The Association Between Maladaptive Metacognitive Beliefs and Emotional Distress in People Living With Amyotrophic Lateral Sclerosis

ObjectiveApproximately half of all people living with amyotrophic lateral sclerosis (ALS) experience persistent or recurrent emotional distress, yet little is known about the psychological processes that maintain emotional distress in this population. The self-regulatory executive functioning (S-REF) model specifies that maladaptive metacognitive beliefs and processes are central to the development and maintenance of emotional distress. This study explored whether maladaptive metacognitive beliefs are associated with emotional distress after controlling for demographic factors, time since diagnosis, and current level of physical functioning.DesignIn a cross-sectional design, 75 adults with a diagnosis of ALS completed self-report questionnaires. Participants had a mean age of 60.40 years, mean duration of symptoms 63.92 months, and male:female gender ratio of 14:11.Main Outcome MeasuresQuestionnaires assessed emotional distress (HADS, adapted for ALS), physical functioning (ALSFRS-R), repetitive negative thinking (RTQ-10), metacognitive beliefs (MCQ-30), and demographic factors.ResultsMaladaptive metacognitive beliefs explained additional variance in emotional distress after controlling for age, gender, time since diagnosis, physical functioning, and repetitive negative thinking. Repetitive negative thinking partially mediated the relationships between positive and negative metacognitive beliefs and emotional distress.ConclusionsThese data support the utility of the metacognitive model in understanding emotional distress in people with ALS. Examination of the temporal relationship between maladaptive metacognitive beliefs and emotional distress in people living with ALS may help to guide the development of therapeutic approaches.</jats:sec

Acceptance and Commitment Therapy plus usual care for improving quality of life in people with motor neuron disease (COMMEND) : a multicentre, parallel, randomised controlled trial in the UK

Background Motor neuron disease is a progressive, fatal neurodegenerative disease for which there is no cure. Acceptance and Commitment Therapy (ACT) is a psychological therapy incorporating acceptance, mindfulness, and behaviour change techniques. We aimed to evaluate the effectiveness of ACT plus usual care, compared with usual care alone, for improving quality of life in people with motor neuron disease. Methods We conducted a parallel, multicentre, two-arm randomised controlled trial in 16 UK motor neuron disease care centres or clinics. Eligible participants were aged 18 years or older with a diagnosis of definite or laboratory-supported probable, clinically probable, or possible familial or sporadic amyotrophic lateral sclerosis; progressive muscular atrophy; or primary lateral sclerosis; which met the World Federation of Neurology's El Escorial diagnostic criteria. Participants were randomly assigned (1:1) to receive up to eight sessions of ACT adapted for people with motor neuron disease plus usual care or usual care alone by a web-based system, stratified by site. Participants were followed up at 6 months and 9 months post-randomisation. Outcome assessors and trial statisticians were masked to treatment allocation. The primary outcome was quality of life using the McGill Quality of Life Questionnaire-Revised (MQOL-R) at 6 months post-randomisation. Primary analyses were multi-level modelling and modified intention to treat among participants with available data. This trial was pre-registered with the ISRCTN Registry (ISRCTN12655391). Findings Between Sept 18, 2019, and Aug 31, 2022, 435 people with motor neuron disease were approached for the study, of whom 206 (47%) were assessed for eligibility, and 191 were recruited. 97 (51%) participants were randomly assigned to ACT plus usual care and 94 (49%) were assigned to usual care alone. 80 (42%) of 191 participants were female and 111 (58%) were male, and the mean age was 63·1 years (SD 11·0). 155 (81%) participants had primary outcome data at 6 months post-randomisation. After controlling for baseline scores, age, sex, and therapist clustering, ACT plus usual care was superior to usual care alone for quality of life at 6 months (adjusted mean difference on the MQOL-R of 0·66 [95% CI 0·22–1·10]; d=0·46 [0·16–0·77]; p=0·0031). Moderate effect sizes were clinically meaningful. 75 adverse events were reported, 38 of which were serious, but no adverse events were deemed to be associated with the intervention. Interpretation ACT plus usual care is clinically effective for maintaining or improving quality of life in people with motor neuron disease. As further evidence emerges confirming these findings, health-care providers should consider how access to ACT, adapted for the specific needs of people with motor neuron disease, could be provided within motor neuron disease clinical services

Predictors of distress in amyotrophic lateral sclerosis: A systematic review

Objective Understanding of the factors that predict emotional distress in Amyotrophic Lateral Sclerosis (ALS) is limited. This systematic review critically appraised and synthesised the findings of longitudinal studies into baseline clinical, demographic, social, and psychological predictors of later emotional distress in people with ALS. Methods Medline, PsycINFO and CINAHL Plus were searched for relevant published literature from their inception until April 2018. Inclusion criteria were prospective or retrospective peer-reviewed studies, written in English, assessing baseline clinical, demographic, psychological or social predictors of emotional distress or psychological quality of life ≥1 month later in adults with ALS. Results Eight studies were included in this review. Demographic or clinical factors were investigated in seven studies, social factors in two studies, and psychological factors in three studies. There was scarce evidence that any baseline demographic or clinical factors predicted emotional distress. The only factor that consistently predicted emotional distress across multiple studies was lower social support. Mindfulness and psychological quality of life each predicted emotional distress but were only evaluated in single studies. Conclusions Further research is needed to investigate the mechanisms underlying emotional distress, particularly modifiable factors such as psychological factors, to reduce emotional distress in people with ALS

