key: cord-0945519-12vzsl6p
authors: Pinto, Susana; Quintarelli, Stefano; Silani, Vincenzo
title: New technologies and Amyotrophic Lateral Sclerosis – Which step forward rushed by the COVID-19 pandemic?
date: 2020-08-05
journal: J Neurol Sci
DOI: 10.1016/j.jns.2020.117081
sha: ffbf17ed6ff3849e83dcf4ffafedc937b31745c6
doc_id: 945519
cord_uid: 12vzsl6p

Amyotrophic Lateral Sclerosis (ALS) is a fast-progressive neurodegenerative disease leading to progressive physical immobility with usually normal or mild cognitive and/or behavioural involvement. Many patients are relatively young, instructed, sensitive to new technologies, and professionally active when developing the first symptoms. Older patients usually require more time, encouragement, reinforcement and a closer support but, nevertheless, selecting user-friendly devices, provided earlier in the course of the disease, and engaging motivated carers may overcome many technological barriers. ALS may be considered a model for neurodegenerative diseases to further develop and test new technologies. From multidisciplinary teleconsults to telemonitoring of the respiratory function, telemedicine has the potentiality to embrace other fields, including nutrition, physical mobility, and the interaction with the environment. Brain-computer interfaces and eye tracking expanded the field of augmentative and alternative communication in ALS but their potentialities go beyond communication, to cognition and robotics. Virtual reality and different forms of artificial intelligence present further interesting possibilities that deserve to be investigated. COVID-19 pandemic is an unprecedented opportunity to speed up the development and implementation of new technologies in clinical practice, improving the daily living of both ALS patients and carers. The present work reviews the current technologies for ALS patients already in place or being under evaluation with published publications, prompted by the COVID-19 pandemic.

The Information and Telecommunications Revolution, also known as Digital Revolution or Third Industrial Revolution, was the most impacting technological revolution in the Human History so far, through the development and implementation of the Internet (6) . "Emerging technology" is defined as a "radically novel and relatively fast growing technology characterised by a certain degree of coherence persisting over time and with the potential to exert a considerable impact on the socio-economic domain(s) which is observed in terms of the composition of actors, institutions and patterns of interactions among those, along with the associated knowledge production processes" (7) . Telemedicine is an open and constant evolving science incorporating new information and communication technological advancements to adapt and respond to the changing health care needs of the societies. The word "telemedicine" is used since the 1970s, initially meaning "healing at a distance" (8) and defined by the World Health Organization (WHO) as "The delivery of health care services, where distance is a critical factor, by all health care professionals using information and communication technologies for the exchange of valid information for diagnosis, treatment and prevention of disease and injuries, research and evaluation, and for the continuing education J o u r n a l P r e -p r o o f of health care providers, all in the interests of advancing the health of individuals and their communities" (9) . Home tele-management for chronic disease surveillance is of main concern for the European Community and the ""vision for Europe 2020"" calls for the implementation of telemedicine collaborative arrangements. In high income countries, it is mostly focused on diagnosis and therapeutics but, nowadays, it can be as wide as including evaluation, assessment, monitoring, prevention, intervention, supervision, education, consultation, and coaching (10) , with the potentiality to transform health care delivery by shifting it from hospitals/ clinics to home care (11).

A. Telemedicine a) Multidisciplinary consults Multidisciplinary ALS consults have been sparsely done through telemedicine, either by live videoconferences on real-time, synchronous (12) (13) (14) (15) (16) (17) (18) (19) (20) (21) (22) (23) (24) or though the store-and-forward method, asynchronous (25) . Videoconferences connect the multidisciplinary ALS teams at the tertiary centres to the patients at home, or to the local community hospitals/ health centres (12, 21, 22) Two Australian tertiary hospitals established regular videoconferences with the local hospitals or community health services assisting ALS patients living far from the hospitals, thereby replacing the regular multidisciplinary in-clinic 3-mo consults (12) . Videoconferences were considered feasible, although respiratory J o u r n a l P r e -p r o o f and palliative concerns arose (12) . Feasibility, even in advanced stages, was also reported by the tele-visit program as a supplement of the regular visits developed by the Massachusetts General Hospital ALS clinic (13). Patients lived 211 miles from the clinic (median) and the consults involved the contact with a physician and a nurse. Frequent addressed issues were medication management (89%), discussion of goals of care (74%), research (55%) and equipment use (50%). However, acute care issues were rarely discussed (13).

