disease, and 10 with a primary muscle disorder. Additionally, 30 healthy subjects participated in the study. Two clinical illustrations of ambulatory monitoring are provided. RESULTS: The mean (range) right step count of 7 days of monitoring in both healthy and neurological patients proved a reliable measure of activity (intra-class correlations 0.89 and 0.86 respectively). The 7 day mean (range) right step count was 5951 (2886-9955) in healthy subjects, 3818 (1611-5391) in patients with Parkinson's disease, 3003 (716-5302) in those with muscular disorders, and 2985 (689-5340) in those with multiple sclerosis. A moderate correlation was noted between 7 day mean step count and gait speed (r = 0.45, p = 0.01) in the grouped neurological patients but not the RMI (r(s) = 0.3, p = 0.11). CONCLUSION: Ambulatory monitoring provides a reliable and valid measure of activity levels. Neurological patients, living independently, demonstrate lower activity levels than healthy matched controls. Ambulatory monitoring as an outcome measure has potential for improving the evaluation of ambulation and providing insight into participation.

Use of videotape to assess mobility in a controlled randomized crossover trial of physiotherapy in chronic multiple sclerosis.

OBJECTIVES: To determine to what degree assessment of mobility based on comparison of videotape recordings before and after courses of physiotherapy in patients with chronic multiple sclerosis (MS) is reliable, correlates with 'live' assessments and indicates benefit. DESIGN: Prospective data collection within a randomized crossover controlled trial of physiotherapy at home, as an outpatient, or 'no therapy' in 40 patients. SETTING: Hospital outpatients: outpatient and home physiotherapy. OUTCOMES: Mobility change based on a comparison of short video recordings before and after each treatment period was scored independently by two physiotherapists blinded to therapy type and other measures of outcome. Scores were compared with changes in the Rivermead Mobility Index (RMI) and other indices assessed by a physiotherapist in the patient's home. RESULTS: The two video observers agreed substantially on patient outcome. Changes in walking based on video correlated with RMI for home treatment (r = 0.41, p = 0.008) but not for hospital or no treatment periods (r = 0.14 and 0.15): video changes correlated with the 'live' assessor's global change score inconsistently ('no therapy' r = 0.48, p = 0.002, hospital r = 0.30, p = 0.06 and home r = 0.17, p = 0.30 treatment periods). Based on video data alone, improved mobility was evident following home therapy for only one observer but not for the other or the averaged scores of both. CONCLUSION: There was substantial agreement between two observers deciding on change in mobility based on independent blinded evaluation of short video sequences. However the correlations of these with 'live' assessments were variable. Physiotherapy had a less clear benefit on mobility based on video analysis alone compared with 'live' assessments. The study highlights the need for more objective measures of habitual mobility over longer periods.

Inter- and intra-rater reliability of cervical auscultation to detect aspiration in patients with dysphagia.

OBJECTIVE: To measure the inter- and intra-rater reliability of cervical auscultation used alone to detect aspiration in dysphagic patients. SETTING: A university teaching hospital. DESIGN: Comparison of the detection of aspiration in 16 recorded swallow sounds by five speech and language therapists on two occasions. Swallow sounds were recorded simultaneously with videofluoroscopy. SUBJECTS: Sixteen patients referred for assessment of dysphagia with videofluoroscopy. RESULTS: The kappa statistic for multiple raters showed fair agreement between raters (kappa = 0.28). There was high agreement when aspiration occurred but in non-aspirating swallows there was significant overdetection of aspiration (p < 0.001 McNemar's test). The intra-rater reliability within different individuals was widely variable (kappa = 0.55 (range 0.31-0.85)). CONCLUSIONS: Presented with the swallowing sounds in isolation speech and language therapists cannot reliably classify swallows into those with accompanying aspiration and those without. There appears to be a problem of over detection of aspiration. Even in this small study, however, some individual therapists achieve such high reliability (kappa = 0.85) that they must be using successful internal criteria to interpret the swallow sounds correctly and further qualitative research may identify these.

Asthma and multiple sclerosis: an inverse association in a case-control general practice population.

