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Amyotrophic lateral sclerosis/motor neuron disease and other related disorders are fatal progressive neurodegenerative conditions that have a substantial impact on quality of life (QoL). This systematic review examines the different methods and instruments used to assess QoL in this condition from which recommendations are made of those that evidence suggests are the most appropriate. Databases were used to survey the available literature to cover as many different approaches and papers as possible. Due to the wide variety of approaches to the measurement of QoL data across the papers, no meta-analysis was possible. The available evidence suggests that at the present time the most appropriate measures are the SF-36, a generic widely used QoL measure, and the disease specific ALSAQ-40. However, as many of the validation studies on the ALSAQ-40 were undertaken by the developers of this instrument, further independent research is desirable to confirm these conclusions.

The Amyotrophic Lateral Sclerosis Assessment Questionnaire (ALSAQ-40) is the most widely validated measure of health status for use with patients diagnosed with motor neuron disease/amyotrophic lateral sclerosis (MND/ALS). The questionnaire was designed to be used in studies evaluating treatment regimes where missing data may cause problems with data analyses. The purpose of this paper is to evaluate an algorithm for the imputation of missing dimension scores on the ALSAQ-40. We used a postal survey of patients diagnosed with MND/ALS. One thousand, nine hundred and seventy-nine patients were surveyed and 1093 (55.2%) questionnaires returned. Of these, complete ALSAQ-40 data was available in 854 (85.8%) cases. Data were deleted from this complete dataset, and in a randomly selected subset of 100 cases, and then imputed using the Expectation Maximization (EM) algorithm: results were then compared to the dataset prior to data deletion. Descriptive statistics, mean scores and spread of scores were almost identical between original and imputed datasets. Furthermore, the two datasets were highly correlated (intra-class correlation coefficient = 0.95 or greater), and mean differences were small (+/-1.00). We concluded that EM imputation for the ALSAQ-40 provides data that closely mirrors the original when this has been deliberately removed. Consequently, EM is likely to be appropriate for studies using the ALSAQ-40 that contain missing data points.

Carers of people with MND may experience changes to their health and lifestyle. Statutory and voluntary organizations are able to support the carer in various ways. This review investigates the personal impact on carers and their experiences of service provision. A systematic search of online and grey literature was made for the period 1994-2004. Thirty-two key texts were retrieved and a narrative synthesis conducted. The main themes were: 1) Impact on carer: general health, emotional state, life satisfaction, socio-economic concerns, relationships, and protective factors; 2) Experience of service provision: primary care, health professionals, social care, written information, and voluntary organizations. There is a paucity of written documentation on the experience of assisting someone with MND. Data collected are diverse and sometimes contradictory. Sample sizes are often small and generalization difficult. Main findings show that maintenance of social support, activities, and a positive outlook may lessen the chance of ill health arising from long hours spent caring, and having a named coordinator may assist with access to resources, and provide practical and emotional support during and after the caring role. Carers may become ill themselves unless adequate support is given to them throughout the course of the disease.

This paper uses the standard error of measurement (SEM) and the standard error of the difference (S(diff)) in relation to data on individuals with amyotrophic lateral sclerosis (ALS) to calculate the minimum change scores required by statistical criteria for each dimension of the Amyotrophic Lateral Sclerosis Assessment Questionnaire (ALSAQ-40). Data collected from a longitudinal study was used to evaluate the usefulness of SEM and S(diff) criteria and to compare these criteria with an anchor-based approach in determining meaningful change. Questionnaires were completed on two occasions, 3 months apart. 764 questionnaires were returned. Questionnaires included the ALSAQ-40 and dimension specific transition items. The ALSAQ-40 is a disease specific health-related quality of life instrument for use in studies of patients with ALS or other motor neuron diseases. For all dimensions of the ALSAQ-40 the patterns of SEMs were similar over time. The results suggest that, for example, six points change on a 0-100 transformed scoring of the physical mobility dimension may be considered on distributional grounds a minimum meaningful change. The demonstrated consistency of SEMs for the dimensions of the ALSAQ-40 is empirical evidence of the theoretically claimed advantage of this measure of sample independence, and supports use of this distributional approach to calculate meaningful change.

Objectives: To determine which factors are predictive of physical and mental health one year after a first measurement of health status in amyotrophic lateral sclerosis (ALS) patients., Methods: The Physical Component Summary (PCS) score and the Mental Component Summary (MCS) score of the SF-36 were used as the main outcome measures in patients enrolled in the European ALS Health Profile Study (ALS-HPS). Correlation and stepwise regression procedures were used to determine the relationship between patients' physical and mental health status at follow-up with baseline measures., Results: A total of 1118 patients were recruited into the ALS-HPS, of which 918 (82.11%) returned fully or partially completed baseline and follow-up surveys. PCS scores declined over time. No significant changes were reported for the MCS scores over time for patients with ALS. Baseline scores were found to be significant predictors of patients' health status over time., Conclusions: Overall, patients' physical health status at the time of recruitment was the major predictor of the physical health status at both first and second follow-up time assessments. The same relationship was found between baseline and follow-up mental health status. The study also confirms the appropriateness of the use of the SF-36 in ALS patients.

