National audit of cognitive assessment in people with pwMND A national audit of cognitive assessment in people with motor neurone disease (pwMND) in Scotland.

Cognitive and behavioral abnormalities are recognized as an integral part of Motor Neurone Disease (MND) and occur at all stages of the disease. The early detection of cognitive and behavioral symptoms in MND is critical. Such symptoms are only reported when we explicitly ask, evaluate, document, and assess. In the National Institute for Health and Care Excellence (NICE) MND guideline (2016), formal cognitive and behavioral assessment is incorporated in MND management and is fundamental to providing appropriate care to pwMND. Cognition is explicitly stated in 14 separate recommendations in the guidelines. The NICE guidelines therefore constitute pre-defined standards which we audited. This audit highlights that health professionals increasingly recognize the significance of cognitive screening in MND and follow more structured approaches in implementing this compared to previous years.

The multiple faces of pain in motor neuron disease: a qualitative study to inform pain assessment and pain management.

Purpose: The aim was to explore personal experiences of pain in people with motor neuron disease.Materials and methods: Sixteen participants were individually interviewed on one occasion concerning their experiences of presentation, consequences, and management of pain. Qualitative content analysis with researcher triangulation was used to synthesize and interpret data.Results: Four themes emerged as the result of the analysis: (1) The multiple faces of pain, (2) The thin line between experience of pain and no pain, (3) The negative effects of pain on role functioning (4) Successful coping with pain requiring personal effort and competent engagement. The important findings were the experiences of unpredictability of pain breakthroughs, the efforts required to manage pain, consequences for activity and quality of life, and the suffering induced by diminishment and neglect of pain from both patients and staff.Conclusions: Pain in motor neuron disease seems to have certain and multiple characteristics, which is why there is a need to develop and implement pain assessment methods adapted to this population. Such methods may help make pain more predictable, and increase the possibilities to provide effective and individually tailored pain treatment.IMPLICATIONS FOR REHABILITATIONPain is a common, but often neglected, ailment in motor neuro disease, which deserves more attention from health care.Staff should provide information about the pain being possible to treat successfully with medication, by contrast to the possibility of curing the disease itself.Pain assessments should be implemented during the entire course of the disease, covering a time frame long enough to cover characteristic fluctuations of pain.Whenever possible, facilitate the performance of painful activities of daily living as much as possible to make room for engagement in other personally valued activities of importance for individual quality of life.

Prevalence of unmet needs for spasticity management in care home residents in the East Midlands, United Kingdom: a cross-sectional observational study.

OBJECTIVE: To establish the prevalence of unmet need for spasticity management in care home residents in two counties of the United Kingdom. DESIGN: Cross-sectional observational study with a six-month follow-up arm for participants with identified unmet needs. SETTING: 22 care homes in Derbyshire and Nottinghamshire. SUBJECTS: 60 care home residents with upper motor neuron syndrome-related spasticity. INTERVENTIONS: No intervention. When unmet needs around spasticity management were identified, the participant's general practitioner was advised of these in writing. MAIN MEASURES: Resistance to Passive Movement Scale to assess spasticity; recording of (a) the presence of factors which may aggravate spasticity, (b) potential complications of spasticity, (c) spasticity-related needs and (d) current interventions to manage spasticity. Two assessors judged the presence or absence of needs for spasticity management and whether these needs were met by current care. RESULTS: Out of 60 participants, 14 had no spasticity-related needs; 46 had spasticity-related needs; 11 had needs which were being met by current care and 35 participants had spasticity-related needs at baseline which were not being met by their current care. These were most frequently related to the risk of contracture development or problems with skin hygiene or integrity in the upper limb. In total, 6 participants had one or more pressure sores and 35 participants had one or more established joint contractures. A total of 31 participants were available for follow-up. Informing general practitioners of unmet needs resulted in no change to spasticity management in 23/31 cases. CONCLUSION: Care home residents in this study had high levels of unmet need for spasticity management.

Meaningful Assessment in Patients with Acquired Brain Injuries.