Adjusted cost-savings to medical usefulness of those video tele-visits as compared to in-clinic visits were further addressed (14) , representing, per visit, $997 for the patients (in-clinic visit $1116; video tele-visit adjusted for medical usefulness $119) and $327 for the institution (in-clinic visit $799; video televisit adjusted for medical usefulness $472). Patients and providers rated medical usefulness modestly lower for video tele-visits, despite following the American Academy of Neurology Guidelines (26) , as compared to in-clinic visits.

On the contrary, a study conducted by the Veterans Affairs ALS Center in Ohio addressing outcomes, disease progression, malnutrition and survival, reported that videoconsult multidisciplinary care provided by physicians, nurses and other health providers when needed, had the same quality and similar clinical outcomes as in-clinic visits (15) . The latter group had previously used direct mobile videoconsults with patients or interaction through platforms with healthcare providers at the hospital in close connection with the ALS team referring economic and time benefits (22) . High levels of satisfaction were reported in a prospective study addressing the feasibility and satisfaction of ALS patients, carers and healthcare providers with multidisciplinary tele-visits, J o u r n a l P r e -p r o o f conducted by the Penn State Health Hershey ALS Center (16) . Despite negative aspects (video/audio problems; no physical examination; no body language cues; more impersonal, less emotional connection; lack of privacy in individual conversations between the patient/ carer and the team), benefits included continuing providing care, understanding home dynamics of patients/ carers dynamics, no travel requirements, time savings in addition to less fatigue and stress for the patients, who were more comfortable and talkative (16) . Two modalities of care were further assessed -full multidisciplinary visits with the ALS team on ALS clinic days or 1-on-1 visits with individual ALS healthcare providers on other days (17) . The tele-visit modality was preferred by the patients living farthest, also the ones having poorer physical and respiratory function and more likely to use NIV (17) . Despite patients" satisfaction, good acceptance and reduced time burden, psychological and emotion issues have also been identified as needing to be discussed with face-to-face contact.

Therefore, teleconsults (performed through a platform with access to a chat room and videoconsults) were considered to be an addition to the face-to-face consult and not to replace them (20) .

In Sheffield, questionnaires on the condition of patients and carers weekly collected by the Tele-health in Motor Neuron Disease (TiM) were evaluated by a nurse from the multidisciplinary ALS team, who could, afterwards, clarify the information by phoning the patient/ carer, expedite clinic appointments or establish a liaison with the team. Recordings included weight and balance, the revised ALS functional rating scale (ALSFRS-R), pain and saliva assessments, quality of life (QoL) scales, the Hospital Anxiety and Depression (HAD) scale, in J o u r n a l P r e -p r o o f addition to the carers" strain, depression and anxiety (18, 19) . Reported potential benefits included improved communication and care coordination, reassurance, identification of complications and being an alternative or addition to clinic, although a friendlier algorithm to deal with clinical alerts was needed (18, 19) .

A store-and-forward method (25) by having nurse home-visits videorecorded and later discussed by the multidisciplinary team was considered to be feasible and gathering a good team satisfaction. The less positive points included lack of physical examination and being time-consuming to review the videos, write recommendations and further discuss the plan with the patient, in addition to the necessary technological, financial and ethical support and considerations as well as the time required to train the nurse (25) .

Multidisciplinary consults via telemedicine due to COVID-19 pandemic have been started in multiple centers worldwide. In Italy, a group has recently followed 32 patients with motor neuron disease (29 with ALS, 4 of whom with associated fronto-temporal dementia) with a structured questionnaire collecting demographics, clinical information and functional scale (ALSFRS-R) but also information addressing possible signs/symptoms of COVID-19 infection (23) .

