Inspiratory muscle training in neuromuscular patients: Assessing the benefits of a novel protocol.

BACKGROUND: Neuromuscular diseases are characterized by the compromise of respiratory muscles, thoracic ventilation, muscle strength and coughing capacity. Patients have low quality of life and increased morbidity and mortality mostly due to respiratory impairment. OBJECTIVE: To assess the benefits of adding inspiratory muscle training to neuromuscular patients' treatment and their compliance to the approach. METHODS: We conducted a single-center prospective study with neuromuscular patients with decreased maximal inspiratory pressure. We developed an inspiratory muscle training protocol with three-month duration and once-daily training. The protocol had a progressive intensity that was individually tailored based on patients' baseline characteristics and tolerance. We used Powerbreathe Medic Classic devices to perform the training. RESULTS: There were 21 patients who met the inclusion criteria and were enrolled in the study. Muscular dystrophy (n= 12, 57.3%) and amyotrophic lateral sclerosis (n= 4, 19%) were the most common diseases. After three months of training, patients increased their maximal inspiratory muscle pressure (p= 0.002) and peak cough flow (p= 0.011). Compliance to the protocol was 99 ± 5.5%. CONCLUSIONS: This protocol showed significant improvements on pulmonary muscles function and might be considered as an adjunct treatment to neuromuscular treatment. However, these positive results require larger further studies to validate the clinical benefits long-term.

Indicaciones y seguimiento domiciliario de la tos asistida mecánica en el paciente neuromuscular / Indications and domiciliary follow-up of mechanical insufflation-exsufflation in patients with neuromuscular disorders

Introducción: La insuflación-exuflación mecánica (MI-E) es una importante terapia respiratoria para el enfermo neuromuscular, pero existen pocos datos publicados en relación con su uso en el domicilio. Objetivo: Evaluar las características clínicas de los pacientes en los que se prescribe la MI-E y analizar su cumplimiento, seguridad y causas de retirada a largo plazo. Material y métodos: Estudio observacional retrospectivo de 78 adultos con enfermedad neuromuscular a los que se les prescribió MI-E a domicilio en un hospital de tercer nivel entre enero de 2009 y junio de 2015. Se describen las características clínicas y funcionales en el momento de la indicación de la terapia, la frecuencia y los parámetros de uso, los efectos secundarios, así como las causas de abandono de la terapia y la mortalidad. Resultados: La mayoría de los pacientes eran hombres (67%), con una mediana de edad de 58 años. El diagnóstico mayoritario fue la esclerosis lateral amiotrófica (45% de los casos). Tenían afectación bulbar moderada-grave el 36% y el 22% utilizaba el dispositivo solo en fase insuflatoria. El 14% de los pacientes eran portadores de traqueostomía y el 69% de los casos utilizaban también ventilación mecánica. El cuidador era mayoritariamente quien realizaba la técnica (86%), diariamente en el 73% de los casos. Desde la prescripción fallecieron el 38% de los pacientes, la mayoría afectados de esclerosis lateral amiotrófica (96%), con una mediana de supervivencia de 8 meses. En un 6% la MI-E se retiró por ineficacia. Se describió un solo caso de efecto secundario, que no contraindicó el uso de la terapia. Conclusiones: La MI-E prescrita a domicilio es una técnica segura, con cumplimiento adecuado, incluso en pacientes con afectación bulbar y asociada a ventilación mecánica. Se constata la dependencia del enfermo hacia su cuidador para su práctica