There are several key components to the meaningful and comprehensive assessment of patients with acquired brain injuries with respect to management of the upper motor neuron syndrome. Type of brain injury, trajectory of recovery, relevant concomitant complications, development of appropriate goals, and an understanding of resources available for patients are all factors to assess when developing a treatment plan. Using appropriate outcome measures will help monitor the efficacy of interventions and guide ongoing management of spasticity.

Muscle Overactivity in the Upper Motor Neuron Syndrome: Assessment and Problem Solving for Complex Cases: the Role of Physical and Occupational Therapy.

The role of the physical or occupational therapist in addressing muscle hyperexcitability is to carefully assess the implications that the abnormal tone has on function, especially active movement patterns. A thorough evaluation that includes neurologic and nonneurologic attributes allows the clinician to determine the most efficacious treatment interventions, especially when considering severity and chronicity of deficits. A holistic assessment that includes patient factors and resources guides the clinician's plan of care to allow for optimal functional outcomes.

The experience of informal caregivers of patients with motor neurone disease: A thematic synthesis.

ABSTRACTObjective:Research is required in order to illustrate and detail the experiences of informal caregivers of patients with motor neurone disease (pwMND) to further advance the research base and to inform the development of future support structures and services. Due to the heterogeneous nature of caregiving for pwMND, one way in which this can be achieved is through a qualitative review. A qualitative thematic analysis of existing qualitative studies has not, to the best of the authors' knowledge, been previously undertaken. Thus, the present synthesis aims to identify caregivers' experiences and to suggest factors that contribute to these experiences in order to fulfill the required research needs. METHOD: A thematic synthesis of qualitative literature was conducted. AMED, Medline, SPORTDiscus, CINAHL, and PubMed were electronically searched from inception until September of 2015. Studies were eligible if they included qualitative literature reporting on firsthand experience of informal caregivers of patients with MND, were published in English, and contained verbatim quotations. Critical appraisal was undertaken using a 13-item consolidated criteria for reporting qualitative studies (COREQ) checklist. RESULTS: A total of 10 studies met the inclusion criteria, with 148 (50 male) current or previous informal caregivers of pwMND identified. Critical appraisal demonstrated that study design and reflexivity were underreported. The synthesis derived three themes: (1) loss of control, (2) inability to choose, and (3) isolation. SIGNIFICANCE OF RESULTS: The synthesis highlighted the factors that contribute to both positive and negative caregiving experiences. Through these experiences, such suggestions for service provision as improving communication with healthcare professionals and having a single point of contact emerged. However, the outcome of such suggestions on the experience of caregivers is beyond the scope of our synthesis, so that further research is required.

Supporting wellbeing in motor neurone disease for patients, carers, social networks, and health professionals: A scoping review and synthesis.

ABSTRACTObjective:Disease management in motor neurone disease (MND) is focused on preserving quality of life. However, the emphasis has so far been on physical symptoms and functioning and not psychosocial wellbeing. MND affects the wellbeing of carers, of family and social network members, and of healthcare providers, as well as of the patients. We therefore aimed to assess and synthesize the knowledge about maximizing MND-related psychosocial wellbeing across all these groups. METHOD: We used a systematic search and selection process to assess the scope of the literature along with a narrative synthesis of recent high-quality reviews. RESULTS: The original studies were mainly observational studies of patients and, to a lesser extent, of carers. There were few interventional studies, mainly of patients. There were very few studies of any type on wellbeing in their wider social network or in healthcare professionals. All the review literature looked at MND patient or carer wellbeing, with some covering both. No reviews were found of wellbeing in other family members, patients' social networks, or their healthcare professionals. The reviews demonstrated wellbeing problems for patients linked to psychosocial issues. Carer wellbeing is also compromised. Psychotherapies, social supports, improved decision supports, and changes to healthcare delivery are among the suggested strategies for improved patient and carer wellbeing, but no proven interventions were identified for either. Early access to palliative care, also not well-tested but recommended, is poorly implemented. SIGNIFICANCE OF RESULTS: Work on interventions to deal with well-established wellbeing problems for patients and carers is now a research priority. Explicit use of current methods for patient and public involvement and for design and testing of interventions provide a toolkit for this research. Observational research is needed in other groups. There is a potential in considering needs across patients' social networks rather than looking individually at particular groups.

