The Role of Attachment Style, Adverse Childhood Experiences and Dissociation in Migraine.

Migraine and chronic migraine are caused by a combination of modifiable and non-modifiable genetic, social, behavioral and environmental risk factors. Further research of possible modifiable risk factors for this headache disorder is merited, given its role as one of the leading causes of years lived with disability per year. The first aim of this online cross-sectional study was to investigate the psychosocial risk factors that predicted chronic migraine and severe migraine-related disability in 507 Irish and UK participants, focusing specifically on childhood maltreatment, attachment and tendency to dissociate, or experience depressed mood and/or anxiety. Additionally, this study aimed to examine variables that mediated the relationships between these psychosocial risk factors and migraine chronicity or severe migraine-related disability. Adjusted binary logistic regression revealed that shutdown dissociation (Odds Ratio [OR] 4.57, 95% Confidence Interval [CI] 2.66-7.85) and severe physical abuse (OR 4.30, 95% CI 1.44-12.83 had significant odds of predicting migraine chronicity, while depression (OR 3.28, 95% CI 1.86-5.77) significantly predicted severe migraine-related disability. Mediation analyses indicated that shutdown dissociation mediated the relationship between seven predictor variables and both chronicity and severe disability including possible predisposing factors emotional abuse, physical neglect, avoidant attachment and anxious attachment. These findings suggest that early life stressors (such as childhood trauma and avoidant attachment style), shutdown dissociation and depression may impact on migraine trajectory. To investigate whether these psychosocial factors are risk factors for migraine chronicity or disability, prospective research should be conducted in this area to account for fluctuations in migraine chronicity over time.

Individual quality of life in spousal ALS patient-caregiver dyads.

BACKGROUND: Quality of life is a basic goal of health and social care. The majority of people with Amyotrophic Lateral Sclerosis (ALS) are cared for at home by family caregivers. It is important to recognize the factors that contribute to quality of life for individuals to better understand the lived experiences in a condition for which there is currently no curative treatment. AIM: To explore individual quality of life of people with ALS and their informal caregivers over time. METHODS: Over three semi-structured home interviews, 28 patient-caregiver dyads provided information on a range of demographic and clinical features, psychological distress, caregiver burden, and individual quality of life. Quality of life data were analysed using quantitative and qualitative methods with integration at the analysis and interpretation phases. RESULTS: Individual Quality of Life was high for patients and caregivers across the interviews series, and higher among patients than their care partners at each time point. Family, hobbies and social activities were the main self-defined contributors to quality of life. The importance of health declined relative to other areas over time. Friends and finances became less important for patients, but were assigned greater importance by caregivers across the illness trajectory. Psychological distress was higher among caregivers. Caregiver burden consistently increased. CONCLUSION: The findings from this study point to the importance of exploring and monitoring quality of life at an individual level. Self-defined contributory factors are relevant to the individual within his/her context. As an integrated outcome measure individual quality of life should be assessed and monitored as part of routine clinical care during the clinical encounter. This can facilitate conversations between health care providers, patients and families, and inform interventions and contribute to decision support mechanisms. The ascertainment of self-defined life quality, especially in progressive neurodegenerative conditions, mean health care professionals are in a better position to provide person-centred care.

Equivalency and practice effects of alternative versions of the Edinburgh Cognitive and Behavioral ALS Screen (ECAS).

To examine the equivalency of ECAS versions A, B, and C in an Irish cohort, and to examine potential practice effects, 236 healthy controls were recruited through the Irish ALS control database. One hundred and seventy-six (176) controls completed ECAS version A, B, or C. Separately, 60 controls completed all three versions (A-B-C), consecutively, four months apart. TOST analysis found that ECAS A was equivalent to ECAS B and C. ECAS B and C were not statistically equivalent, however the difference between them was minimal. Participants showed improvement in ECAS performance over time, indicative of practice effects. Significant improvement was observed from time 1 to 2, but not from time 2 to 3. We propose Irish specific reliable change index (RCI) scores that take into consideration practice effects and measurement error. These thresholds will help quantify clinically meaningful cognitive decline in ALS patients, leading to improved quality of care.

ALS-specific cognitive and behavior changes associated with advancing disease stage in ALS.

OBJECTIVE: To 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). METHODS: A 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. RESULTS: Significant 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. CONCLUSION: ALS-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.

Needs of informal caregivers across the caregiving course in amyotrophic lateral sclerosis: a qualitative analysis.

OBJECTIVES: Amyotrophic 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. DESIGN: In 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 PARTICIPANTS: Home 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. RESULTS: The 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. CONCLUSION: This 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.

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

BACKGROUND: 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 change.

Caregivers of patients with amyotrophic lateral sclerosis: investigating quality of life, caregiver burden, service engagement, and patient survival.

