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. FUNDING: National Institute for Health and Care Research Health Technology Assessment and Motor Neurone Disease Association.

Measuring Change over Time on Cognitive Screening Measures: An Evaluation of the Psychometric Properties of ACE-III, CBI-R, and EMQ for the Purpose of Dementia Screening.

INTRODUCTION: Cognitive screening measures are an established part of a dementia assessment and often include measures of subjective difficulties, e.g., Everyday Memory Questionnaire (EMQ), informant-rated difficulty, Cambridge Behavioural Inventory - Revised (CBI-R), and objective cognitive assessments like Addenbrooke's Cognitive Examination (ACE-III). While these measures have validity for the purpose of diagnosing dementia, in clinical practice they are often used outside of their evidence base for the purpose of cognitive re-assessment. The current study sought to evaluate the psychometric properties for the repeated use of these assessments. METHODS: This study used a longitudinal design, which included 49 healthy controls, 33 people with subjective memory difficulties, and 10 people with Alzheimer's disease (AD) being assessed twice, with approximately a 1-year interval between assessments. RESULTS: The EMQ has adequate re-test reliability (r = 0.78), but people with an AD diagnosis rated their memory as better than those with SMD, making it unsuitable as a measure. The CBI-R had moderate re-test reliability (r = 0.62). However, deterioration on the CBI-R was not useful for diagnosing AD. The ACE-III has high re-test reliability (r = 0.89). A change of five was associated with reasonable classification accuracy for identifying AD and achieved statistical significance. CONCLUSION: Using a 1-year interval, of the three measures used in this study, only the ACE-III total score may be a useful measure of change over time, although it should be applied cautiously due to the lack of representativeness of the sample, and change scores should always be triangulated with other forms of evidence of deterioration.

Specialist healthcare services for concussion/mild traumatic brain injury in England: a consensus statement using modified Delphi methodology.

OBJECTIVE: To establish a consensus on the structure and process of healthcare services for patients with concussion in England to facilitate better healthcare quality and patient outcome. DESIGN: This consensus study followed the modified Delphi methodology with five phases: participant identification, item development, two rounds of voting and a meeting to finalise the consensus statements. The predefined threshold for agreement was set at ≥70%. SETTING: Specialist outpatient services. PARTICIPANTS: Members of the UK Head Injury Network were invited to participate. The network consists of clinical specialists in head injury practising in emergency medicine, neurology, neuropsychology, neurosurgery, paediatric medicine, rehabilitation medicine and sports and exercise medicine in England. PRIMARY OUTCOME MEASURE: A consensus statement on the structure and process of specialist outpatient care for patients with concussion in England. RESULTS: 55 items were voted on in the first round. 29 items were removed following the first voting round and 3 items were removed following the second voting round. Items were modified where appropriate. A final 18 statements reached consensus covering 3 main topics in specialist healthcare services for concussion; care pathway to structured follow-up, prognosis and measures of recovery, and provision of outpatient clinics. CONCLUSIONS: This work presents statements on how the healthcare services for patients with concussion in England could be redesigned to meet their health needs. Future work will seek to implement these into the clinical pathway.

A pilot examination of the validity of stylus and finger drawing on visuomotor-mediated tests on ACEmobile.

INTRODUCTION: Cognitive assessments, such as the Addenbrooke's Cognitive Examination (ACE-III) and Montreal Cognitive Assessment (MoCA), have been modified for administration using tablet computers. While this offers important advantages for practice, it may also threaten the test validity. The current study sought to test whether administering visuospatial and writing tests using a tablet (finger or stylus drawing), would demonstrate equivalence to traditional pencil and paper administration on ACEmobile. METHOD: This study recruited 26 participants with Alzheimer's disease and 23 healthy older adults. Most participants had low familiarity with using a tablet computer. Participants completed ACEmobile in its entirety, after which they repeated the infinity loops, cube, and clock drawing and sentence writing tests by drawing with a stylus and their finger onto an iPad. Performance on the drawing and writing tests using a stylus, finger, and pencil were compared. RESULTS: Statistically significant differences were observed between the finger and pencil administration on the ACEmobile, with participants performing worse on the finger drawing trials. Differences in scores were most apparent on the sentence writing task. In contrast, no statistical differences were observed between the pencil and stylus administration. DISCUSSION: The findings of this pilot study have important implications for clinical neuropsychology and demonstrate that administering ACEmobile drawing tests with finger drawing is invalid. However, due to the small sample size, a lack of counterbalancing and the narrow range of scores of the dependent variable, we are unable to confidently interpret the validity of stylus drawing. This is an important consideration for future research.

Acceptance and Commitment Therapy for people living with motor neuron disease: an uncontrolled feasibility study.