The questionnaire was reported to be feasible and the team was able to detect respiratory deterioration at distance, including respiratory decompensation requiring urgent hospitalization. No patients were diagnosed with COVID-19. In the satisfaction survey via email or WhatsApp, 90% of the patients or carers were satisfied with the teleconsult (70% very satisfied), 85% felt as being talking to the neurologist face-to-face, and 90% were interested in continuing J o u r n a l P r e -p r o o f the the follow-up visits through teleconsult, which could overcome social isolation, "abandoned" feelings during the illness as well as economic and time constraints associated to in-hospital visits (23) . Telephone and/or videoconsults has also been reported to be implemented/ ongoing in ALS centres integrating ALS consortiums/ networks, namely the Northeast ALS Consortium (NEALS) (24) (28, 29) . The authors used NIV devices with an integrated software, with internet access by TCP/IP connection and a modem station, allowing for real-time bidirectional tele-medical assistance, with immediate exchange of the ventilator data as well as parameter adjustments at distance. Home tele-monitoring and tele-assistance of home ventilated patients, not only with ALS but also with other neuromuscular disorders, were also effective by using a modem and a phone line connection to acquire patients" symptomatology and data from pulse oximeters at the Pulmonology Rehabilitation Center, IRCCS, in Brescia (30) (31) (32) . The number of hospitalizations J o u r n a l P r e -p r o o f and costs decreased, which was also reported when telemedicine was used to determine the need, on-demand, for MIE (33, 34) . In both the Portuguese and the Italian groups, telemedicine relied on a central control station, run by rehabilitation physicians in the Portuguese setting and by nurses with close connection to pulmonologists in the Italian group. In addition, both groups developed specific red flag alerts to call for a rapid medical response when needed. Hazenberg et al. (2014) followed patients with neuromuscular disorders using telemedicine (telephone calls in addition to data transmission from ventilator, nocturnal pulse oximeters and capnography) not only for monitoring NIV follow-up but also for its initiation, which was safe, feasible and cost-effective, with improvements in blood gas and in QoL not inferior to hospital initiation (35) . Transmission of data from spirometry was also feasible with high acceptability by patients, carers and respiratory therapists, showing forced vital capacity (FVC) and maximal inspiratory pressure (MIP) a strong correlation between remote and the standard assessments (36) . Recently, data from a self-reported questionnaire together with weekly ventilator and oximetry monitoring facilitated the maintenance of ventilation and SpO2 levels despite ALS progression (37) . In patients with Duchenne Muscle Dystrophy (38) and

Facio-Scapulo-Humeral Muscle Dystrophy (38, 39) tele-monitoring for home ventilatory support through videoconferencing with a multidisciplinary team and tele-monitoring of cardiorespiratory variables (oxygen saturation, heart rate, blood pressure and electrocardiogram) was feasible, user-friendly, efficient and reduced the need for hospital admissions. Weaning a patient with Duchenne Muscle Dystrophy off the ventilator using telemedicine to connect the in-patient J o u r n a l P r e -p r o o f visits by a nurse with a specialist at distance was also successful (40) . In accordance with the statement of the European Respiratory Society Telemonitoring of Ventilator-Dependent Patients Task Force (41), more evidence is needed for the applicability and efficacy of telemedicine in ventilated-dependent patients. Nonetheless, its developments (including hardware, software and cloud platforms) are considered to be likely to change future home NIV management (41,42). Despite improving healthcare access, particularly in rural/ remote areas, a risk of reducing standard services in health systems of developed countries is a concern (41). oxygen saturation during the exercise were transmitted via Bluetooth from a non-intrusive biosensor to a mobile phone, wireless connected to a digital platform, being the data directly available for analyses (44) . Telephone calls and email contacts with the multidisciplinary team were provided if necessary.

The exercise monitored at distance through telemedicine was safe, feasible, with good compliance (44) .

Controlling physical activity overtime in ALS is also feasible by its remote evaluation through accelerometers, with good compliance (93%), being the results strongly correlated with ALSFRS-R, with low variability (45) . Physical activity has also been successfully assessed at-home through accelerometers and an ECG sensor in addition to in-hospital evaluations every 3-months, also including speech recording in the latter setting (46) . Although comfortable and well tolerable, sensors can be associated to skin and subcutaneous lesions, specially dermatitis (46) .

The Penn State Health Hershey ALS Center is presently assessing movement in relationship to falls in ALS at home through UL and LL wearable devices as well as a pendant monitor assessing general activity (17) .

Developing an automated fall detection algorithm is planned (17) .

A randomized open-label standard of care controlled trial compared nutritional counselling in ALS through an app to provide tailored nutrition recommendations as compared to counselling by a physician/ nurse (standard of care) and to in-person counselling provided by a dietician (in-person) (47) .