BACKGROUND: Th1/Th2 imbalance is hypothesized to up-regulate some diseases and down-regulate others. Compared to controls, multiple sclerosis (MS) (Th1-mediated) has been linked to a reduced risk of allergy and asthma (Th2-mediated), based on patient questionnaire studies and a review of asthma medication. AIM: To investigate whether MS is associated with a reduced risk of Th2-associated diseases and an increased risk of Th1-associated diseases. DESIGN: Retrospective matched case-control study. METHODS: Three hundred and twenty MS patients and controls matched for age, gender, location and smoking were selected from the Welsh General Practice Morbidity Database from 1995-99. Case and control records were assessed for Th1-mediated and Th2-mediated diseases. RESULTS: Overall, 346 MS patients were identified, giving a prevalence of 127 per 100 000. There was an inverse relationship between multiple sclerosis (MS) and asthma (OR 0.33; 95%CI 0.15-0.77). No statistically significant relationships emerged between other Th2-associated (eczema, dermatitis) or any Th1-associated (rheumatoid arthritis, thyroid disorders, inflammatory bowel disease [IBD], type 1 diabetes) diseases and MS, although no patient in either group had treated type 1 diabetes. A trend existed for IBD, with 5/320 of cases affected and no controls; OR infinity; 95%CI 1.30-infinity; p=0.063. DISCUSSION: This inverse association between MS and asthma is compatible with a Th1/Th2 imbalance. Although the Th1/Th2 theory is probably an over-simplification in MS, a shift from Th1 cytokine dominance towards Th2 may provide drug-targeting routes for MS.

Intravenous immunoglobulin in neurological disease: a specialist review.

Treatment of neurological disorders with intravenous immunoglobulin (IVIg) is an increasing feature of our practice for an expanding range of indications. For some there is evidence of benefit from randomised controlled trials, whereas for others evidence is anecdotal. The relative rarity of some of the disorders means that good randomised control trials will be difficult to deliver. Meanwhile, the treatment is costly and pressure to "do something" in often distressing disorders considerable. This review follows a 1 day meeting of the authors in November 2000 and examines current evidence for the use of IVIg in neurological conditions and comments on mechanisms of action, delivery, safety and tolerability, and health economic issues. Evidence of efficacy has been classified into levels for healthcare interventions (tables 1 and 2).

Prescribing for multiple sclerosis patients in general practice: a case-control study.

OBJECTIVES: To examine the prescribing patterns for multiple sclerosis (MS) patients resident in Wales by general practitioners (GPs), compared to an age, gender and GP surgery matched control population. METHODS: Anonymised data for 1996 were obtained for all patients from 24 GP practices in the all-Wales General Practice Morbidity Database (GPMD). This covered 220 538 patient years at risk for 1996. Cases were selected as those with a Read code of MS at some point from 1993 to 1996 (therefore had consulted the GP at least once during this time). The controls were age, gender and surgery matched patients randomly selected from the GPMD. RESULTS: A total of 216 cases were identified, giving a prevalence of 97.9 per 105. Cases were prescribed a mean of 15 drugs each in 1996 compared to eight drugs for controls (P < 0.0005). Compared with controls, MS patients were prescribed significantly more laxatives, diuretics, hypnotics and anxiolytics, antidepressants, antiepileptics (mainly carbamazepine), corticosteroids, oxybutynin, vitamin B12 and skeletal muscle relaxants (predominantly baclofen; P < 0.05). Certain 'MS specific' drugs were not frequently prescribed, such as cytotoxic immunosuppressants (two cases), amantadine (one case) and isoniazid (no cases). No case was prescribed medication for erectile dysfunction. Over 80% (44/53) of corticosteroid prescriptions for MS were for oral prednisolone. Over one-third (39%, 9/23) of cases prescribed a corticosteroid received a 'chronic' course. Over one-third (5/14) of courses of selective-serotonin re-uptake inhibitors (SSRI) for cases were identified as subtherapeutic. CONCLUSIONS: MS patients were high users of prescribed medicines, having almost twice as many prescriptions from the GP compared to controls. GP prescribing often reflected available evidence from published controlled trials, hence cytotoxic immunosuppressants, drugs for fatigue and tremor were seldom prescribed, whereas drugs such as oxybutynin and skeletal muscle relaxants were frequently prescribed. However, the increased use of certain drugs compared to controls such as diuretics, vitamin B12, hypnotics and anxiolytics were unsubstantiated in the literature. Furthermore, no published well-controlled clinical trials were found utilizing oral prednisolone or assessing the possible therapeutic benefit of chronic courses of corticosteroids in MS, both of which were prescribed by the GP. The absence of medication for sexual dysfunction (prelicensing of sildenafil), a reportedly common MS problem, was discussed. The relatively high incidence of subtherapeutic courses of SSRIs needs further investigation, given the increased incidence of depression and suicide associated with MS.