Interventions to promote oral nutritional behaviours in people living with neurodegenerative disorders of the motor system: A systematic review.

BACKGROUND & AIMS: Weight loss is common in people with neurodegenerative diseases of the motor system (NDMS), such as Parkinson's disease and Amyotrophic Lateral Sclerosis, and is associated with reduced quality of life, functional ability and survival. This systematic review aims to identify interventions and intervention components (i.e. behaviour change techniques [BCTs] and modes of delivery [MoDs]) that are associated with increased effectiveness in promoting oral nutritional behaviours that help people with NDMS to achieve a high calorie diet. METHODS: Eight electronic databases including MEDLINE and CINAHL were searched from inception to May 2018. All interventions from included studies were coded for relevant BCTs and MoDs. Methodological quality of studies was assessed using the Cochrane risk of bias tool. RESULTS: Fourteen studies were included. Of these, eight studies reported interventions to assist with swallowing difficulties and six studies reported interventions targeting dietary content. Beneficial effects in managing swallowing difficulties were observed with video assisted swallowing therapy, lung volume recruitment and swallowing management clinics with outpatient support. In contrast, studies reporting effectiveness of chin down posture, use of thickened liquids and respiratory muscle training were inconclusive. Positive effects in interventions targeting dietary content included the use of food pyramid tools, individualised nutritional advice with nutritional interventions, electronic health applications, face-to-face dietary counselling and high fat, high carbohydrate and milk whey protein supplements. Individualised nutritional advice with weekly phone contact did not appear to be effective. Most frequently coded BCTs were 'instructions on how to perform the behaviour', 'self-monitoring' and 'behavioural practice/rehearsal'. Most commonly identified MoDs were 'human, face-to-face' and 'somatic therapy'. However, the robustness of these findings are low due to the small number of studies, small sample sizes and large between-study variability. CONCLUSIONS: Despite the limited evidence, these findings may help inform the development of more effective interventions to promote oral nutritional behaviours in people with NDMS. However, further research is needed to demonstrate which interventions, or intervention components, yield most benefit.

Exercise therapy for muscle and lower motor neuron diseases.

Muscle and lower motor neuron diseases share a common denominator of perturbed muscle function, most often related to wasting and weakness of muscles. This leads to a number of challenges, such as restricted mobility and respiratory difficulties. Currently there is no cure for these diseases. The purpose of this review is to present research that examines the effects of exercise in muscle and lower motor neuron diseases. Evidence indicates that moderate intensity aerobic- and strength exercise is advantageous for patients with muscle diseases, without causing harmful exercise-induced muscle damage. On the contrary, motor neuron diseases show a rather blunted response from exercise training. High-intensity training is a modality that seems safe and a promising exercise method, which may circumvent neural fatigue and provide effect to patients with motor neuron disease. Although we have come far in changing the view on exercise therapy in neuromuscular diseases to a positive one, much knowledge is still needed on what dose of time, intensity and duration should be implemented for different disease and how we should provide exercise therapy to very weak, non-ambulatory and wheelchair bound patients.

A self-care, problem-solving and mindfulness intervention for informal caregivers of people with motor neurone disease: A pilot study.

BACKGROUND: Informal caregivers of people with motor neurone disease (MND) take on an extensive role. Caregivers are at increased risk of experiencing psychological distress and burden, yet, there is a lack of intervention programmes to support them. AIM: The aim of this study was to investigate the feasibility and acceptability of a therapeutic group intervention promoting self-care, problem-solving and mindfulness to informal caregivers of people with MND. DESIGN: Pilot study that utilised a one-arm pre- and post-design. Acceptability of the intervention was assessed 2 weeks post intervention with a questionnaire designed specifically for this study. Feasibility was assessed with consent, adherence and reasons for non-participation, refusal and attrition. Participants completed baseline and follow-up (6-week post intervention) questionnaires for psychological morbidity, burden, problem-solving, mindfulness and preparedness. Settings/participants: Caregivers of people with a diagnosis of MND within the past 12 months who were 18 years or older; who could speak, read and write in English and who were attending a progressive neurological diseases clinic were eligible. RESULTS: A total of 13 caregivers participated in one of three group intervention sessions which were focused on self-care, problem-solving and mindfulness. The intervention appeared to be feasible and acceptable. All participants stated that they would recommend the intervention to others. The group format appeared to be highly valued. There was no significant change in measures between pre-intervention and 6 weeks post intervention. CONCLUSION: This pilot serves as an initial step for examining interventions for MND caregivers, with the hope of identifying effective, efficient and sustainable strategies to best support this group.

Identification-Based Closed-Loop NMES Limb Tracking With Amplitude-Modulated Control Input.