Introduction: Mechanical insufflation-exsufflation (MI-E) is an important respiratory therapy for neuromuscular disorders, but there are few published data on its use at home. Objective: To assess the clinical characteristics of patients prescribed MI-E, and to analyse compliance, safety, and causes for long-term withdrawal. Material and methods: Retrospective observational study of 78 adults with neuromuscular disorders who were prescribed MI-E at home in a third level hospital from January 2009 to June 2015. The clinical and functional characteristics when the therapy was indicated, the frequency and parameters of use, adverse effects and causes of withdrawal and mortality were evaluated. Results: Most patients were men (67%) with a median age of 58 years. The main diagnosis was amyotrophic lateral sclerosis (45% of cases). Moderate-severe bulbar involvement was present in 36%, and 22% used the device only in the insufflation phase. Fourteen percent of patients had a tracheostomy and 69% of the patients underwent mechanical ventilation. MI-E was mostly performed by the caregiver (86%), on a daily basis in 73% of the patients. After prescription, 38% patients died; most of these patients had amyotrophic lateral sclerosis (96%), with a median survival of 8 months. In 6%, MI-E was withdrawn due to ineffectiveness. An adverse effect was reported in only one patient and did not contraindicate the use of the therapy. Conclusions: In our experience, MI-E prescribed at home was well tolerated and safe, even in patients with bulbar involvement. Patients showed good compliance with MI-E alone or associated with mechanical ventilation, but required help from caregivers for its performance

A Human-Humanoid Interaction Through the Use of BCI for Locked-In ALS Patients Using Neuro-Biological Feedback Fusion.

This paper illustrates a new architecture for a human-humanoid interaction based on EEG-brain computer interface (EEG-BCI) for patients affected by locked-in syndrome caused by Amyotrophic Lateral Sclerosis (ALS). The proposed architecture is able to recognise users' mental state accordingly to the biofeedback factor , based on users' attention, intention, and focus, that is used to elicit a robot to perform customised behaviours. Experiments have been conducted with a population of eight subjects: four ALS patients in a near locked-in status with normal ocular movement and four healthy control subjects enrolled for age, education, and computer expertise. The results showed as three ALS patients have completed the task with 96.67% success; the healthy controls with 100% success; the fourth ALS has been excluded from the results for his low general attention during the task; the analysis of factor highlights as ALS subjects have shown stronger (81.20%) than healthy controls (76.77%). Finally, a post-hoc analysis is provided to show how robotic feedback helps in maintaining focus on expected task. These preliminary data suggest that ALS patients could successfully control a humanoid robot through a BCI architecture, potentially enabling them to conduct some everyday tasks and extend their presence in the environment.

Do Psychosocial Interventions Improve Quality of Life and Wellbeing in Adults with Neuromuscular Disorders? A Systematic Review and Narrative Synthesis.

Quality of life and well-being are frequently restricted in adults with neuromuscular disorders. As such, identification of appropriate interventions is imperative. The objective of this paper was to systematically review and critically appraise quantitative studies (RCTs, controlled trials and cohort studies) of psychosocial interventions designed to improve quality of life and well-being in adults with neuromuscular disorders. A systematic review of the published and unpublished literature was conducted. Studies meeting inclusion criteria were appraised using a validated quality assessment tool and results presented in a narrative synthesis. Out of 3,136 studies identified, ten studies met criteria for inclusion within the review. Included studies comprised a range of interventions including: cognitive behavioural therapy, dignity therapy, hypnosis, expressive disclosure, gratitude lists, group psychoeducation and psychologically informed rehabilitation. Five of the interventions were for patients with Amyotrophic Lateral Sclerosis (ALS). The remainder were for patients with post-polio syndrome, muscular dystrophies and mixed disorders, such as Charcot-Marie-Tooth disease, myasthenia gravis and myotonic dystrophy. Across varied interventions and neuromuscular disorders, seven studies reported a short-term beneficial effect of intervention on quality of life and well-being. Whilst such findings are encouraging, widespread issues with the methodological quality of these studies significantly compromised the results. There is no strong evidence that psychosocial interventions improve quality of life and well-being in adults with neuromuscular disorders, due to a paucity of high quality research in this field. Multi-site, randomised controlled trials with active controls, standardised outcome measurement and longer term follow-ups are urgently required.

Self-regulation of brain rhythms in the precuneus: a novel BCI paradigm for patients with ALS.