Assessment, treatment goals and interventions for oedema/lymphoedema in palliative care.

BACKGROUND: Little is known about the treatment of oedema in palliative care patients. AIM: To outline the assessment, goals, and interventions for patients with oedema. METHOD: A 6-month chart review of a specialist physiotherapy-led oedema service was carried out. RESULTS: Of the sample group (n=63), 28.6% of patients had lymphoedema, 15.9% had non-lymphatic oedema, 46% had mixed oedema and 9.8% had lymphorrhoea; 58 patients (92%) had cancer. The most common interventions were providing education (100%, n=63), using compression garments (58%, n=37), bandaging (51%, n=32), exercise (38%, n=24), lymphatic massage and kinesio-taping (35%, n=22 each). Treatment regime differed depending on the type of oedema present. Treatment goals were pragmatic: the most common were to maintain skin quality (54%, n=34), reduce limb volume (52%, n=32), and improve quality of life (48%, n=30). CONCLUSION: Palliative care oedema can be treated using manual methods, including compression and massage. Goals differ from other oedema populations. Research is hampered by lack of suitable measures to record skin changes and quality of life.

Factors to consider for motor neurone disease carer intervention research: A narrative literature review.

OBJECTIVE: The experience of caregiving in the context of motor neurone disease (MND) is extremely challenging. Over the past 15 years, quantitative and qualitative studies have delineated the psychosocial aspects of this experience, exploring its impact on caregivers' quality of life, rates of depression, distress, anxiety, and burden. Our paper aimed to provide an overview of the lived experience of MND caregivers, identifying the variables that can influence MND caregiver functioning that are relevant to the development of an intervention. METHOD: A narrative review was conducted, synthesizing the findings of literature retrieved from 2000 to early 2016. RESULTS: A total of 37 articles were included in the review. The articles varied considerably in terms of methodology and quality. The main influential aspects reported and identified were factors pertaining to the patient, factors intrinsic to the caregiver, relationship factors, and social support factors. SIGNIFICANCE OF RESULTS: There is evidence to support the fact that caregivers have poorer outcomes when they care for patients with a more severe clinical profile, poorer emotional health or neurobehavioral concerns, or when the caregivers themselves struggle with adaptive problem-solving and coping skills. The availability and use of social support are also likely to be important for caregiver psychosocial outcomes. Further investigation is required to clarify the influence of changes in the relationship with the patient. Significant factors affecting the caregiver experience are considered in relation to their amenability to psychosocial intervention. Recommendations are made regarding the optimal features of future psychosocial intervention research.

Identifying and addressing the support needs of family caregivers of people with motor neurone disease using the Carer Support Needs Assessment Tool.

OBJECTIVE: Family caregivers of people with motor neurone disease (MND) experience adverse health outcomes as a result of their caregiving experience. This may be alleviated if their support needs are identified and addressed in a systematic and timely manner. The objective of the present study was to assess the feasibility and relevance of the Carer Support Needs Assessment Tool (CSNAT) in home-based care during the period of caregiving from the perspectives of the family caregivers of people with MND and their service providers. METHOD: The study was conducted during 2014 in Western Australia. Some 30 family caregivers and 4 care advisors participated in trialing the CSNAT intervention, which involved two visits from care advisors (6-8 weeks apart) to identify and address support needs. The feedback from family caregivers was obtained via telephone interviews and that of care advisors via a self-administered questionnaire. RESULTS: A total of 24 caregivers completed the study (80% completion rate) and identified the highest support priorities as "knowing what to expect in the future," "knowing who to contact if concerned," and "equipment to help care." The majority found that this assessment process adequately addressed their needs and gave them a sense of validation, reassurance, and empowerment. Care advisors advocated the CSNAT approach as an improvement over standard practice, allowing them to more clearly assess needs, to offer a more structured follow-up, and to focus on the caregiver and family. SIGNIFICANCE OF RESULTS: The CSNAT approach for identifying and addressing family caregivers' support needs was found to be relevant and feasible by MND family caregivers and care advisors. The tool provided a formal structure to facilitate discussions with family caregivers and thus enable needs to be addressed. Such discussions can also inform an evidence base for the ongoing development of services, ensuring that new and improved services are designed to meet the explicit needs of the family caregivers of people with a motor neurone disease.