An upper motor neuron lesion (UMNL) can be caused by various neurological disorders or trauma and leads to disabilities. Neuromuscular electrical stimulation (NMES) is a technique that is widely used for rehabilitation and restoration of motor function for people suffering from UMNL. Typically, stability analysis for closed-loop NMES ignores the modulated implementation of NMES. However, electrical stimulation must be applied to muscle as a modulated series of pulses. In this paper, a muscle activation model with an amplitude modulated control input is developed to capture the discontinuous nature of muscle activation, and an identification-based closed-loop NMES controller is designed and analyzed for the uncertain amplitude modulated muscle activation model. Semi-global uniformly ultimately bounded tracking is guaranteed. The stability of the closed-loop system is analyzed with Lyapunov-based methods, and a pulse frequency related gain condition is obtained. Experiments are performed with five able-bodied subjects to demonstrate the interplay between the control gains and the pulse frequency, and results are provided which indicate that control gains should be increased to maintain stability if the stimulation pulse frequency is decreased to mitigate muscle fatigue. For the first time, this paper brings together an analysis of the controller and modulation scheme.

Why don't they accept non-invasive ventilation?: insight into the interpersonal perspectives of patients with motor neurone disease.

OBJECTIVES: Although non-invasive ventilation (NIV) can benefit survival and quality of life, it is rejected by a substantial proportion of people with motor neurone disease (MND). The aim of this study was to understand why some MND patients decline or withdraw from NIV. METHOD: Nine patients with MND (male = 7, mean age = 67 years) participated in this study. These patients, from a cohort of 35 patients who were offered NIV treatment to support respiratory muscle weakness, did not participate in NIV treatment when it was clinically appropriate. Semi-structured interviews and interpretative phenomenological analysis (IPA) were employed to explore these patient's experience of MND and their thoughts and understanding of NIV treatment. RESULTS: Using IPA, four themes were identified: preservation of the self, negative perceptions of NIV, negative experience with health care services, and not needing NIV. Further analysis identified the fundamental issue to be the maintenance of perceived self, which was interpreted to consist of the sense of autonomy, dignity, and quality of life. CONCLUSIONS: The findings indicate psychological reasons for disengagement with NIV. The threat to the self, the sense of loss of control, and negative views of NIV resulting from anxiety were more important to these patients than prolonging life in its current form. These findings suggest the importance of understanding the psychological dimension involved in decision-making regarding uptake of NIV and a need for sensitive holistic evaluation if NIV is declined. Statement of contribution What is already known on this subject? Non-invasive ventilation is widely used as an effective symptomatic therapy in MND, yet about a third of patients decline the treatment. Psychological disturbance generated by NIV use leads to negative experiences of the treatment. Decision-making about treatment potentials is complex and unique to each individual affected by perceived impact of disease. What does this study add? A decision concerning NIV uptake was influenced by perceived impact on individuals' sense of self. Sense of self was influenced by the maintenance of autonomy, dignity, and quality of life. Individuals' sense of self was identified to have been challenged by the disease, NIV, and their experience of health care service.

Data condensed synthesis regarding kinesiotherapeutic procedures used in spasticity therapy.

Spasticity represents an important feature of the upper motoneuron syndrome (UMNS). The clinical signs, such as the abnormal movement models, the unwanted muscular co-contractions, the muscular and joint rigidity with a consecutive deformity can be signs of spasticity and, also of upper motoneuron lesion. The different therapeutic options applied in the management of spasticity are a basic component of UMNS treatment scheme. This article presents the main kinesiotherapeutic procedures used in spasticity therapy.

Integrated health care for patients with motor neurone disease.

This article presents the findings from a study trip to Kaiser Permanente (KP), a private healthcare provider in the USA. The aim of the trip was to understand how healthcare integration is managed in KP and how this might help patients in the UK with motor neurone disease (MND). This article makes reference to the American and British healthcare systems, identifying the simple differences between health economies, and their impact on health care, with specific reference to MND. The trip was undertaken as part of the author's ongoing work on how patients with MND rate services delivered by the multidisciplinary team (MDT) in the UK. The author's community matron role involves caring for patients with long-term conditions (LTCs) including long-term neurological conditions (LTNCs). In executing this role and in service delivery to patients with LTNCs, specifically MND, the author noticed a lack of robust integration, highlighting the need to consider and address the various contributory factors. This article presents a literature review and analyses the role of the MDT including specialist neurological professionals in executing duties and in delivering healthcare services to patients diagnosed with MND. The implications for practice are also presented along with areas for practice development.

Experiences of dying, death and bereavement in motor neurone disease: a qualitative study.