OBJECTIVE: Electroencephalographic (EEG) brain-computer interfaces (BCIs) hold promise in restoring communication for patients with completely locked-in stage amyotrophic lateral sclerosis (ALS). However, these patients cannot use existing EEG-based BCIs, arguably because such systems rely on brain processes that are impaired in the late stages of ALS. In this work, we introduce a novel BCI designed for patients in late stages of ALS based on high-level cognitive processes that are less likely to be affected by ALS. APPROACH: We trained two ALS patients via EEG-based neurofeedback to use self-regulation of theta or gamma oscillations in the precuneus for basic communication. Because there is a tight connection between the precuneus and consciousness, precuneus oscillations are arguably generated by high-level cognitive processes, which are less likely to be affected by ALS than processes linked to the peripheral nervous system. MAIN RESULTS: Both patients learned to self-regulate their precuneus oscillations and achieved stable online decoding accuracy over the course of disease progression. One patient achieved a mean online decoding accuracy in a binary decision task of 70.55% across 26 training sessions, and the other patient achieved 59.44% across 16 training sessions. We provide empirical evidence that these oscillations were cortical in nature and originated from the intersection of the precuneus, cuneus, and posterior cingulate. SIGNIFICANCE: Our results establish that ALS patients can employ self-regulation of precuneus oscillations for communication. Such a BCI is likely to be available to ALS patients as long as their consciousness supports communication.

A cognitive brain-computer interface for patients with amyotrophic lateral sclerosis.

Brain-computer interfaces (BCIs) are often based on the control of sensorimotor processes, yet sensorimotor processes are impaired in patients suffering from amyotrophic lateral sclerosis (ALS). We devised a new paradigm that targets higher-level cognitive processes to transmit information from the user to the BCI. We instructed five ALS patients and twelve healthy subjects to either activate self-referential memories or to focus on a process without mnemonic content while recording a high-density electroencephalogram (EEG). Both tasks are designed to modulate activity in the default mode network (DMN) without involving sensorimotor pathways. We find that the two tasks can be distinguished after only one experimental session from the average of the combined bandpower modulations in the theta- (4-7Hz) and alpha-range (8-13Hz), with an average accuracy of 62.5% and 60.8% for healthy subjects and ALS patients, respectively. The spatial weights of the decoding algorithm show a preference for the parietal area, consistent with modulation of neural activity in primary nodes of the DMN.

Active music therapy approach in amyotrophic lateral sclerosis: a randomized-controlled trial.

This randomized controlled study assessed the efficacy of active music therapy (AMT) on anxiety, depression, and quality of life in amyotrophic lateral sclerosis (ALS). Communication and relationship during AMT treatment were also evaluated. Thirty patients were assigned randomly to experimental [AMT plus standard of care (SC)] or control (SC) groups. AMT consisted of 12 sessions (three times a week), whereas the SC treatment was based on physical and speech rehabilitation sessions, occupational therapy, and psychological support. ALS Functional Rating Scale-Revised, Hospital Anxiety and Depression Scale, McGill Quality of Life Questionnaire, and Music Therapy Rating Scale were administered to assess functional, psychological, and music therapy outcomes. The AMT group improved significantly in McGill Quality of Life Questionnaire global scores (P=0.035) and showed a positive trend in nonverbal and sonorous-music relationship during the treatment. Further studies involving larger samples in a longer AMT intervention are needed to confirm the effectiveness of this approach in ALS.

Respiratory training improved ventilatory function and respiratory muscle strength in patients with multiple sclerosis and lateral amyotrophic sclerosis: systematic review and meta-analysis.