App recordings were done biweekly for dietary intake and weight or monthly if in-person. The app nutritional counselling was feasible, safe, but not superior to the in-person or standard of care at 6-mo follow-up (47) . On the contrary, another group studied self-reporting through an app of weight (weekly) in addition to daily well-being and monthly functional status and considered it J o u r n a l P r e -p r o o f easy, helpful and not burdensome, with an 83% adherence, adding value to the in-clinic visits and providing additional personalized feedback (48) . e) Telephone calls, emails, apps, and clinical trials Surprisingly, immediate telephone calls to the healthcare team or emails are not a frequent resource for getting medical care, as represented respectively by 27% and 17% in a clinical survey carried out in the United Kingdom and Australia involving ALS patients (49) (50) (51) . The exponential growth of telephone apps and different platforms have, however, been frequently used.

Physicians can monitor, at distance, patient"s symptoms, functional status through functional scales, and medication. It also engages and empowers patients in their clinical status and medical decisions and directly connects the patients one-another, as exemplified by the PatientsLikeMe platform (52).

Defining automatic alerts to track clinical changes could allow the health teams to rapidly act upon them (18).

The need to promote patient recruitment in ALS clinical trials, at distance, decreasing the burden on patients/ carers, drove the Barrow Neurological Institute and the Beth Israel Deaconess Medical Centers to develop the ALS AT HOME web portal (53) . Patients were recruited all over US, enrolled, consented and trained entirely at distance with online videos. The data from handgrip dynamometry, actigraphy, spirometry, electrical impedance myography, ALSFRS-R and patient reported experience measures, as well as speech trough a different application, were recorded and analysed (53) . ET and BCIs allow ALS patients and others with severe motor involvement, particularly locked-in syndrome, to live, communicate and control the surrounding with software, electronic or electromechanical systems through brain activity, without muscle contribution. Controlling computers or wheelchairs and switching on-off electrical devices is thereby possible. In ALS, studies in ET and BCIs focus on its applicability in AAC and, lately, on the administration of neuro-cognitive tests/ tasks.

ET applicability for AAC in ALS patients addressed internet surfing, emailing, phone-calls and social networking, with good performance (54,55). In regular ET users, in late-stage ALS with tetraplegia and anarthria, ET increased communication abilities, QoL and patients" satisfaction (56) . Not only ET for AAC improves Qol but it diminishes significantly caregivers" burden (57) . QoL in locked-in state ALS patients is maintained on the cost of the QoL of their next J o u r n a l P r e -p r o o f of kin, as assessed by ET (58) , with well-being usually underestimated by carers (59) .

Assessing cognition in non-verbal, unable to write ALS patients led to the development of neuropsychological assessments to be performed through ET. Keller et al. (2015) showed that performance accuracy in Raven's coloured progressive matrices was significantly correlated, as verified for d2-test only in controls but not in ALS (60) . Results either in paper or through ET were lower in patients and discriminative of cognitive impairment. In a second study (61) can also be present (64) . P300 evoked potentials, steady state visual evoked potentials, mu rhythm or slow cortical potentials have been addressed in BCI overpassing ET"s ocular limitations.

McCane et al. (2014) showed that most severely disabled ALS patients could use the Wadsworth BCI (P300-based) home system (65) . Good BCI accuracies measures were achieved, despite disease severity, age, EEG montages or recording quality, with higher P300 amplitudes and more anterior located (fronto-centrally) in patients with good accuracies. Auditory and eyesclosed steady-state visual evoked potentials were suggested as an alternative if visual impairment was not overcome (by ptosis-glasses or eye-patch for diplopia). Using P300-based auditory BCI in ALS was feasible by using visual and/or auditory P300-based BCI to test controls and 3 patients in a four-choice oddball paradigm ("yes", "no", "pass", "end") (66) . Nevertheless, the visual and visual plus auditory modality had higher accuracy levels, with higher speed.

By using a P300-based matrix speller, Nijboer et al. (2008) showed that severely disabled ALS patients could communicate producing both cued and spontaneous text, with relatively stable performance over a 40-week period, as demonstrated by P300 amplitude and latency (67) . Not only in late stages of the disease but also in early and middle stages can ALS patients use a P300-high consistently, increasingly steadily overtime.