Lived-through past, experienced present, anticipated future: Understanding "existential loss" in the context of life-limiting illness.

OBJECTIVE: Motor Neurone Disease (MND) is a rare, devastating neurodegenerative disease of middle/later life, usually presenting in the sixth and seventh decades (McDermot & Shaw, 2008). People have to wait many months to receive a diagnosis of MND (Donaghy et al., 2008), and during this period they have already experienced the degenerative nature that characterizes MND (Bolmsjö, 2001). However, information on the meaning of life with MND through time is limited. The aim of the present research was to answer the research question "What does it mean to be a person living through the illness trajectory of MND?" and to research the phenomenon of existence when given a diagnosis of MND and in the context of receiving healthcare. METHOD: Hermeneutic phenomenology, inspired by the philosophers Heidegger and Gadamer, informed the methodological approach employed, which asked people to tell their story from when they first thought something untoward was happening to them. The hermeneutic analysis involved a five-stage process in order to understand (interpret) the lifeworld 1 of four people diagnosed with MND, and a lifeworld perspective helped to make sense of the meaning of existence when given a terminal diagnosis of MND. RESULTS: The concept of "existential loss" identified in relation to MND was the loss of past ways of being-in-the-world, and the loss of embodiment, spatiality, and the future. SIGNIFICANCE OF RESULTS: The concept of existential loss requires closer attention by healthcare professionals from the time of diagnosis and on through the illness trajectory. The study findings are conceptualized into a framework, which when used as a clinical tool may prompt healthcare professionals to focus on their patients' existential loss and existential concerns. This research adds to the existing literature calling for a lifeworld approach to healthcare.

Diagnostic yield and cost-effectiveness of investigations in patients presenting with isolated lower motor neuron signs.

Our objective was to investigate the yield and cost-effectiveness of investigations and therapeutic trials of intravenous immunoglobulin (IVIg) in patients presenting with isolated lower motor neuron (LMN) signs. We performed a retrospective chart review of cases diagnosed between January 2007 and September 2013. Investigation results and their impact on outcome, and outcome of IVIg treatment trials were abstracted. Cost was calculated in Canadian dollars (C$). Fifty-nine of 333 patients presented with isolated LMN signs. The majority of patients (61%) evolved to amyotrophic lateral sclerosis (ALS) within 36 months of presentation, while 37.3% remained with progressive muscular atrophy (PMA) with mean follow-up 29.6 months. Of the 1210 tests performed, 4.9% were abnormal. The diagnosis was changed in only one patient where a muscle biopsy revealed a distal myopathy. Fourteen patients received therapeutic trials of IVIg to rule out an IVIg-responsive inflammatory motor neuropathy with no objective clinical benefit. Total group cost was C$630,484.72 (C$10,686.18/patient). IVIg represented 58.7% of total costs. In conclusion, extensive investigations and treatment trials of IVIg have low yield in the work-up of patients with isolated LMN signs and are not cost-effective when clinical features do not suggest an alternative diagnosis to PMA.

Oral health and dental treatment needs of people with motor neurone disease.

BACKGROUND: People with motor neurone disease (MND) may present with physical limitations impacting on oral health and access to oral health care. This study aimed to assess the oral health status and treatment needs of people with MND in Victoria, Australia. METHODS: Patients with advanced MND attending a multidisciplinary MND clinic in Melbourne were recruited. Data collection included self-reporting questionnaires on previous dental experience, current oral hygiene practices and current dietary habits, a medical questionnaire, and a clinical examination charting participants' dentition, restorations, caries and periodontal status, plaque and gingival indices, and assessment of oral health. RESULTS: Thirty-three participants took part in the study with eight self-reporting regular dental visits. No participant exhibited probing depths of more than 3 mm. Ten out of 27 dentate participants required extractions and restorations for retained roots and caries, while three presented with non-carious cavities, lost restorations and fractured cusps. Oral health status was not affected by MND presentation in these participants. CONCLUSIONS: The study found that oral health was not affected by advanced MND. Participants' and clinical teams' motivation towards oral health care may have contributed to oral health. The dental profession should be involved as part of the multidisciplinary effort towards ongoing care.