BACKGROUND: Among neurodegenerative diseases, multiple sclerosis (MS) and amyotrophic lateral sclerosis (ALS) have a high rate of respiratory disability. OBJECTIVES: To analyze the effects of respiratory muscle training (RMT) on ventilatory function, muscle strength and functional capacity in patients with MS or ALS. DATA SOURCES: A systematic review and meta-analysis of randomized controlled trials (RCTs) was performed. The sources were MEDLINE, PEDro, Cochrane CENTRAL, EMBASE, and LILACS, from inception to January 2015. STUDY SELECTION/ELIGIBILITY CRITERIA: The following were included: RCTs of patients with neurodegenerative diseases (MS or lateral ALS) who used the intervention as RMT (inspiratory/expiratory), comparison with controls who had not received RMT full time or were receiving training without load, and evaluations of ventilatory function (forced vital capacity - FVC, forced expiratory volume in one second - FEV1, maximum voluntary ventilation - MVV), respiratory muscle strength (maximal expiratory pressure/maximum inspiratory pressure - MEP/MIP) and functional capacity (6-minute walk test - 6MWT). RESULTS: The review included nine papers, and a total of 194 patients. It was observed that RMT significantly increased at MIP (23.50cmH2O; 95% CI: 7.82 to 39.19), MEP (12.03cmH2O; 95% CI: 5.50 to 18.57) and FEV1 (0.27L; 95% CI: 0.12 to 0.42) compared to the control group, but did not differ in FVC (0.48L; 95% CI: -0.15 to 1.10) and distance in 6MWT (17.95m; 95% CI: -4.54 to 40.44). CONCLUSION: RMT can be an adjunctive therapy in the rehabilitation of neurodegenerative diseases improving ventilatory function and respiratory strength.

Inspiratory muscle training in patients with Amyotrophic Lateral Sclerosis: A systematic review.

BACKGROUND: Amyotrophic Lateral Sclerosis is a neurodegenerative disease with rapid involvement of the inspiratory muscles, leading to respiratory insufficiency. Death often occurs by aspiration and pneumonia. Endurance- and strength therapy within ALS are discussed controversially. OBJECTIVE: To review the current literature to assess the efficacy of inspiratory muscle training for ALS. METHOD: Systematic review, using databases as PubMed, PEDro, Cochrane and Google Scholar. INTERVENTION: Inspiratory muscle training vs. sham training or inspiratory muscle training alone. OUTCOME MEASURES: Inspiratory muscle strength, dyspnoea, quality of life and survival time. RESULTS: Four studies could be included in this review, two RCT's, one pre-experimental study and one with a historical control group. In total 73 patients underwent inspiratory muscle training. CONCLUSION: Studies varied in onset of the training, the training protocol and the outcomes measured. At time, there is limited evidence that inspiratory muscle training leads to strengthening of inspiratory muscles in ALS. Improvements made were minor, in only a few parameters and also in control groups. Survival time was significantly longer in the experimental group in one study. Interesting suppositions (diaphragm training vs. other IM training, improvement of chest wall and lung compliance) need to be examined in robustly designed future trials, defining exact therapeutic windows and interventions.

Aquatherapy for neurodegenerative disorders.

Aquatherapy is used for rehabilitation and exercise; water provides a challenging, yet safe exercise environment for many special populations. We have reviewed the use of aquatherapy programs in four neurodegenerative disorders: Parkinson's disease, multiple sclerosis, amyotrophic lateral sclerosis, and Huntington's disease. Results support the use of aquatherapy in Parkinson's disease and multiple sclerosis, however further evidence is required to make specific recommendations in all of the aforementioned disorders.

[Communication with ALS patients: neurosurgical approach].

By progression of the disease, motor neurons degenerate in patients with amyotrophic lateral sclerosis (ALS) eventually lose nearly all voluntary muscles in the body. They are awake and aware but cannot move or communicate (locked-in state). Since the function of the brain is preserved, one possible measure to support their communication is to interpret their motor intention by decoding (deciphering) brain signals and present it with external devices. This technology called "brain-machine interface (BMI)" is now close to clinical use in Japan and USA.In our system, we record electrocorticogram (ECoG) obtained with subudural electrodes during their motor imagery, decode it and determine the movement they intended. So far, one patient of ALS with severe paralysis, implanted with this electrodes, successfully operated the PC communication tool only by thinking.