Cognitive functions in late-stage ALS patients were assessed by Iversen et al. by developing a SCPs EEG-BCI system, which required extensive initial training for controlling EEG components to move a cursor on a monitor (72) . A series of two-choice cognitive discriminative tasks as well as matching-tosample paradigm, assessing performance in discriminating numbers, letters and colours, and simple computations was addressed. In a second study (73) Few studies have directly compared ET with BCI. When comparing ET with P300 BCI, Pasqualotto et al. (2015) showed that the information transfer rate and System Usability Scale score were significantly higher for ET, with a significantly lower cognitive workload (76) . Therefore, ET seems to be more suitable for patients when no ocular involvement limits it, which can be overcome by BCI. The comparison of electrooculography, ET and auditory BCI for AAC tested by a regular low-tech AAC user in locked-in stage, was feasible but none were considered of an additional use although auditory BCI was recognized as favouring independence from eye control, but more tiring (77) . b) Controlling the environment A robot tele-operated by ALS patients with a joystick and buttons and able to move around obstacles, pick up objects (despite different configurations and various types of flooring) and deliver them back (78) was successfully tested with high overall satisfaction (6.7 out of 7). It was considered to be significantly easier to use than the patients" own hands, asking family members for help or using mechanical reaching devices. 

Multi-functional robotics can not only be used for assisting the patients but also for their assessment, training and reassessment overtime, possible by using the same robot. Robotic rehabilitation in ALS is giving its first steps.

Contrary to other non-progressive diseases that begin as an acute event, the required support by the robot in ALS patients would be minimal at early stages of the disease, when the intensity and frequency of the training can be higher.

The progressive physical weakness in ALS requires higher support provided by the robot and successive adjustments in the intensity and frequency of the training (decrease effort). More complex robots and software will provide a wider range of programs, relevant for motor learning as it is task-specific.

Associating visual and auditory biofeedback, as provided by softwares with task-specific exercises/ games, increase the efficacy of the training. In a recent study, a 2-mo specific task-oriented right-UL exoskeleton training (Armeo For further information on the mentioned articles on other emergent technologies, please refer to Table 1 .

In the context of a highly contagious virus, for which the world population had not previous contact with, and without vaccines or effective therapies, more than 3.9 billion people (half of the world population) have been home confined in more than 90 countries/ territories. The population was asked to seek medical help only in acute cases to prevent being infected at healthcare infrastructures, with special advice for high risk patients, as ALS patients (84). (Table 2) . Planning, creativity and adaptability are essential to maintain the clinical care to these patients.

Steps have already been done in the way to move forward, being the widespread of telemedicine crucial. It has been shown to be feasible and safe, with good compliance and with time and cost savings for both patients/ carers and healthcare systems. The creation of platforms for bidirectional transmission of encrypted data, synchronization of devices, storage and data analyses, accordingly to ethical and political policies across countries, is essential. The availability of hardware and software, with the necessary adaptabilities to the patients during the progression of the disease, and the training of patients/ carers is a limiting factor. Nevertheless, tele-consults via internet or even over the phone (if no internet/ hardware availability), more focused on symptomatology and functional assessments, including nutritional, respiratory and psychological functional assessments, is feasible from day 1. In the market, not-so expensive sensors or basic devices are already available, which can, wireless or not, be connected to smartphones, to measure different biological parameters, including cutaneous temperature, heart rate, blood pressure, ML is a one of the most common forms of AI, a statistical technique for fitting models to data and to "learn" by training models with data, with high importance to healthcare. The most common application of traditional ML in healthcare is precision medicine -predicting which treatment protocols are likely to succeed based on various patient attributes and the treatment context. 

From the intersection of technology and medicine emerges the generation and processing of people-related data. Although no global regulation exists, in many jurisdictions there are limitations, sometimes particularly strict on how to handle and use this data. In some countries, health data is treated as personal data while, in others, as particular personal data. In the United States, in general, personal data is considered marketable. In Europe, as opposed to the previous directive (European Directive 95/46/EC) (93) in which the protection of personal data was regulated with specific limitations on health data processing, the General Data Privacy Regulation (GDPR), European Union (EU) Regulation n.2016/679 (94,95), has no specific regulation regarding health data, beyond specific references related to the application of certain standards or institutions. "Health-related data" or "data concerning health" is the "personal data Table 1 . Other emerging technologies being tested in ALS J o u r n a l P r e -p r o o f Table 2 . Proposed conditions for when to seek physical contact with the medical personnel at health facilities vs when to maintain remote contact. 

EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS)-revised report of an EFNS task force

Methods of communication at end of life for the person with amyotrophic lateral sclerosis

Communication support for people with ALS

Quality of Life in Amyotrophic Lateral Sclerosis Patients and Caregivers: Impact of Assistive Communication From Early Stages

Primary communication facilitators

Digital Revolution

What Is an Emerging Technology?

One hundred years of telemedicine: does this new technology have a place in paediatrics?

A health telematics policy in support of WHO"s Health-fos-all strategy for global health development: report of the WHO group consultation on health telematics

Telemedicine Opportunities and Developments in State Members

Telehealth for motor neurone disease

Experience with telemedicine in a multi-disciplinary ALS clinic

Adjusted cost analyses of video televisits for the care of prople with Amyotrophic Lateral Sclerosis

Delivering tertiary centre specialty care to ALS patients via telemedicine: a retrospective cohort analysis

Incorporation of telehealth into a multidisciplinary ALS clinic: feasibility and acceptability

The use of telehealth to enhance care in ALS and other neuromuscular disorders

Process evaluation and explotation of telehealth in motor neuron disease in a UK specialist centre

Tele-treatment of patients with amyotrophic lateral sclerosis (ALS)

Comprehensive care and home telehealth to veterans with ALS

Early and innovative symptomatic care to improve quality of life of ALS patients at Cleveland VA ALS Center

Telemedicine is a useful tool to deliver care to patients with Amyotrophic Lateral Sclerosis during COVID-19 pandemic: results from Southern Italy

Amyotrophic Lateral Sclerosis Care and Research in the United States During the COVID-19 Pandemic: Challenges and Opportunities

Multidisciplinary ALS telemedicine care: the store and forward method

Quality improvement in neurology: amyotrophic lateral sclerosis quality measures: report of the quality measurement and reporting subcommittee of the Ameri-can academy of neurology

Implementation of a Wireless Device for Real-Time Telemedical Assistance of Home-Ventilated Amyotrophic Lateral Sclerosis Patients: A Feasibility Study

Home telemonitoring of non-invasive ventilation decreases healthcare utilisation in a prospective controlled trial of patients with amyotrophic lateral sclerosis

Economic cost of hometelemonitoring care for BiPAP-assisted ALS individuals

A pilot study of nurse-led, home monitoring for

A pilot trial of telemedicine-assisted, integrated care for patients with advanced amyotrophic lateral sclerosis and their caregivers

Tele-assistance in Patients With Amyotrophic Lateral Sclerosis: Long Term Activity and Costs

At home and on-demand mechanical cough assistance program for patients with ALS

Pulmonary rehabilitation at home guided by telemonitoring and access to healthcare facilities for respiratory complications in patients with neuromuscular disease

Initiation of Home Mechanical Ventilation at Home: A Randomised Controlled Trial of Efficacy, Feasibility and Costs

Evaluation of remote pulmonary function testing in motor neuron disease

Incorporating Self-Reported Questions for Telemonitoring to Optimize Care of Patients With MND on Noninvasive Ventilation (MND OptNIVent)

Telemedicine system for the care of patients with neuromuscular disease and chronic respiratory failure

Telemedicine for Facio-Scapulo-Humeral Muscular Dystrophy: A multidisciplinary approach to improve quality of life and reduce hospitalization rate?