Evidence-based review and assessment of botulinum neurotoxin for the treatment of adult spasticity in the upper motor neuron syndrome.

Botulinum neurotoxin (BoNT) can be injected to achieve therapeutic benefit across a large range of clinical conditions. To assess the efficacy and safety of BoNT injections for the treatment of spasticity associated with the upper motor neuron syndrome (UMNS), an expert panel reviewed evidence from the published literature. Data sources included English-language studies identified via MEDLINE, EMBASE, CINAHL, Current Contents, and the Cochrane Central Register of Controlled Trials. Evidence tables generated in the 2008 Report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology (AAN) review of the use of BoNT for autonomic disorders were also reviewed and updated. The panel evaluated evidence at several levels, supporting BoNT as a class, the serotypes BoNT-A and BoNT-B, as well as the four individual commercially available formulations: abobotulinumtoxinA (A/Abo), onabotulinumtoxinA (A/Ona), incobotulinumtoxinA (A/Inco), and rimabotulinumtoxinB (B/Rima). The panel ultimately made recommendations on the effectiveness of BoNT for the management of spasticity, based upon the strength of clinical evidence and following the AAN classification scale. While the prior report by the AAN provided recommendations for the use of BoNT as a class of drug, this report provides more detail and includes recommendations for the individual formulations. For the treatment of upper limb spasticity, the evidence supported a Level A recommendation for BoNT-A, A/Abo, and A/Ona, with a Level B recommendation for A/Inco; there was insufficient evidence to support a recommendation for B/Rima. For lower limb spasticity, there was sufficient clinical evidence to support a Level A recommendation for A/Ona individually and BoNT-A in aggregate; the clinical evidence for A/Abo supported a Level C recommendation; and there was insufficient information to recommend A/Inco and B/Rima (Level U). There is a need for further comparative effectiveness studies of the available BoNT formulations for the management of spasticity.

Assessment of swallowing in motor neuron disease and Asidan/SCA36 patients with new methods.

BACKGROUND: We report on a unique complication of cerebellar ataxia and motor neuron disease named Asidan/SCA36 with a high frequency of tongue atrophy. We aimed to elucidate dysphagia in amyotrophic lateral sclerosis (ALS) and spinal, bulbar muscular atrophy (SBMA), and Asidan/SCA36 patients with new methods. METHODS: Patients diagnosed with ALS (n=20), SBMA (n=6), and Asidan (n=12) were included. A videofluoroscopic swallow study (VFS), an assessment of maximal tongue pressure (MTP), and impedance pharyngography (IPG) were applied. RESULTS: The frequencies of VFS abnormalities were 70%, 50%, and 33% in ALS, SBMA, and Asidan/SCA36, respectively. Compared with control subjects (31.6 ± 6.3 kPa, mean ± SD), MTP was significantly decreased in ALS patients and SBMA patients, but was relatively preserved in Asidan patients. ALS patients performed more swallowing actions (Ns) detected by IPG than did control subjects, but SBMA and Asidan/SCA36 patients performed similar Ns to control subjects. CONCLUSIONS: VFS showed a higher frequency of swallowing abnormalities in ALS patients. MTP and IPG measurements showed the most severe involvement in ALS patients and a relatively preserved swallowing function in SBMA and Asidan/SCA36 patients.

Survey of UK speech and language therapists' assessment and treatment practices for people with progressive dysarthria.