Speech therapy and communication device: impact on quality of life and mood in patients with amyotrophic lateral sclerosis.

Dysarthria has a drastic impact on the quality of life of ALS patients. Most patients suffering from dysarthria are offered speech therapy. Communication devices are prescribed less frequently. In the present study we investigated the impact of these therapeutic arrangements on quality of life in ALS patients. Thirty-eight ALS patients with dysarthria or anarthria, who underwent speech therapy and/or used communication devices answered three standardized questionnaires (Beck Depression Inventory - II (BDI), SF-36 Health Survey questionnaire (SF-36) and ALS Functional Rating Scale-revised (ALSFRS-R)) and were further interviewed about their experience with and benefit of speech therapy and communication devices. Most of the patients described a high impact of the communication device on their quality of life while the influence of speech therapy was rated less. By multiple regression analysis we confirmed an independent positive effect of communication device use on depression and psychological distress. In conclusion, communication systems improve or at least stabilize quality of life and mood in dysarthric ALS patients, and should be provided early in the disease course.

Comparison of classification methods for P300 brain-computer interface on disabled subjects.

We report on tests with a mind typing paradigm based on a P300 brain-computer interface (BCI) on a group of amyotrophic lateral sclerosis (ALS), middle cerebral artery (MCA) stroke, and subarachnoid hemorrhage (SAH) patients, suffering from motor and speech disabilities. We investigate the achieved typing accuracy given the individual patient's disorder, and how it correlates with the type of classifier used. We considered 7 types of classifiers, linear as well as nonlinear ones, and found that, overall, one type of linear classifier yielded a higher classification accuracy. In addition to the selection of the classifier, we also suggest and discuss a number of recommendations to be considered when building a P300-based typing system for disabled subjects.

[What physical therapy techniques can be used to improve airway freedom in amyotrophic lateral sclerosis?]. / Quelles sont les modalités de thérapie physique symptomatique incluant les techniques de désencombrement bronchique?

In individuals with ALS rehabilitation is mainly designed to prevent fatigue and contracture, to improve independence and activities for as long as possible, to optimize ability to live with the handicap, and finally to maximize quality of life. The functional impairment must be defined and physical therapy techniques have to be adapted to each patient and reevaluated frequently during the course of the disease. Various types of massage and exercise, monitored by a physical therapist are effective. Strengthening or endurance exercises are controversial as exercise may injure muscle fibres and motor neurons. Isometric exercise, short of fatigue, of unaffected muscles is recommended. Range of motion exercise is critically important for preventing contraction. Assistive and adaptative equipments are essential for maintaining the patient's activities of daily living and home equipment preserves independence. Several orthoses for hand, arm, foot or cervical weakness are available. A wheelchair is an important adaptative device when walking becomes too fatiguing or impossible. Choice for special options and features may require attention. Pulmonary complications are prevented with adapted techniques for bronchic obstruction. Based on the degree of weakness of limb and axial muscles six stages of functional impairment can be defined ranging from fully ambulatory in stage I to bedridden and totally dependent in stage VI. This staging provides a framework for physical therapy evaluation and guidance for appropriate rehabilitation in ALS patients.

The thought-translation device (TTD): neurobehavioral mechanisms and clinical outcome.

The thought-translation device (TTD) consists of a training device and spelling program for the completely paralyzed using slow-cortical brain potentials (SCP). During the training phase, the self-regulation of SCPs is learned through visual-auditory feedback and positive reinforcement of SCPs; during the spelling phase, patients select letters or words with their SCPs. A psychophysiological system for detection of cognitive functioning in completely paralyzed patients is an integral part of the TTD. The neurophysiological and anatomical basis of SCP-regulation was investigated by recording of BOLD-response in functional magnetic resonance imaging. Results showed involvement of basal ganglia and premotor cortex for required SCP positivity. The clinical outcome of 11 paralyzed patients using the TTD and quality of life of severely paralyzed patients is described. First attempts to improve learning of brain regulation with transcranial magnetic stimulation were successful.