Few studies in amyotrophic lateral sclerosis (ALS) have profiled disease-specific features of the condition in conjunction with assessment of caregivers' burden, distress, quality of life, and investigated patient survival. Eighty-four ALS patients and their primary caregivers were enrolled. Patients completed ALS-specific measures of physical and cognitive function, while caregivers completed measures of anxiety, depression, caregiver burden, and quality of life. Patient-caregiver dyads were interviewed about their health-service utilisation. Survival data were obtained through the Irish register for ALS. Participants were dichotomised into low/high groups according to the severity of self-reported caregiver burden, based on statistically derived cut-off scores. High-burdened caregivers (n = 43) did not significantly differ from low-burdened caregivers (n = 41) with respect to disease-specific characteristics, i.e., ALSFRS-R, bulbar- or spinal-onset ALS, disease duration, or survival data. However, significant differences were reported on subjective measures of anxiety (p < 0.000), depression (p < 0.001), distress (p < 0.000), and quality of life (p < 0.000). These data demonstrate the limited impact of ALS patient-related variables, i.e., ALSFRS-R and onset, on caregiver burden in ALS, and identify the importance of the psychological composition of caregivers. This study suggests that the subjective experience of individual caregivers is an important factor influencing the severity of experienced caregiver burden.

From first symptoms to diagnosis of amyotrophic lateral sclerosis: perspectives of an Irish informal caregiver cohort-a thematic analysis.

OBJECTIVES: Informal caregivers play an integral part in the management of amyotrophic lateral sclerosis (ALS). The objective of this study was to explore the journey from first problem symptoms to diagnosis from the perspective of informal caregivers providing care to people with ALS. DESIGN: As part of a semistructured interview, information was collected on a range of caregiver demographic details, and from an open-ended question their experiences of the time of symptom onset to diagnosis. We carried out descriptive statistical analysis and thematic analysis of qualitative data. SETTING AND PARTICIPANTS: Home interviews with informal caregivers (n=74) of people with ALS attending the National ALS/Motor Neuron Disease Clinic at Beaumont Hospital, Dublin, Ireland. RESULTS: This was a largely female and spousal cohort of caregivers, living with the patient for whom they provided informal care. The majority of patients were men and were spinal onset. Caregivers described the time from first symptoms to diagnosis. Using a primarily inductive approach, the coding was data driven and the codes and themes derived from the content of these descriptions. Two main themes were identified (1) problem signs and symptoms (A) noticing and (B) reaction; (2) interaction with the health services. CONCLUSIONS: Exploring the perspectives of caregivers from first problem symptoms to diagnosis provides valuable insights into the development of the condition, impediments to its recognition, help-seeking behaviours and interactions with healthcare services. The journey from early symptoms to diagnosis is important for future decision-making, affects readiness for caregiving and could negatively impact on caregiver health and well-being. The early acknowledgement by healthcare professionals of stressors along the journey to diagnosis, and appreciation of their possible impact on caregivers is important. The separate needs of caregivers should be assessed on a regular basis.

Screening for cognitive dysfunction in ALS: validation of the Edinburgh Cognitive and Behavioural ALS Screen (ECAS) using age and education adjusted normative data.

BACKGROUND: Cognitive and behavioural changes are an important aspect in Amyotrophic Lateral Sclerosis (ALS). The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) briefly assesses these changes in ALS. OBJECTIVE: To validate the ECAS against a standardised neuropsychological battery and assess its sensitivity and specificity using age and education adjusted cut-off scores. METHOD: 30 incident ALS cases were assessed on both, ECAS and neuropsychological battery. Age and education adjusted cut-off scores were created from a sample of 82 healthy controls. RESULTS: ECAS composite scores (Total, ALS Specific and Non-Specific) were highly correlated with battery composite scores. High correlations were also observed between ECAS and full battery cognitive domains and subtests. The ECAS Total, ALS Specific and Non-Specific scores were highly sensitive to cognitive impairment. ECAS ALS-Specific cognitive domains also evidenced high sensitivity. Individual subtest sensitivity was medium to low, suggesting that caution should be used when interpreting these scores. Low positive predictive values indicated the presence of false positives. CONCLUSIONS: 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.

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.

Health and social care costs of managing amyotrophic lateral sclerosis (ALS): an Irish perspective.

The aim of this study was to quantify the health and social care costs associated with managing amyotrophic lateral sclerosis (ALS) in Ireland. Resource use of a representative group of deceased ALS patients attending a multidisciplinary ALS clinic was identified from a retrospective chart review and telephone interview with the main caregiver. Unit cost estimates were applied to each resource to identify the cost per patient. Cost drivers were identified using multivariate linear regression. Results showed that from time of diagnosis to death, the cost per month was €1795, 21% of which was attributable to costs associated with the multidisciplinary clinic, 72% to community based care and 7% to aids and appliances. Higher monthly cost was associated with shorter survival and use of gastrostomy and non-invasive ventilation. In conclusion, ALS imposes a significant cost burden on the health services. More work is required to quantify the costs in other sectors, including informal care and productivity losses.