BACKGROUND: Motor neuron disease (MND) is a fatal, progressive neurodegenerative disease that causes progressive weakening and wasting of limb, bulbar, thoracic and abdominal muscles. Clear evidence-based guidance on how psychological distress should be managed in people living with MND (plwMND) is lacking. Acceptance and Commitment Therapy (ACT) is a form of psychological therapy that may be particularly suitable for this population. However, to the authors' knowledge, no study to date has evaluated ACT for plwMND. Consequently, the primary aim of this uncontrolled feasibility study was to examine the feasibility and acceptability of ACT for improving the psychological health of plwMND. METHODS: PlwMND aged ≥ 18 years were recruited from 10 UK MND Care Centres/Clinics. Participants received up to 8 one-to-one ACT sessions, developed specifically for plwMND, plus usual care. Co-primary feasibility and acceptability outcomes were uptake (≥ 80% of the target sample [N = 28] recruited) and initial engagement with the intervention (≥ 70% completing ≥ 2 sessions). Secondary outcomes included measures of quality of life, anxiety, depression, disease-related functioning, health status and psychological flexibility in plwMND and quality of life and burden in caregivers. Outcomes were assessed at baseline and 6 months. RESULTS: Both a priori indicators of success were met: 29 plwMND (104%) were recruited and 76% (22/29) attended ≥ 2 sessions. Attrition at 6-months was higher than anticipated (8/29, 28%), but only two dropouts were due to lack of acceptability of the intervention. Acceptability was further supported by good satisfaction with therapy and session attendance. Data were possibly suggestive of small improvements in anxiety and psychological quality of life from baseline to 6 months in plwMND, despite a small but expected deterioration in disease-related functioning and health status. CONCLUSIONS: There was good evidence of acceptability and feasibility. Limitations included the lack of a control group and small sample size, which complicate interpretation of findings. A fully powered RCT to evaluate the clinical and cost-effectiveness of ACT for plwMND is underway. TRIAL REGISTRATION: The study was pre-registered with the ISRCTN Registry (ISRCTN12655391).

The Utility of Visual and Spatial Perception Tests in Alzheimer's Disease: A Systematic Review.

INTRODUCTION: Visual and spatial perception (VSP) are cognitive domains frequently assessed in the screening and neuropsychological assessment of dementia. Evidence suggests that VSP impairment is common in the early stages of Alzheimer's disease (AD). Despite this evidence, the ability of VSP tests to discriminate between healthy older adults and people with AD remains mixed. The purpose of this literature review was to employ a systematic search strategy to identify empirical evidence supporting the diagnostic utility of VSP tests which may be used in the screening and diagnosis of AD. METHODS: Specified criteria were used to perform a systematic literature search of the PsycINFO and PubMed databases with no date restrictions. Relevant data from the selected studies were extracted, and a published appraisal tool (the Quality Assessment of Diagnostic Accuracy Studies-2) was used to evaluate methodological quality. RESULTS: Of the 144 articles returned, six studies and 11 VSP tests met review inclusion criteria. Four tests demonstrated both sensitivity and specificity values above 80%. A computerised 3D Visual Task demonstrated the highest sensitivity and specificity values (90% and 95%, respectively). The quality of the identified studies was considered to be satisfactory. Identified limitations and the implications of issues relating to study methodology are discussed, and recommendations for future research are suggested. CONCLUSION: The evidence from this review suggests that certain tests of VSP may be a useful addition to the routine screening of AD.

Oxford cognitive screen: A critical review and independent psychometric evaluation.

The Oxford cognitive screen (OCS) is a stroke-specific cognitive screening assessment. Although the test developers have provided psychometric information for the assessment, the OCS has received minimal external scrutiny, with which to triangulate the underpinning psychometrics. The purpose of this study is to provide a critical review and independent validation of the OCS. This study analysed data from an anonymised clinical database, which consisted of 316 patients who were assessed using the OCS on an Acute Stroke Unit. The rates of impairment on tests of memory and receptive communication were lower than expectation, suggesting that these subtests may be relatively insensitive. Patients with aphasia were more likely to be unable to categorised as impaired on non-language tests, suggesting that they remain sensitive to language processing or non-dominant hand usage. Some of the subtests of the OCS achieve high retest reliability, which makes them good candidates for measuring cognitive change over time. Although the OCS has many advantages, it is also important to adequately consider its limitations, that is insensitivity to memory problems, the potential confounding impact of non-dominant hand usage, and the potential that some tests may sample overall cognitive ability instead of domain-specific functioning.

Evaluating the GAD-2 to screen for post-stroke anxiety on an acute stroke unit.