OBJECTIVES: to explore the experiences of people with Motor Neurone Disease (MND), current and bereaved carers in the final stages of the disease and bereavement period. METHODS: a qualitative study using narrative interviews was used to elicit accounts from 24 people with MND and 18 current family carers and 10 former family carers. RESULTS: the needs of patients and carers are not being adequately met in the final stages of MND and there appears a need for increased, co-ordinated support from palliative care services. The use of advance care planning tools is regarded as beneficial for patients and carers, but health professionals demonstrate a limited understanding of them. Anxiety and distress in patients, carers and bereaved carers is heightened during this period. Carer burden is excessive and may exacerbate patient distress and desire for hastening death. CONCLUSION: this study has identified a number of issues people with MND and their carers face in the final stages of the illness, indicating some ways in which health, social and palliative care services could be improved or co-operate more effectively in order to better meet their needs.

Muscle, tendons, and bone: structural changes during denervation and FES treatment.

OBJECTIVES: This paper describes a novel approach to determine structural changes in bone, muscle, and tendons using medical imaging, finite element models, and processing techniques to evaluate and quantify: (1) progression of atrophy in permanently lower motor neuron (LMN) denervated human muscles, and tendons; (2) their recovery as induced by functional electrical stimulation (FES); and (3) changes in bone mineral density and bone strength as effect of FES treatment. METHODS: Briefly, we used three-dimensional reconstruction of muscle belly, tendons, and bone images to study the structural changes occurring in these tissues in paralysed subjects after complete lumbar-ischiadic spinal cord injury (SCI). These subjects were recruited through the European project RISE, an endeavour designed to establish a novel clinical rehabilitation method for patients who have permanent and non-recoverable muscle LMN denervation in the lower extremities. This paper describes the use of segmentation techniques to study muscles, tendons, and bone in several states: healthy, LMN denervated-degenerated but not stimulated, and LMN denervated-stimulated. Here, we have used medical images to develop three-dimensional models and advanced imaging, including computational tools to display tissue density. Different tissues are visualized associating proper Hounsfield intervals defined experimentally to fat, connective tissue, and muscle. Finite element techniques are used to calculate Young's modulus on the patella bone and to analyse correlation between muscle contraction and bone strength changes. RESULTS: These analyses show restoration of muscular structures, tendons, and bone after FES as well as decline of the same tissues when treatment is not performed. This study suggests also a correlation between muscle growth due to FES treatment and increase in density and strength in patella bone. CONCLUSION: Segmentation techniques and finite element analysis allow the study of the structural changes of human skeletal muscle, tendons, and bone in SCI patient with LMN injury and to monitor effects and changes in tissue composition due to FES treatment. This work demonstrates improved bone strength in the patella through the FES treatment applied on the quadriceps femur.

Conservative treatment with hyperbaric oxygen therapy for cervical spondylotic amyotrophy.

STUDY DESIGN: Small case series of patients with cervical spondylotic amyotrophy (CSA) managed by conservative treatment with hyperbaric oxygen (HBO) therapy. OBJECTIVE: To study the effects of conservative treatment with HBO therapy of CSA patients. SETTING: Department of Orthopaedic Surgery, Imakiire General Hospital, Kagoshima, Japan. METHODS: This study included 10 patients with CSA who underwent rehabilitation, including cervical traction and muscle exercise, for some period of time but did not respond well to it, and were then managed by additional HBO therapy for rehabilitation. Information was obtained on the duration of symptoms and strength of the most atrophic muscle, intramedullary high-signal-intensity changes on T2-weighted magnetic resonance imaging, presence of 'snake-eyes' appearance and the number of stenotic canal levels. RESULTS: The mean duration of symptoms before HBO treatment was 3.1 months. The axial T2-weighted magnetic resonance images of all 10 patients showed a 'snake-eyes' appearance. The mean number of stenotic canal levels was 0.3. There was marked improvement on manual muscle testing from a mean of 1.9 pretreatment to a mean of 4.4 at the last follow-up after HBO therapy. The outcomes of all 10 patients, whose results were classified as excellent or good, were considered clinically satisfactory. CONCLUSION: To our knowledge, conservative treatment with HBO therapy for CSA patients has not previously been described. It appears that HBO therapy might improve ischemic injury of the anterior horns in CSA patients with short duration of symptoms.

Subcutaneous immunoglobulin therapy for multifocal motor neuropathy.