Training locked-in patients: a challenge for the use of brain-computer interfaces.

Training severely paralyzed patients to use a brain-computer interface (BCI) for communication poses a number of issues and problems. Over the past six years, we have trained 11 patients to self-regulate their slow cortical brain potentials and to use this skill to move a cursor on a computer screen. This paper describes our experiences with this patient group including the problems of accepting and rejecting patients, communicating and interacting with patients, how training may be affected by social, familial, and institutional circumstances, and the importance of motivation and available reinforcers.

Graz-BCI: state of the art and clinical applications.

The Graz-brain-computer interface (BCI) is a cue-based system using the imagery of motor action as the appropriate mental task. Relevant clinical applications of BCI-based systems for control of a virtual keyboard device and operations of a hand orthosis are reported. Additionally, it is demonstrated how information transfer rates of 17 b/min can be acquired by real time classification of oscillatory activity.

A non-invasive communication device for the paralyzed.

An EEG-based communication system has been developed to re-establish communication in severely paralyzed patients who operate the device by generating shifts of their slow cortical potentials. Training to gain control over slow cortical potentials was based on visual feedback and operant conditioning strategies. The vertical movement of a graphic signal on a computer screen informs the patients about the course of their slow cortical potential amplitude. Positive slow cortical potential shifts move the cursor up, negative shifts move it down. These shifts are then translated into binary responses. When a patient has achieved reliable control over his/her slow cortical potential shifts, these responses can be used to select or reject items presented at the bottom of the screen. As learning processes and applications differ considerably between patients, the present paper describes the data from one patient with amyotrophic lateral sclerosis. After about three months of training, this patient gained stable, near-perfect control over his slow cortical potentials. This skill enabled him to operate a specially designed program to communicate messages to his caregivers.

Self-initiation of EEG-based communication in paralyzed patients.

OBJECTIVES: Severely paralyzed patients could learn to voluntarily generate slow cortical potential (SCP) shifts in their electroencephalogram and to use these signals to operate a communication device. To enhance the patients' autonomy, the present study describes the development of a permanently available communication system that can be turned on and off by locked-in patients without external assistance. A skill necessary for turning the system on is the ability to regulate one's slow potentials in the absence of continuous feedback. METHODS: A stepwise learning approach was employed to train two paralyzed patients to regulate their SCPs without continuous feedback. Elements of the original communication system were gradually removed and elements of the new stand-by mode were introduced. RESULTS: At the end of the learning procedure, both patients achieved correct response rates of above 84% in training sessions without continuous feedback. This skill enabled them to turn the communication device on and off without assistance from others. CONCLUSIONS: These findings suggest that severely paralyzed individuals can learn to operate an EEG-based communication device autonomously.

Maximum insufflation capacity.

OBJECTIVE: To investigate the effect of deep lung insufflations on maximum insufflation capacities (MICs) and peak cough flows (PCFs) for patients with neuromuscular disease. METHOD: Forty-three patients with neuromuscular disease were trained in stacking delivered volumes of air to deep lung insufflation and were prescribed a program of air stacking once their vital capacities (VCs) were noted to be < 2,000 mL. VC, MIC, and unassisted and assisted PCF were monitored. The initial data were compared with the highest MICs subsequently achieved. For those patients whose MICs only decreased, we compared the initial data with the most recent data. RESULTS: The MICs increased from (mean +/- SD) 1,402 +/- 530 mL to 1,711 +/- 599 mL (p < 0.001) for 30 patients and only decreased for 13 patients. Patients for whom the MICs increased also had a significant increase in assisted PCF from 3.7 +/- 1.4 to 4.3 +/- 1.6 L/s (p < 0.05) despite having somewhat decreasing VCs and unassisted PCFs. CONCLUSION: With training, the capacity to stack air to deep insufflations can improve despite progressive neuromuscular disease. This can result in increased cough effectiveness.