This paper examines the sensitivity to change over time of the Amyotrophic Lateral Sclerosis Assessment Questionnaire (ALSAQ-40). Individuals' health status change was assessed by means of the Rasch-based Reliable Change Index (RCI) for ALSAQ-40 questionnaires completed on two occasions, three months apart. In addition, at follow-up respondents indicated how much change they had experienced since baseline via dimension-specific self reported transition questions. 764 individuals returned questionnaires at baseline and follow-up. For all dimensions, of respondents defined by the RCI as worse, a majority rated themselves as worse. However, on two dimensions over 60% of the respondents who rated themselves as being worse were defined as unchanged by the RCI. As with effect size smaller RCI cut-off points might be needed for subjects with ALS. This study confirms that the ALSAQ-40 is a valid and responsive disease specific health related quality of life instrument for use in studies of patients with ALS or other motor neuron diseases.

Objectives: To evaluate response rate, data quality, score reliability and scaling assumptions of the 36 item Short Form Health Survey (SF-36) in a large scale pan European survey of amyotrophic lateral sclerosis (ALS) patients., Design: A questionnaire based survey of patients diagnosed with ALS across 15 European countries. SAMPLE PATIENTS: presenting at neurological clinics for treatment of their condition were asked to partake in the survey., Results: 948 patients have been recruited into the survey, from whom responses have been gained in 754 (79.5%). Scores on the eight dimensions of the SF-36 were found to manifest high internal consistency reliability. Items were, in most instances, found to be most highly correlated with their own (corrected) scale score than with other scale scores. However, on two dimensions (role-physical and role-emotional) there was high levels of missing data, together with substantial floor and ceiling effects. The two factor model (of underlying constructs of physical and emotional health) for the SF-36 suggested by the developers was not supported in this patient group. CONCLUSION The SF-36 appears to provide reliable information for this patient group, and for the most part there are high levels of item completeness and good spread of scores. This is not, however, true for the role functioning dimensions. Furthermore, the underlying two factor model for the SF-36 was not supported. The implications for measuring health status in this patient group are discussed.

OBJECT: The purpose of this study was to compare the performance of the five-item mental health dimension of SF-36 (the Mental Health Index, MHI-5) with that of the 22-item Psychological General Well-Being Index (PGWB) in patients presenting with amyotrophic lateral sclerosis/motor neuron disease, using tests of reliability and validity. DESIGN: A questionnaire-based survey of patients diagnosed with ALS across 15 European countries. SAMPLE: Patients presenting at neurological clinics for treatment of their ALS were asked to join the survey. RESULTS: 1048 patients with ALS have been recruited, of whom 861 (82.2%) have returned baseline questionnaires. There was evidence supporting the internal consistency of both instruments, and for their construct validity in distinguishing between groups, in terms of gender and age. The scores on the two instruments were highly correlated (Spearman rank correlation 0.85). CONCLUSION: Both the MHI-5 and the PGWB showed high levels of construct validity and internal reliability in this patient group. The MHI-5 has comparable psychometric performance to the PGWB, and can be used to measure and compare mental health in defined populations. [ABSTRACT FROM AUTHOR]

OBJECTIVES: The purpose of the study was to assess the validity and reliability of a new disease specific measure of health-related quality of life for ALS/MND: the 40-item ALS Assessment Questionnaire (ALSAQ-40). DESIGN: We carried out a longitudinal postal survey. The two administrations of the questionnaire were separated by 3 months SAMPLE: Subjects were patients diagnosed with ALS/MND and registered with the UK MND Association. RESULTS: Dimensions of the ALSAQ-40 were shown to have high levels of internal reliability. The ALSAQ-40 was found to be sensitive to changes in self reported overall health state. CONCLUSION: We concluded that the ALSAQ-40 has high levels of reliability and validity and can be used as an outcome measure in studies evaluating new treatment regimes and therapies. (Amyot Lat Scler 1999; 1:33–40). [ABSTRACT FROM AUTHOR]

Editorial. Discusses the evidence for an environmental factor in the causation of amyotrophic lateral sclerosis (ALS). Possible influences of genetic factors, of inherited susceptibility and of acquired exposure to environmental or endogeneous toxins; Health-related quality of life of patients with ALS.