ObjectiveTo elucidate the relationship between disease stage in amyotrophic lateral sclerosis (ALS), as measured with the King's Clinical Staging System, and cognitive and behavioral change, measured with the Edinburgh Cognitive and Behavioural ALS Screen (ECAS).MethodsA large multicenter observational cohort of 161 cross-sectional patients with ALS and 80 healthy matched controls were recruited across 3 research sites (Dublin, Edinburgh, and London). Participants were administered the ECAS and categorized into independent groups based on their King's clinical disease stage at time of testing.ResultsSignificant differences were observed between patients and controls on all subtests of the ECAS except for visuospatial functioning. A significant cross-sectional effect was observed across disease stages for ALS-specific functions (executive, language, letter fluency) and ECAS total score but not for ALS-nonspecific functions (memory, visuospatial). Rates of ALS-specific impairment and behavioral change were also related to disease stage. The relationship between cognitive function and disease stage may be due to letter fluency impairment, whereas higher rates of all behavioral domains were seen in later King's stage. The presence of bulbar signs, but not site of onset, was significantly related to ALS-specific, ECAS total, and behavioral scores.ConclusionALS-specific cognitive deficits and behavioral impairment are more frequent with more severe disease stage. By end-stage disease, only a small percentage of patients are free of neuropsychological impairment. The presence of bulbar symptoms exaggerates the differences observed between disease stages. These findings suggest that cognitive and behavioral change should be incorporated into ALS diagnostic criteria and should be included in future staging systems.

BackgroundCaregiver 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).MethodsUsing 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.ResultsThe 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.ConclusionsThe 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.Electronic supplementary materialThe online version of this article (doi:10.1186/s12904-016-0153-0) contains supplementary material, which is available to authorized users.

ObjectivesAmyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND), is a debilitating terminal condition. Informal caregivers are key figures in ALS care provision. The physical, psychological and emotional impact of providing care in the home requires appropriate assistance and support. The objective of this analysis is to explore the needs of informal ALS caregivers across the caregiving course.DesignIn an open-ended question as part of a semistructured interview, caregivers were asked what would help them in their role. Interviews took place on three occasions at 4-month to 6-month intervals. Demographic, burden and quality of life data were collected, in addition to the open-ended responses. We carried out descriptive statistical analysis and thematic analysis of qualitative data.Setting and participantsHome interviews at baseline (n=81) and on two further occasions (n=56, n=41) with informal caregivers of people with ALS attending the National ALS/MND Clinic at Beaumont Hospital, Dublin, Ireland.ResultsThe majority of caregivers were family members. Hours of care provided and caregiver burden increased across the interview series. Thematic analysis identified what would help them in their role, and needs related to external support and services, psychological-emotional factors, patient-related behaviours, a cure and ‘nothing’. Themes were interconnected and their prevalence varied across the interview time points.ConclusionThis study has shown the consistency and adaptation in what caregivers identified as helpful in their role, across 12–18 months of a caregiving journey. Support needs are clearly defined, and change with time and the course of caregiving. Caregivers need support from family, friends and healthcare professionals in managing their tasks and the emotional demands of caregiving. Identifying the specific needs of informal caregivers should enable health professionals to provide tailored supportive interventions.

Psychometric properties of the ECAS using age and education adjusted norms indicate that the ECAS, when used as an overall measure of cognitive decline, is highly sensitive. Further comprehensive assessment is required for patients that present as impaired on the ECAS.