The objective of this study was to investigate whether subcutaneous immunoglobulin (SCIg) treatment is feasible and safe in maintaining muscle strength of patients with multifocal motor neuropathy (MMN). Patients fulfilling the EFNS/PNS criteria for definite MMN treated with intravenous immunoglobulin (IVIg) were switched to weekly SCIg in a single-center, open-label pilot intervention study. The first group of patients started with a SCIg dose equivalent to 50% of the IVIg maintenance dose. In case of deterioration, patients received a loading dose of IVIg and doubling of SCIg dose. The second group started with a dose equivalent to the IVIg maintenance dose. Primary outcome was the Medical Research Council (MRC) sum score from 10 muscle groups. Secondary outcomes were grip and pinch strength, dexterity, disability, quality of life, adverse events, and serum immunoglobulin concentrations. Ten patients were included, five in both groups. In the first group, one patient withdrew informed consent due to local adverse events, four deteriorated. In the second group, four out of five patients maintained muscle strength with SCIg during the 6 months follow-up. Local adverse events were frequent, especially during first weeks of treatment, but generally well tolerated. Seven mild systemic adverse events were reported, all but one in the first week of treatment. In some, but not all MMN patients in this study, SCIg therapy was feasible and safe and maintained strength as well as IVIg. SCIg may be a viable alternative maintenance therapy in some patients with MMN currently receiving IVIg.

Lullament: lullaby and lament therapeutic qualities actualized through music therapy.

Lullabies and laments promote new awareness, enculturation, adaptation, and grief expression. These concepts' relevance to palliative care, however, has not been examined. In this study, a music therapist used a grounded theory-informed design to reflexively analyze lullaby and lament qualities, evident in more than 20 years of personal palliative care practice. Thus, the construct "lullament" emerged, which signified helpful moments when patients' and families' personal and sociohistorical relationship with lullabies and laments were actualized. Specific music could be both a lullaby and a lament. A music therapist can enable the lullament through providing opportunities for music-contextualized "restorative resounding," expressed psychobiologically, verbally, musically, and metaphorically.

Modulation of motoneuronal firing behavior after spinal cord injury using intraspinal microstimulation current pulses: a modeling study.

We simulated the effects of delivering focal electrical stimuli to the central nervous system to modulate the firing rate of neurons and alleviate motor disorders. Application of these stimuli to the spinal cord to reduce the increased excitability of motoneurons and resulting spasticity after spinal cord injury (SCI) was examined by means of a morphologically detailed computer model of a spinal motoneuron. High-frequency sinusoidal and rectangular pulses as well as biphasic charge-balanced and charge-imbalanced pulses were examined. Our results suggest that suprathreshold high-frequency sinusoidal or rectangular current pulses could inactivate the Na+ channels in the soma and initial segment, and block action potentials from propagating through the axon. Subthreshold biphasic charge-imbalanced pulses reduced the motoneuronal firing rate significantly (up to approximately 25% reduction). The reduction in firing rate was achieved through stimulation-induced hyperpolarization generated in the first node of Ranvier. Because of their low net DC current, these pulses could be tolerated safely by the tissue. To deliver charge-imbalanced pulses with the lowest net DC current and induce the largest reduction in motoneuronal firing rate, we studied the effect of various charge-imbalanced pulse parameters. Short pulse durations were found to induce the largest reduction in firing rate for the same net DC level. Subthreshold high-frequency sinusoidal and rectangular current pulses and low-frequency biphasic charge-balanced pulses, on the other hand, were ineffective in reducing the motoneuronal firing rate. In conclusion, the proposed electrical stimulation paradigms could provide potential rehabilitation interventions for suppressing the excitability of neurons to reduce the severity of motor disorders after injury to the central nervous system.

Muscle biopsies show that FES of denervated muscles reverses human muscle degeneration from permanent spinal motoneuron lesion.

This paper presents biopsy analyses in support of the clinical evidence of muscle recovery induced by a new system of life-long functional-electrical-stimulation (FES) training in permanent spinal-motoneuron-denervated human muscle. Not earlier than 1 year after subjects experienced complete conus cauda lesion, their thigh muscles were electrically stimulated at home for several years with large skin surface electrodes and an expressly designed stimulator that delivered much longer impulses than those presently available for clinical use. The poor excitability of long-term denervated muscles was first improved by several months of twitch-contraction training. Then, the muscles were tetanically stimulated against progressively increased loads. Needle biopsies of vastus lateralis from long-term denervated subjects showed severe myofiber atrophy or lipodystrophy beginning 2 years after spinal cord injury (SCI). Muscle biopsies from a group of 3.6- to 13.5-year denervated subjects, who underwent 2.4 to 9.3 years of FES, show that this progressive training almost reverted long-term muscle atrophy/degeneration.

Living with motor neurone disease: lives, experiences of services and suggestions for change.