BACKGROUND: Dysarthria knowledge is predominantly impairment-based. As a result, speech and language therapists (SLTs) have traditionally adopted impairment-focused management practices. However, guidance for best practice suggests that SLTs should consider the client holistically, including the impact of dysarthria beyond the impairment. AIMS: To investigate the current assessment and treatment practices used by UK SLTs with clients with progressive dysarthria and to identify whether these satisfy the needs of SLTs in their everyday practice. To investigate the extent to which they consider oromotor abilities, intelligibility, functional communication, participation and interaction to be important regarding assessment and treatment decisions. To explore whether management decisions are affected by level of clinical experience or settings in which SLTs work. METHODS & PROCEDURES: An online survey of UK SLTs working with adults with progressive dysarthria. OUTCOMES & RESULTS: A total of 119 SLTs completed the survey. Respondents considered that targeting the levels of impairment, activity and participation are important in the management of clients with progressive dysarthria, as recommended by clinical guidelines and recent research. However a particularly high proportion of respondents reported the use of impairment-based assessments. Respondents reported lacking the necessary tools to target interaction in assessment and intervention. The intervention that respondents use with clients varies according to the progressive disorder and dysarthria severity. There is evidence for a trend that less experienced SLTs and those working predominantly in hospital-based settings focus on the impairment, whereas more SLTs with more experience and those based in predominantly community-based settings look beyond the impairment. CONCLUSIONS & IMPLICATIONS: The values held by SLTs match guideline recommendations for best practice, however the clinical reality is that the assessment of progressive dysarthria remains predominantly impairment-focused. New tools need to be developed and integrated into practice to target interaction in assessment and intervention, to reduce the gap between best practice recommendations and clinical reality. Ongoing research into the effectiveness of SLT intervention with clients with progressive dysarthria is required to guide clinical management decisions.

Clinical assessment and management of spasticity: a review.

Spasticity is a sign of upper motor neurone lesion, which can be located in the cerebrum or the spinal cord, and be caused by stroke, multiple sclerosis, spinal cord injury, brain injury, cerebral paresis, or other neurological conditions. Management is dependent on clinical assessment. Positive and negative effects of spasticity should be considered. Ashworth score and the modified Ashworth score are the most used scales for assessment of spasticity. These and other spasticity scales are based on assessment of resistance during passive movement. The main goal of management is functional improvement. A novel 100-point score to assess disability, function related to spasticity (Rekand disability and spasticity score) is proposed. Management of spasticity should be multimodal and should always include physiotherapy or exercise. Oral medications such as baclofen and tizanidine have limited efficacy and considerable side effects, but are easiest to use. Botulinum toxin combined with physiotherapy and/or orthopaedic surgery is effective treatment of localized spasticity. Treatment with intrathecal baclofen via programmable implanted pump is effective in generalized spasticity, particularly in the lower extremities. Neurosurgical and orthopaedic procedures may be considered in intractable cases.

The effect of stance control orthoses on gait characteristics and energy expenditure in knee-ankle-foot orthosis users.

Stance Control knee-ankle foot orthoses (SCO) differ from their traditional locked knee counterparts by allowing free knee flexion during swing while providing stability during stance. It is widely accepted that free knee flexion during swing normalizes gait and therefore improves walking speed and reduces the energy requirements of walking. Limited research has been carried out to evaluate the benefits of SCOs when compared to locked knee-ankle foot orthoses (KAFOs). The purpose of this study was to evaluate the effectiveness of SCOs used for patients with lower limb pathology. Energy expenditure and walking velocity were measured in 10 subjects using an orthosis incorporating a Horton Stance Control knee joint. A GAITRite walkway was used to measure temporospatial gait characteristics. A Cosmed K4b2 portable metabolic system was used to measure energy expenditure and heart rate during walking. Two conditions were tested: Walking with stance control active (stance control) and walking with the knee joint locked. Ten subjects completed the GAITRite testing; nine subjects completed the Cosmed testing. Walking velocity was significantly increased in the stance control condition (p < 0.001). There was no difference in the energy cost of walking (p = 0.515) or physiological cost index (PCI) (p = 0.093) between conditions. This study supports previous evidence that stance control knee-ankle foot orthoses increase walking velocity compared to locked knee devices. Contrary to expectation, the stance control condition did not decrease energy expenditure during walking.