Pharmacological interventions for epilepsy in people with intellectual disabilities

Background The prevalence of epilepsy among people with intellectual disabilities is much higher than in the general population. Seizures in this population are often complex and refractory to treatment and antiepileptic medication may have a profound effect upon behaviour (Kerr 1997). This is an updated version of a Cochrane Review first published in Issue 3, 2007. Objectives To assess the data available from randomised controlled trials (RCTs) of the efficacy of antiepileptic drug (AED) interventions in people with epilepsy and intellectual disabilities. Search methods For the latest update of this review, we searched the Cochrane Epilepsy Group Specialised Register (2 September 2014), the Cochrane Central Register of Controlled Trials (CENTRAL) via the Cochrane Register of Studies Online (CRSO) (2 September 2014), MEDLINE (Ovid, 1946 to 3 September 2014) and PsycINFO (EBSCOhost, 1887 to 3 September 2014). Selection criteria Randomised and quasi-randomised controlled trials (RCTs) of pharmacological interventions for people with epilepsy and a learning disability. Data collection and analysis Two review authors independently assessed trial quality and extracted data. We contacted study authors for additional information. We assessed epilepsy/seizure, behavioural and cognitive outcomes, as well as quality of life and adverse effects. Main results We included 14 RCTs (1116 participants) in the present review. Data were heterogenous and a descriptive analysis is presented. In the majority of cases where antiepileptic drugs (AEDs) were trialled in this population, we found moderate reductions in seizure frequency in that there was a significantly higher rate of responders (reduction of 50% or more) in the treatment group compared with the placebo group, with some studies reporting a higher incidence of seizure freedom in the treatment group. In general, AEDs that are proven to be effective in the general epilepsy population are also effective for refractory epilepsy in people with intellectual disability. It is not possible to comment on the relative efficacy of medications, making clinical decisions difficult. In trial settings patients continued on treatment in the majority of cases. Placebo groups often experienced fewer adverse events. Where adverse events were experienced they appeared similar to those in the general population. The methods by which adverse events were recorded and reported appeared to be inconsistent, resulting in very large variation between studies. This is problematic as clinically relevant interpretation of these findings is limited. The quality of evidence provided in the present review is low to moderate. Additionally the majority of studies lacked or used non-reliable measures of behavioural exacerbation. However, where measured, little obvious impact on behaviour was seen in terms of behaviour disorder. Authors' conclusions This review broadly supports the use of AEDs to reduce seizure frequency in people with refractory epilepsy and intellectual disability. The evidence suggests that adverse events are similar to those in the general population and that behavioural adverse events leading to discontinuation are rare; however, other adverse effects are under-researched

Visual Hallucinations in Eye Disease and Lewy Body Disease

Objective: Visual hallucinations (VH) most commonly occur in eye disease (ED), Parkinson’s disease (PD), and Lewy body dementia (LBD). The phenomenology of VH is likely to carry important information about the brain areas within the visual system generating them. Methods: Data from five controlled cross-sectional VH studies (164 controls, 135 ED, 156 PD, 79 (PDD 48 + DLB 31) LBD) were combined and analysed. The prevalence, phenomenology, frequency, duration, and contents of VH were compared across diseases and gender. Results: Simple VH were most common in ED patients (ED 65% vs. LBD 22% vs. PD 9%, Chi-square [χ2] test: χ2=31.43, df=2, p<0.001), whilst complex VH were more common in LBD (LBD 76% vs. ED 38%, vs PD 28%, Chi-square test: χ2=96.80, df=2, p<0.001). The phenomenology of complex VH was different across diseases and gender. ED patients reported more “flowers” (ED 21% vs. LBD 6% vs. PD 0%, Chi-square test: χ2=10.04, df=2, p=0.005) and “body parts” (ED 40% vs. LBD 17% vs. PD 13%, Chi-square test: χ2=11.14, df=2, p=0.004); in contrast LBD patients reported “people” (LBD 85% vs. ED 67% vs. PD 63%, Chi-square test: χ2=6.20, df=2, p=0.045) and “animals/insects” (LBD 50% vs. PD 42% vs. ED 21%, Chi-square test: χ2=9.76, df=2, p=0.008). Males reported more “machines” (13 % vs. 2%, Chi-square test: χ2=6.94, df=1, p=0.008), whilst females reported more “family members/children” (48% vs. 29%, Chi-square test: χ2=5.10, df=1, p=0.024). Conclusions: The phenomenology of VH is likely related to disease specific dysfunctions within the visual system and to past, personal experiences