Measuring reliable change in cognition using the Edinburgh Cognitive and Behavioural ALS Screen (ECAS)

doi:https://doi.org/10.1080/21678421.2017.1407794Background: Cognitive impairment affects approximately 50% of people with amyotrophic lateral sclerosis (ALS). Research has indicated that impairment may worsen with disease progression. The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) was designed to measure neuropsychological functioning in ALS, with its alternate forms (ECAS-A, B, and C) allowing for serial assessment over time. Objective: The aim of the present study was to establish reliable change scores for the alternate forms of the ECAS, and to explore practice effects and test-retest reliability of the ECAS?s alternate forms. Method: Eighty healthy participants were recruited, with 57 completing two and 51 completing three assessments. Participants were administered alternate versions of the ECAS serially (A-B-C) at four-month intervals. Intra-class correlation analysis was employed to explore test-retest reliability, while analysis of variance was used to examine the presence of practice effects. Reliable change indices (RCI) and regression-based methods were utilized to establish change scores for the ECAS alternate forms. Results: Test-retest reliability was excellent for ALS Specific, ALS Non-Specific, and ECAS Total scores of the combined ECAS A, B, and C (all?>?.90). No significant practice effects were observed over the three testing sessions. RCI and regression-based methods produced similar change scores. Conclusion: The alternate forms of the ECAS possess excellent test-retest reliability in a healthy control sample, with no significant practice effects. The use of conservative RCI scores is recommended. Therefore, a change of ?8, ?4, and ?9 for ALS Specific, ALS Non-Specific, and ECAS Total score is required for reliable chang

The path to specialist multidisciplinary care in amyotrophic lateral sclerosis: A population- based study of consultations, interventions and costs

<div><p>Background</p><p>Amyotrophic Lateral Sclerosis (ALS) is a devastating neurological condition that requires coordinated, multidisciplinary clinical management. ALS is prone to misdiagnosis as its signs and symptoms may be non-specific, which may prolong patients’ journey to multidisciplinary ALS care.</p><p>Methods</p><p>Using chart review and national register data, we have detailed the journey of a national cohort of ALS patients (n = 155) from the time of first symptom to presentation at a multidisciplinary clinic (MDC). Key milestones were analysed, including frequency of consultations, clinical interventions, and associated economic cost.</p><p>Results</p><p>A majority of patients was male (60%), 65 years of age and over (54%), and had spinal onset ALS (72%). Time from onset of first symptoms to ALS diagnosis was a mean of 15.1 months (median, 11). There was a mean interval of 17.4 months (median 12.5) from first symptoms to arrival at the MDC, and a mean of 4.09 (median, 4) consultations with health care professionals. Electromyography and nerve conduction studies were among the most common interventions. Direct referral by a general practitioner (GP) to a neurologist was associated with reduced cost, but not reduced diagnostic delay. Bulbar ALS was associated with shorter time from symptom onset to diagnosis. Neurologist consultation in the first three consultations was associated with lower costs prior to the ALS clinic attendance but not a shorter time from first symptom to final diagnosis. Mean cost prior to attending the MDC was €3,486 per patient.</p><p>Conclusions</p><p>Expedited referral to the multidisciplinary ALS clinic would have reduced costs by an estimated €2,072 per patient. Development of a standardised pathway with early referral to neurology of patients with suspected symptoms of ALS could limit unnecessary interventions and reduce cost of care.</p></div

Temporal pathway: Time from first symptom (months).

<p>Temporal pathway: Time from first symptom (months).</p

Clinical referrals.

<p>Clinical referrals.</p

Additional file 1: of Caregiving in ALS – a mixed methods approach to the study of Burden

Extracts from semi-structured interview guide and standardised questionnaires. (DOCX 16 kb

Caregiving in ALS - a mixed methods approach to the study of burden

Background: Caregiver burden affects the physical, psychological and emotional well-being of the caregiver. The purpose of this analysis was to describe an informal caregiver cohort (n = 81), their subjective assessment of burden and difficulties experienced as a result of providing care to people with Amyotrophic Lateral Sclerosis (ALS). Methods: Using mixed methods of data collection and analysis, we undertook a comprehensive assessment of burden and difficulties associated with informal caregiving in ALS. As part of a semi-structured interview a series of standardised measures were used to assess quality of life, psychological distress and subjective burden, and in an open-ended question caregivers were asked to identify difficult aspects of their caregiving experience. Results: The quantitative data show that psychological distress, hours of care provided and lower quality of life, were significant predictors of caregiver burden. From the qualitative data, the caregiving difficulties were thematised around managing the practicalities of the ALS condition, the emotional and psychosocial impact; limitation and restriction, and impact on relationships. Conclusions: The collection and analysis of quantitative and qualitative data better explores the complexity of caregiver burden in ALS. Understanding the components of burden and the difficulties experienced as a result of caring for someone with ALS allows for better supporting the caregiver, and assessing the impact of burden on the care recipient.</p

Patient characteristics—Summary statistics.

<p>Patient characteristics—Summary statistics.</p

Cost estimates.

<p>Cost estimates.</p