Use of home telemedicine for critical illness rehabilitation: an Indian success story

Technological advances in home non-invasive ventilation monitoring: Reliability of data and effect on patient outcomes

Respiratory exercise in amyotrophic lateral sclerosis

The Role of Moderate Aerobic Exercise as Determined by Cardiopulmonary Exercise Testing in ALS

Accelerometry for remote monitoring of physical activity in amyotrophic lateral sclerosis: a longitudinal cohort study

Objectively Monitoring Amyotrophic Lateral Sclerosis Patient Symptoms During Clinical Trials With Sensors: Observational Study

Nutritional counseling with or without mobile health technology: a randomized open-label standard-of-care-controlled trial in ALS

Telehealth as Part of Specialized ALS Care: Feasibility and User Experiences With "ALS Home-Monitoring and Coaching

Anything that makes life"s journey better. Exploring the use of digital technology by people living with motor neurone disease

Communications Technology and Motor Neuron Disease: An Australian Survey of People with Motor Neuron Disease

perspectives of multidisciplinary homebased e-Health service delivery for motor neurone disease

ALS longitudinal studies with frequent data collection at home: study design and baseline data

Eye tracking communication devices in amyotrophic lateral sclerosis: impact on disability and quality of life

An eye-tracking assistive device improves the quality of life for ALS patients and reduces the caregivers" burden

Eye-tracking-based Assessment Suggests Preserved Well-Being in Locked-In Patients

An Observational Study on Quality of Life and Preferences to Sustain Life in Locked-In State

Eye-tracking Controlled Cognitive Function Tests in Patients With Amyotrophic Lateral Sclerosis: A Controlled Proof-Of-Principle Study

A first approach to a neuropsychological screening tool using eye-tracking for bedside cognitive testing based on the Edinburgh Cognitive and Behavioural ALS Screen

An Eye-Tracker Controlled Cognitive Battery: Overcoming Verbal-Motor Limitations in ALS

The Arrows and Colors Cognitive Test (ACCT): A New Verbal-Motor Free Cognitive Measure for Executive Functions in ALS

Oculomotor Dysfunction in Amyotrophic Lateral Sclerosis: A Comprehensive Review

Brain computer interface (BCI) evaluation in people with amyotrophic lateral sclerosis

A P300-based brain-computer interface: initial tests by ALS patients

A P300-based Brain-Computer Interface for People With Amyotrophic Lateral Sclerosis

Based Brain-Computer Interface Communication: Evaluation and Follow-up in Amyotrophic Lateral Sclerosis

Independent home use of a brain computer interface by people with amyotrophic lateral sclerosis

Fully implanted brain-computer interface in a locked-in patient with ALS NEJMoa1608085

Stability of a Chronic Implanted Brain-Computer Interface in late-stage Amyotrophic Lateral Sclerosis

A braincomputer interface tool to assess cognitive functions in completely paralyzed patients with amyotrophic lateral sclerosis

Conditional associative learning examined in a paralyzed patient with amyotrophic lateral sclerosis using brain-computer interface technology

Cognitive assessment of executive functions using brain computer interface and eye-tracking

Cognitive assessment in Amyotrophic Lateral Sclerosis by means of P300-Brain Computer Interface: a preliminary study

Usability and Workload of Access Technology for People With Severe Motor Impairment: A Comparison of Brain-Computer Interfacing and Eye Tracking

Comparison of eye tracking, electrooculography and an auditory brain-computer interface for binary communication: a case study with a participant in the locked-in state

Dusty: An Assistive Mobile Manipulator That Retrieves Dropped Objects for People With Motor Impairments

Eye-controlled, power wheelchair performs well for ALS patients

A robotic neck brace to characterize head-neck motion and muscle electromyography in subjects with amyotrophic lateral sclerosis

The Feasibility of Using Robotic Technology to Quantify Sensory, Motor, and Cognitive Impairments Associated With ALS

A promising tool for flail arm in amyotrophic lateral sclerosis rehabilitation: a case report

Robotic gait rehabilitation and substitution devices in neurological disorders: where are we now?

Effects of robot-assisted upper limb rehabilitation in stroke patients: a systematic review with meta-analysis

The role of virtual reality in improving motor performance as revealed by EEG: a randomized clinical trial

The clinical utility if virtual reality in Neurorehabilitation: A systematic review

Machine Learning in Amyotrophic Lateral Sclerosis: Achievements, Pitfalls, and Future Directions

The ALSFRS-R: A revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS study group (phase III)

A Proposed Staging System for Amyotrophic Lateral Sclerosis

Development and evaluation of a clinical staging system for amyotrophic lateral sclerosis

The authors are particularly grateful to Dr Barbara Poletti for providing Figure 1 .

ET conditions. BCI with high levels of satisfaction, progressive usage effort, reliable long-term functional stabi Cognition ET