Post stroke anxiety is associated with reduced quality of life, worse rehabilitation outcomes and poor social functioning in stroke survivors. Despite its clinical relevance, there are a lack of screening tools to identify post-stroke anxiety. The Hospital Anxiety and Depression Scale (HADS-A) and Geriatric Anxiety Inventory (GAI) are the only self-report screening test for anxiety validated in stroke patients. However, both of these measures have limitations associated with their clinical utility, length of time to administer and cost to purchase. This study aimed to assess the classiciation accuracy of a freely available and shorter alternative, the Generalized Anxiety Disorder 2-item (GAD-2) for use in the acute stroke setting. This study used retrospective analysis of a sequential sample in a clinical database of mood and cognitive scores. Patients' scores on the GAD-2 and HADS-A were compared. The GAD-2 demonstrated strong convergent validity with the HADS-A and good specificity and sensitivity for clinical anxiety. In conclusion, the GAD-2 may be a useful screening assessment of post-stroke anxiety in the acute setting; however, it should be used with due consideration of the methodological limitations of this study.

Improving the quality of cognitive screening assessments: ACEmobile, an iPad-based version of the Addenbrooke's Cognitive Examination-III.

INTRODUCTION: Ensuring reliable administration and reporting of cognitive screening tests are fundamental in establishing good clinical practice and research. This study captured the rate and type of errors in clinical practice, using the Addenbrooke's Cognitive Examination-III (ACE-III), and then the reduction in error rate using a computerized alternative, the ACEmobile app. METHODS: In study 1, we evaluated ACE-III assessments completed in National Health Service (NHS) clinics (n = 87) for administrator error. In study 2, ACEmobile and ACE-III were then evaluated for their ability to capture accurate measurement. RESULTS: In study 1, 78% of clinically administered ACE-IIIs were either scored incorrectly or had arithmetical errors. In study 2, error rates seen in the ACE-III were reduced by 85%-93% using ACEmobile. DISCUSSION: Error rates are ubiquitous in routine clinical use of cognitive screening tests and the ACE-III. ACEmobile provides a framework for supporting reduced administration, scoring, and arithmetical error during cognitive screening.

Long-Term Follow-up of a Successfully Treated Case of Congenital Pyridoxine-Dependent Epilepsy.

Autosomal recessive disorders affecting pyridoxine (vitamin B6) metabolism are a rare but well-recognized cause of neonatal seizures. Antiquitin deficiency, caused by mutations in ALDH7A1, is a disorder of the lysine degradation pathway causing accumulation of an intermediate that complexes with pyridoxal phosphate. Reports of long-term follow-up of neonatal pyridoxine-dependent seizures (PDS) remain scarce and prognostic information is varied. We report a case of PDS in a 47-year-old lady who originally presented shortly after birth in 1964. Pyridoxine replacement was successful and diagnostic confirmation was obtained later in life, initially by biochemical analysis of serum pipecolic acid. Subsequently we organized genetic analysis of ALDH7A1, which revealed compound heterozygous mutations. To our knowledge, this represents the longest duration of follow-up published to date.

Behavioural management of migraine.

It is important to recognise that migraine is a 'biological' and not a 'psychological' entity. However, psychological factors can be involved in migraine in 4 different ways:- 1) Migraines can be triggered by psychological stressors; 2) Severe migraine can itself be a cause of significant psychological stress which can, in turn, exacerbate the problem; 3) Even if psychological stress is not significantly involved in the genesis of the headache, pain management techniques can help people cope with their pain more effectively; 4) Longitudinal data demonstrate a complex bidirectional association between mood disorders and migraine. Treatment of a co-existing mood disorder, for example with cognitive behavioural techniques, may therefore reduce the impact of migraine. It would thus appear logical to view medical and psychological approaches as potentially synergistic rather than mutually exclusive. Functional imaging indicates that cognition, emotions, and pain experiences change the way the brain processes pain inputs. This may provide a physiological rationale for psychological interventions in pain management. As most studies of psychological management of migraine have been relatively small and the approach often varies between clinicians, the magnitude of benefit, optimum method of delivery, and the length of intervention are uncertain.

Memory, metamemory and their dissociation in temporal lobe epilepsy.

Patients with temporal-lobe epilepsy (TLE) present with memory difficulties. The aim of the current study was to determine to what extent these difficulties could be related to a metamemory impairment. Fifteen patients with TLE and 15 matched healthy controls carried out a paired-associates learning task. Memory recall was measured at intervals of 30min and 4 weeks. We employed a combined Judgement-of-Learning (JOL) and Feeling-of-Knowing (FOK) task to investigate whether participants could monitor their memory successfully at both the item-by-item level and the global level. The results revealed a clear deficit of episodic memory in patients with epilepsy compared with controls, but metamemory in TLE patients was intact. Patients were able to monitor their memory successfully at the item-by-item level, and tended to be even more accurate than controls when making global judgements.