Palliative care involves the complete, holistic care of people with progressive illness and their families. People living with motor neurone disease (MND) require a range of multidisciplinary palliative care services. However, there are significant gaps in our understanding of these people's lives, experiences of services and their suggestions for service change. The present study addressed the following questions: (1) What are the lived experiences of people living with MND? (2) What are people's experiences of services? and (3) Can improvements to care be identified? A qualitative research design was adopted using semi-structured interviews. The topic guide was developed from existing literature. The study was based in three boroughs in London, UK. People living with MND and professionals were drawn from a database at King's College Hospital, and additionally, through 'snowball' sampling. Nine people with MND, five carers/family members and 15 professionals took part in the interviews. These interviews were audio-taped, transcribed and checked for accuracy against the original recordings. Themes within the interviews were coded and grouped. The analysis was facilitated with the NVIVO computer software package. The findings are presented within three substantive groups: (1) the impact of MND on people's lives (the physical impacts of the illness, including increasing disability; social issues, including restrictions on social activities; and adjustments to people's lives, including methods of coping with the illness); (2) experiences of services (accessing service entitlements, information sources, professionals' attitudes and approaches, and professionals' knowledge and understanding of MND); and (3) suggestions for service change (better information and communication, including information on service entitlements; improved knowledge amongst professionals about MND; and some suggestions for service restructuring). This study brings a fresh approach to understanding the impacts of MND and the ways in which services can be improved to meet people's needs. The paper concludes with methodological considerations, the implications of the findings for practitioners and policy makers, and suggestions for further research.

Therapeutic vaccine for acute and chronic motor neuron diseases: implications for amyotrophic lateral sclerosis.

Therapeutic vaccination with Copaxone (glatiramer acetate, Cop-1) protects motor neurons against acute and chronic degenerative conditions. In acute degeneration after facial nerve axotomy, the number of surviving motor neurons was almost two times higher in Cop-1-vaccinated mice than in nonvaccinated mice, or in mice injected with PBS emulsified in complete Freund's adjuvant (P < 0.05). In mice that express the mutant human gene Cu/Zn superoxide dismutase G93A (SOD1), and therefore simulate the chronic human motor neuron disease amyotrophic lateral sclerosis, Cop-1 vaccination prolonged life span compared to untreated matched controls, from 211 +/- 7 days (n = 15) to 263 +/- 8 days (n = 14; P < 0.0001). Our studies show that vaccination significantly improved motor activity. In line with the experimentally based concept of protective autoimmunity, these findings suggest that Cop-1 vaccination boosts the local immune response needed to combat destructive self-compounds associated with motor neuron death. Its differential action in CNS autoimmune diseases and neurodegenerative disorders, depending on the regimen used, allows its use as a therapy for either condition. Daily administration of Cop-1 is an approved treatment for multiple sclerosis. The protocol for non-autoimmune neurodegenerative diseases such as amyotrophic lateral sclerosis, remains to be established by future studies.

Nutritional issues and supplements in amyotrophic lateral sclerosis and other neurodegenerative disorders.

PURPOSE OF REVIEW: Aggressive nutritional intervention has become a cornerstone of treatment for many patients with neuromuscular diseases, in particular, motor neuron disease. Malnutrition is a common problem among patients with amyotrophic lateral sclerosis. Over the past decade, the recognition of nutrition as an independent, prognostic factor for survival and disease complications in amyotrophic lateral sclerosis has illustrated the importance of individualized nutritional management in symptomatic treatment. Paramount issues for nutritional management in amyotrophic lateral sclerosis include caloric supplementation, the diagnosis/treatment of dysphagia, and the timing/safety/efficacy of percutaneous endoscopic gastrostomy placement. RECENT FINDINGS: In addition, many amyotrophic lateral sclerosis patients self-medicate with a variety of vitamins, herbs, and other dietary supplements. Outcome-based research for the use of nutraceuticals and functional foods in the treatment and prevention of amyotrophic lateral sclerosis and other neuromuscular diseases is in its early stages. In the past year, however, several interesting papers have been published that lend support to the use of dietary supplements as primary treatments for amyotrophic lateral sclerosis and other motor neuron disorders. SUMMARY: Common or overlapping etiologies in disparate neurodegenerative diseases have led to the promise that optimal nutritional care and the appropriate use of dietary supplements in amyotrophic lateral sclerosis will have implications for the nutritional management of other degenerative conditions such as Parkinson's, Alzheimer's, and Huntington's disease. Furthermore, evidence supporting the efficacy of dietary supplements in amyotrophic lateral sclerosis may lend clues to the treatment of other neuromuscular disorders such as the muscular dystrophies.